Haematology past paper questions

Question 1

She received bridging therapy with low molecular weight heparin (LMWH) until her INR was > 2 for 24 hours. For the past week she has been taking 4mg of warfarin.

Her target INR is 2.5. Her INR has been checked today and is 1.3.

What is the most appropriate management?

Continue 4mg of warfarin and start low molecular weight heparin

Increase dose of warfarin to 6mg and start low molecular weight heparin

Increase dose of warfarin to 6mg

Start low molecular weight heparin and stop warfarin

Re-load the patient with 5mg or 10mg Warfarin (as per local protocol)

Answer 1

Increase dose of warfarin to 6mg and start low molecular weight heparin

As her INR is < 2 she needs immediate anticoagulation with rapid acting low molecular weight heparin. Her INR should be carefully monitored and the LMWH discontinued when has adequate anticoagulation (INR>2)

Question 2

A 34 year old female with a history of Beta-thalessemia major presents to her general practitioner. She has noted that her periods have stopped,she has leg swelling and shortness of breath. She has also been recently diagnosed with diabetes mellitus and hypothyroidism. Examination is unremarkable.

Given the likely diagnosis, what is the most appropriate treatment?

Blood transfusion

Desferrioxamine

Levothyroxine

Venesection

Ferrous Sulphate

Answer 2

Desferrioxamine

This patient has features of pituitary failure and heart failure. This is secondary due to deposition of excess iron from her repeated blood transfusions. Mortality for iron overload may be treated by chelating excess iron which is how desferrioxamine works

Question 3

A 65-year-old man attends the emergency department with a 6-hour history of a severe generalised headache and blurred vision. He has had similar headaches on and off for the past few months. He reports no photophobia or neck stiffness. He has a past medical history of Waldenström macroglobulinaemia (WM). On examination, his blood pressure is 170/107 mmHg and he is apyrexial. Fundoscopy reveals bilateral papilloedema and widespread fundal haemorrhages.

He has a CT head, which shows no evidence of any intracranial masses or bleeds.

Which of the following is the most likely diagnosis?

Subarachnoid haemorrhage

Hyperviscosity syndrome

Tension headache

Idiopathic intracranial hypertension

Answer 3

Hyperviscosity syndrome

Hyperviscosity syndrome is a condition characterised by a high plasma viscosity. WM is the most common cause, although it may also be associated with polycythaemia, leukaemia, paraneoplastic syndromes and connective tissue disorders. Hyperviscosity syndrome typically presents with headache, blurred vision, hypertension, heart failure, dilutional anaemia/thrombocytopenia, ischaemic stroke and venous thromboembolism. The presence of features of hyperviscosity (headache, blurred vision, and papilloedema) with a past medical history of WM makes hyperviscosity syndrome the most likely diagnosis.

Question 4

A 2 year old boy is brought to the Accident and Emergency department by his parents in pain. A couple of hours ago, whilst out for a walk with his parents, he began crying excessively saying “it hurts!”. It is a particularly cold day outside. There was no history of trauma.

He was diagnosed with sickle cell disease at birth. He has otherwise been well recently, as has everyone else in his household.

Examination reveals no abnormalities. His observations are stable.

Investigations show that he has a haemoglobin (Hb of 90g/L), with measurements 3 months ago being recorded as 88g/L and 6 months ago as 93g/L.

What type of sickle cell “crisis” is this child most likely experiencing?

Acute chest syndrome

Vaso-occlusive crisis

Aplastic crisis

Sequestration crisis

Acute haemolytic anaemia

Answer 4

Vaso-occlusive crisis

This is correct. The acute onset of the pain and with no abnormalities on examination and no significant drop in haemoglobin make a vaso-occlusive or “painful” crisis the most likely explanation. Common triggers include the cold (as in this scenario), dehydration, infection and hypoxia. Vaso-occlusive crises may present with pain anywhere in the body and in toddlers under 3 years, hands and feet are commonly affected. Vaso-occlusive crises are caused by micro-occlusion of blood vessels by sickled red blood cells

Question 5

Mechanism of hyposplenism in SS

Answer 5

In addition, sickle cells commonly sequester in the spleen and undergo phagocytosis by the reticular endothelial system leading to extravascular haemolysis. Commonly this leads to splenic congestion and splenomegaly. The spleen is necessary for phagocytosis of encapsulated bacteria. Consequently, due to a compromised spleen, there is reduced immune function and individuals with sickle cell disease are prone to bacteraemia.

Question 6

Most common acute presentation in SC

Answer 6

Painful vaso occlusive crises are the most common acute presentation of sickle cell anaemia, caused primarily by microvascular obstruction due to cellular sickling, which may be triggered by local hypoxia (e.g. in cold weather).

Question 7

other presentations of sickle cell

Answer 7

• vaso-occlusive crises
• acute chest sydnrome
• aplastic crises
• sequestration crises

Question 8

Vaso-occlusive crisis

Answer 8

This is correct. The acute onset of the pain and with no abnormalities on examination and no significant drop in haemoglobin make a vaso-occlusive or “painful” crisis the most likely explanation. Common triggers include the cold (as in this scenario), dehydration, infection and hypoxia. Vaso-occlusive crises may present with pain anywhere in the body and in toddlers under 3 years, hands and feet are commonly affected. Vaso-occlusive crises are caused by micro-occlusion of blood vessels by sickled red blood cells

Question 9

Aplastic crisis

Answer 9

Aplastic crisis causes a sudden reduction in marrow production and so a drop in haemoglobin and red blood cell production. It would also not present with acute pain. Furthermore, it is usually caused by parvovirus B19 infection, and there is no indication that the child or any of his close contacts has been unwell recently

Question 10

Sequestration crisis

Answer 10

Sequestration crisis presents with a sudden severe anaemia and signs of shock. Sequestration crisis occurs due to pooling of blood in the spleen as the spleen atrophies

Question 11

Acute chest crises features

Answer 11

In contrast, acute chest crises are the most dangerous acute presentation (3% mortality; cause of 25% of sickle cell related deaths). The cause is often unknown (may be infectious), but patients present with tachypnoea, wheeze, and cough, with hypoxia and pulmonary infiltrates.

Question 12

Management of sickle cell crisis

Answer 12

Conservative management involves high flow oxygen, IV fluids and analgesia. Top-up transfusions may be required in severe cases.

Question 13

A 5-year-old boy is admitted to the paediatric haematology ward for treatment of acute lymphoblastic leukaemia (ALL). He is due to receive chemotherapy. Which of the following is a good prognostic factor in ALL?

Male gender

Increasing age

Slower response to chemotherapy

High white cell count

Hyperdiploid blast cells

Answer 13

Hyperdiploid blast cells

Hyperdiploidy (the presence of extra chromosomes in blast cells compared to the normal 46 in somatic cells) is associated with a better prognosis.

Question 14

A 27-year-old man presents to his general practitioner with a 4-month history of diarrhoea and weight loss. A vesicular, itchy rash is noted over his buttocks. He has no past medical history.

He has a full blood count showing:

Haemoglobin (Hb) 87 g/L (130–170 g/L)
Mean cell volume (MCV) 73 fL (80–96 fL)
White cell count 9 × 109/L (3.0–10.0 × 109/L)
Platelets 345 × 109/L (150–400 × 109/L)
A blood film is performed, which shows Howell–Jolly bodies and pencil cells.

Which of the following is the most likely mechanism of these laboratory findings?

Functional hyposplenism

Anaemia of chronic disease

Previous splenectomy

Chronic lymphocytic leukaemia (CLL)

Acute myeloblastic leukaemia (AML)

Answer 14

Functional hyposplenism

This patient’s most likely diagnosis is coeliac disease. He has a long history of diarrhoea and weight loss, which are typical symptoms of coeliac disease. He has a vesicular rash overlying the buttocks, which is likely dermatitis herpetiformis, an extra-articular feature of coeliac disease. The blood film shows pencil cells (iron deficiency anaemia secondary to coeliac disease) and Howell–Jolly bodies.

Howell–Jolly bodies are remnants of the erythrocyte nuclei that are contained within the erythrocyte. The presence of Howell–Jolly bodies on the blood film indicates functional hyposplenism, which is associated with coeliac disease.

Question 15

A 24-year-old presents to the emergency department with severe haemarthrosis. He has a background of haemophilia A, diagnosed shortly after birth. On examination, his chest is clear, and his observations are as follows:

Heart rate: 110 beats per minute
Blood pressure: 105/87 mmHg
Respiratory rate: 12 breaths per minute
SpO2: 100% on room air
What is the most appropriate treatment?

Fresh frozen plasma (FFP)

Desmopressin

Recombinant factor VIII

Corticosteroids

Vitamin K

Answer 15

Recombinant factor VIII

Haemophilia A affects factor VIII levels, and in major or life-threatening bleeds, recombinant factor VIII is the most appropriate treatment.

Question 16

A 72 year old male is admitted to a general medical ward with sepsis secondary to a chest infection. He subsequently develops epistaxis, haemoptysis, melaena and bleeding from his cannula site. His observations are documented in the table below. On examination he is drowsy but responds to voice. There are large bruises over the sites of previous venepuncture attempts.

Vital Sign Result
Temperature 38.2 degrees
Heart rate 112 bpm
Blood pressure 92/68 mm/Hg
Respiratory Rate 22 breaths per minute
Oxygen Saturations 94% on 4L via nasal cannula

Which of the following investigation findings is consistent with the most likely diagnosis?

Decreased partial thromboplastin time (PTT)

Thrombocytosis

Raised fibrinogen

Raised D-dimer

Decreased prothrombin time (PT)

Answer 16

Raised D-dimer

This is a case of disseminated intravascular coagulation (DIC). DIC occurs when the balance between the formation of new clots (coagulation) and the break down of clots (fibrinolysis) is tipped in favour of coagulation. It causes widespread clot formation and tissue ischaemia whilst also consuming platelets and clotting factors, thereby leading to excess bleeding. Conditions such as sepsis can release procoagulants (such as tissue factor) which trigger this process. D-dimer is a fibrin degradation product produced when fibrin clots are broken down. It is therefore raised in DIC due to increased clot formation and degradation

Question 17

A 60 year old male patient is undergoing a pre-operative review prior to an elective hernia repair. A routine full blood count reveals a haemoglobin of 19.2 g/dL and platelet count of 490 x 10^9/L. The patient has no past medical history of note and is asymptomatic at present. The patient is a non-smoker and drinks alcohol socially.

On physical examination there is mild splenomegaly and the patient is noted to have a red complexion.

Which of the following investigation findings are consistent with the most likely diagnosis?

Raised red cell mass, low serum erythropoetin, JAK2 mutation present

Normal red cell mass, decreased plasma volume, normal serum erythropoietin

Increased red cell mass, raised serum erythropoetin, JAK2 mutation present

Increased red cell mass, raised serum erythropoetin, JAK2 mutation absent

Increased red cell mass, low serum erythropoetin, JAK2 mutation absent

Answer 17

Raised red cell mass, low serum erythropoetin, JAK2 mutation present

This is the correct answer. The patient presents with clinical features consistent with polycythaemia rubra vera (PRV), a clonal haematopoeitic disorder charactersed by erythrocytosis. There is no evidence to suggest a secondary cause of polycythaemia (such as COPD). In PRV erythropoetin will be appropriately low (to prevent further erythrocytosis). PRV is strongly associated with the JAK2 V617F mutation

Question 18

A 26 year old Mediterranean man presents to his GP with recurrent fevers following a trip to Northern India. He is diagnosed with benign Plasmodium vivax malaria and started on oral chloroquine and primaquine. He had not taken malaria prophylaxis.

A few days later he presents to the emergency department with rapidly evolving jaundice and breathlessness.

What is the single most likely underlying diagnosis?

G6PD deficiency

Familial Mediterranean fever

Gilbert’s syndrome

Plasmodium falciparum malaria

Pneumonia

Answer 18

G6PD deficiency

This is the correct answer. Patients with G6PD tend to have ancestry from mediterranean basin and present with episodic haemolytic anaemia following exposure to oxidative stressors, which include anti-malarials (in particular primaquine)

Question 19

A 25-year-old man has presented to his general practitioner after noticing a lump in his neck a few days ago. He is not sure how long this has been there. It is not currently painful but he reports it became painful when he went out for some alcoholic drinks with his friends last Saturday. He has no other symptoms. On examination, a 2-cm rubbery, nontender lymph node is noted in the right anterior cervical chain. There is no evidence of any other lymphadenopathy.

Which of the following is the most likely diagnosis?

Non-Hodgkin’s lymphoma

Hodgkin’s lymphoma

Reactive lymphadenopathy

Infectious mononucleosis

Metastatic lymph nodes

Answer 19

Hodgkin’s lymphoma

Lymphomas typically present with a rubbery, nontender lymph node in the neck, with or without B symptoms. It is impossible to fully differentiate Hodgkin’s lymphoma and Non-Hodgkin’s lymphoma clinically, although there are certain factors in this case that make Hodgkin’s lymphoma more likely. Firstly, although Non-Hodgkin’s lymphoma can present at any age, Hodgkin’s lymphoma would typically present in a young men. Secondly, this patient experienced pain in his enlarged lymph node when he was drinking alcohol; this is a typical feature of Hodgkin’s lymphoma.

Question 20

A 13-year-old girl of Middle-Eastern origin is admitted with jaundice and shortness of breath on exertion. These symptoms have started over the course of the last day. She also complains of dark urine and denies any other symptoms. The patient has a past medical history of gallstones. No drug history or travel history is stated.

On examination conjunctival pallor and jaundice are noted. She also has henna decorating both of her hands.

Blood results show:

Haemoglobin 85 g/dL (120-150) Bilirubin: 30 mcmol/L (20-20) Coomb’s Test: Negative Blood film: Heinz bodies

What is the underlying pathology giving rise to this presentation?

G6PD haemolytic anaemia

Sickle cell anaemia

Iron deficiency anaemia

Autoimmune haemolytic anaemia

Infection induced haemolytic anaemia

Answer 20

G6PD haemolytic anaemia

This patient presents with evidence of haemolysis. The signs and symptoms of haemolysis include shortness of breath on exertion, pallor (anaemia), jaundice (bilirubinaemia) and dark urine (haemoglobinuria). Given her ethnic origin and the fact that she has had henna tattooing which can trigger haemolytic crises in patients with G6PD, this is the most likely answer

Question 21

Glucose-6-phosphate deficiency (G6PD) features

Answer 21

G6PD is an X-linked recessive red-cell enzyme disorder which may present in the neonatal period with jaundice, or later in life with episodic intravascular haemolysis following exposure to oxidative stressors.

Triggers of G6PD
* Intercurrent illness or infection (often forgotten)
* Fava beans: the disease was historically known as favism
* Henna
* Medications: primaquine, sulfa-drugs, nitrofurantoin, dapsone, and NSAIDs/Aspirin

Investigations of G6PD
The blood film typically shows Heinz bodies and bite cells, and the diagnostic test is a G6PD enzyme assay.

Management of G6PD
Treatment involves avoidance of precipitants, and some patients may rarely require transfusions.

Question 22

Key investigation in autoimmune haemolytic anaemia

Answer 22

To elucidate the aetiology of the haemolytic anaemia, the direct Coombs test may be used. It is a test for autoimmune haemolytic anaemia (AIHA).

Patients with AIHA have antibodies directed against cell surface markers on the red blood cell. The Coombs test uses antibodies against these autoantibodies to cross link and agglutinate the red cells.

Question 23

Two types of Coombs-positive haemolytic anaemia

Answer 23

Two major groups of Coombs-positive AIHA are known: - Warm
- Cold autoimmune haemolytic anaemia.

Question 24

Warm autoimmune haemolytic anaemia (AIHA) pathophysiology

Answer 24

Warm AIHA is an IgG mediated extravascular haemolytic disease in which the spleen tags cells for splenic phagocytosis

Causes of warm AIHA
* Idiopathic
* Lymphoproliferative neoplasms (CLL and lymphoma)
* Drugs including methyldopa
* SLE

Question 25

Cold autoimmune haemolytic anaemia (AIHA) pathophysiology

Answer 25

Cold AIHA is an IgM-mediated haemolytic disease in which IgM fixes complement causing direct intravascular haemolysis (also known as cold agglutinins) Causes of the cold AIHA: Idiopathic Post-infectious haemolytic anaemias: occurring 2-3 weeks after infection (examples include EBV and mycoplasma) Why cold The disease is termed “cold” because the antibodies are active and cause hemolysis at cold temperatures can cause clinical symptoms related to RBC agglutination in cooler parts of the body and hemolytic anemia.

Question 26

Coombs-negative haemolytic anaemia

Answer 26

Acquired haemolytic anaemias that are Coombs negative are a large group of diverse haemolytic disorders. Microangiopathic haemolytic anaemia Paroxysmal nocturnal haemoglobinuria Physical lysis of red cells e.g. malaria Haemolytic uraemic syndrome (E. coli 0157:H7) Infectious causes DIC (such as fulminant meningococcemia)

Question 27

A 28 year old man with a known diagnosis of sickle cell anaemia presents to his GP for review. He complains that, over the past 12 months, he has had an increased burden of minor infections, particularlly upper respiratory tract infections (URTI). He is currently well, his examination is normal and he is taking only hydroxyurea prophylaxis. The GP orders blood tests for routine monitoring. A blood film is performed which shows a small, single, peripherally-located, rounded inclusion in 50-60% of the erythrocytes. What is the single most likely underlying diagnosis? Acute bacterial infection Functional hyposplenism Babesiosis Acute haemolysis Acute leukaemia

Answer 27

Functional hyposplenism This therect answer. The question is describing Howell Jolly bodies, which are nuclear remnants normally removed by the spleen – thus their presence is indicative of hyposplenism. Functional hyposplenism caused by splenic autoinfarction is common in sickle cell anaemia. As an additional clue, this patient is also suffering from recurrent URTIs which may be suggestive of underlying immune compromise

Question 28

A 35-year-old woman with a diagnosis of Hodgkin's lymphoma is seen in the haematology clinic. On examination, she has cervical and femoral lymphadenopathy. There is no hepatosplenomegaly and she reports no fever, night sweats, weight loss, abdominal pain or bone pain. She has previously had a chest X-ray showing evidence of mediastinal lymphadenopathy. A positron emission tomography–computed tomography (PET-CT) scan of the thorax, abdomen and pelvis does not reveal any more areas of disease. Her most recent blood tests are shown below: Haemoglobin (Hb) 130 g/L (115–155 g/L) White cell count 7.0 × 109/L (3.0–10.0 × 109/L) Platelets 305 × 109/L (150–400 × 109/L) Which of the following best describes the Ann Arbor stage of this patient's Hodgkin's lymphoma? Stage IIA Stage IVB Stage IIIB Stage IVA Stage IIIA

Answer 28

Stage IIIA Ann Arbor staging is used to classify the extent of disease in Hodgkin's lymphoma. This patient has evidence of cervical, mediastinal and femoral lymphadenopathy. This means she can be classified as having at least stage III lymphoma – she has at least one group of lymph nodes affected above the diaphragm and at least one group below. This patient does not have evidence of stage IV disease as there is no evidence of hepatomegaly, bone marrow involvement (no bone pain, no cytopenias) or other solid organ involvement. As she has no B symptoms, she has a stage IIIA lymphoma, not stage IIIB.

Question 29

Ann Arbor staging

Answer 29

Staging Stage I: involvement of a single nodal group. Stage II: involvement of 2 or more nodal groups, on the same side of the diaphragm. Stage III: involvement of nodal groups on both sides of the diaphragm. Stage IV: disseminated disease with involvement of extra-lymphatic organs (e.g. the bones or lung). Additional staging variables (A) if the patient is asymptomatic or (B) if the patient presents with B symptoms (fever, night sweats, or weight loss). X if there is bulky nodal disease (>10 cm or >1/3 of the intra-thoracic diameter). (S) if there is splenic involvement. (E) if there is extra-nodal disease.

Question 30

A 24 year old lady comes into the GP surgery requesting testing for a genetically inherited disease. Her female cousin has been diagnosed with the disease, her father is known to be a carrier but neither she nor her brother is affected. What is the pattern of inheritance ? X linked Recessive Complex (multifactorial) Inheritance X linked dominant Autosomal Dominant Autosomal Recessive

Answer 30

Autosomal Recessive This means that the individual affected has to have 2 copies of the mutated gene. If an individual has one copy of the mutated gene they will not be affected but are a carrier and can pass on the mutated gene to their children **Why not X linked Recessive?** The father cannot be a carrier in an X-linked condition

Question 31

A 65 year old man present to the GP with ongoing headaches and dizzy spells and new onset tinnitus. He describes being very itchy, particularly after he has a hot bath. On examination, he has facial plethora and splenomegaly. Based on his history and examination findings, what is the most likely diagnosis? Chronic myeloid leukaemia (CML) Haemochromatosis Multiple myeloma Autoimmune haemolytic anaemia (AIHA) Polcythaemia rubra vera

Answer 31

Polcythaemia rubra vera This is correct. The headaches, dizziness and tinnitus are all secondary to the increased viscosity secondary to the proliferation of red blood cells. Itching after a hot bath is also classically associated with polycythaemia vera. Investigations to confirm this diagnosis should include haemoglobin and red cell count which will be raised in polycythaemia rubra vera. Over 95% of people with polycythaemia have a mutation in the JAK2 gene

Question 32

A 10 year old boy presents to a haematology clinic with weakness, fatigue and jaundice after consuming large amounts of fava beans. There is evidence of intravascular haemolysis caused by glucose-6-phosphate dehydrogenase deficiency. Which of the following modes of inheritance is likely to cause this form of haemolytic anaemia? Autosomal recessive Autosomal dominant X-linked recessive Codominance X-linked dominant

Answer 32

X-linked recessive This is the correct answer. Glucose-6-phosphate dehydrogenase is a necessary enzyme required for the generation of reduced glutathione, which protects the red cell from oxidative stress. Lack of this crucial enzyme can lead to haemolytic anaemia. The inheritance of glucose-6-phosphate dehydrogenase deficiency is X-linked recessive and manifests mainly in males

Question 33

A 70 year old man is receiving a blood transfusion for chronic anaemia. Around half an hour into the transfusion he develops dyspnoea and a cough. A chest x-ray is ordered which shows evidence of a 'white out' on his chest xray. There is limited response to IV diuretics. What blood transfusion reaction is most likely to be occurring in this patient? Acute haemolytic reaction Anaphylaxis Transfusion-related acute lung injury (TRALI) Fluid overload Bacterial contamination

Answer 33

Transfusion-related acute lung injury (TRALI) This is correct. TRALI causes respiratory symptoms such as dyspnoea and cough and causes a "white out" on a chest x-ray, as described above. It causes acute respiratory distress syndrome (ARDS) and is usually due to anti-leucocyte antibodies in the donor plasma

Question 34

13-year-old boy presents to the emergency department with fatigue and shortness of breath. He recently had an episode of tonsillitis, which was treated with a course of phenoxymethylpenicillin. His past medical history includes neonatal jaundice, which resolved without treatment. Physical examination shows no abnormalities. His blood tests are shown below: Haemoglobin (Hb) 56 g/L (130–170 g/L) Mean cell volume (MCV) 90 fL (80–96 fL) Reticulocytes 112 × 109/L (25–100 × 109/L) Lactate dehydrogenase (LDH) 300 IU/L (70–250 IU/L) Bilirubin 35 mmol/L (<17 mmol/L) He also has a blood film that shows Heinz bodies and bite cells. Which of the following is the most likely cause of this patient's anaemia? Parvovirus B19 Phenoxymethylpenicillin Fava beans Biliary atresia Hereditary spherocytosis

Answer 34

Fava beans Glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency) is an X-linked genetic disorder characterised by intermittent attacks of haemolysis. There may be a history of neonatal jaundice, typically within the first 24 hours of life. Triggers of G6PD deficiency include fava beans, drugs (nitrofurantoin, ciprofloxacin, sulphonyureas and other sulpha-drugs) and infections. Investigations will show evidence of haemolysis (normocytic anaemia, high reticulocyte count, high LDH etc.) and the blood film may show Heinz bodies and bite cells. In the absence of any predisposing medication, fava beans are the most likely cause in this case.

Question 35

A 60 year old female presents to her GP with fatigue. Some routine blood tests are ordered which shows ↓Hb, ↓neutrophils and ↓platelets, ↓reticulocytes. In secondary care, a bone marrow biopsy is carried out which shows ring sideroblasts. She is given a diagnosis and told that her condition may progress to acute myeloid leukaemia (AML). What is the most likely diagnosis in this patient? Myeloma Myelodysplasia Acute lymphoblastic leukaemia Chronic lymphocytic leukaemia (CLL) Autoimmune haemolytic anaemia (AIHA)

Answer 35

Myelodysplasia Myelodysplasia is considered a "premalignant" disease that affects myeloid cells, which has around a 30% chance of developing into AML. Myelodysplasia can present with bone marrow failure, as shown in the blood results, due to ineffective haematopoeisis. Furthermore, bone marrow biopsy may show ring sideroblasts, as described in this patient

Question 36

A 60 year old female presents to her GP with fatigue. Some routine blood tests are ordered which shows ↓Hb, ↓neutrophils and ↓platelets, ↓reticulocytes. In secondary care, a bone marrow biopsy is carried out which shows ring sideroblasts. She is given a diagnosis and told that her condition may progress to acute myeloid leukaemia (AML). What is the most likely diagnosis in this patient? Myeloma Myelodysplasia Acute lymphoblastic leukaemia Chronic lymphocytic leukaemia (CLL) Autoimmune haemolytic anaemia (AIHA)

Answer 36

Myelodysplasia Myelodysplasia is considered a "premalignant" disease that affects myeloid cells, which has around a 30% chance of developing into AML. Myelodysplasia can present with bone marrow failure, as shown in the blood results, due to ineffective haematopoeisis. Furthermore, bone marrow biopsy may show ring sideroblasts, as described in this patient

Question 37

A 70-year-old male presents to the emergency department with bleeding gums. He has a history of atrial fibrillation and takes warfarin. He is haemodynamically stable. His international normalised ratio (INR) is 9.1. What is the most appropriate treatment? Stop warfarin, give intravenous vitamin K and dried prothrombin complex. Stop warfarin only Stop warfarin, give oral vitamin K Stop warfarin, give intravenous vitamin K and fresh frozen plasma (FFP) Stop warfarin, give intravenous vitamin K

Answer 37

Stop warfarin, give intravenous vitamin K NICE recommends ceasing warfarin and administering intravenous vitamin K for an INR >8 with minor bleeding.

Question 38

A 15-year-old girl presents to the haematology clinic for routine follow-up. She has a past medical history of hereditary spherocytosis, for which she takes folic acid. She reports being fatigued and unable to play sport with her friends, which has persisted since her last appointment. She does not suffer from gallstones. On examination, she has conjunctival pallor and splenomegaly. She thinks that these symptoms are having a major impact on her quality of life and asks if there is any other treatment she can have to help with her symptoms. Haemoglobin (Hb) 78 g/L (115–155 g/L) Mean cell volume (MCV) 84 fL (80–96 fL) Reticulocytes 120 × 109/L (25–100 × 109/L) Iron 20 nmol/L (11–30 nmol/L) Ferritin 145 ng/L (12–200 ng/L) Total iron binding capacity 62 nmol/L (54–75 nmol/L) Which of the following is the best next step in the management of this patient? Salbutamol inhaler Iron supplementation Hydroxycarbamide Splenectomy Avoid fava beans

Answer 38

Splenectomy Hereditary spherocytosis is an inherited haemolytic anaemia, with typical clinical features including a hyperchromic normocytic haemolytic anaemia, jaundice, gallstones and splenomegaly. The management of this condition consists of folate supplementation and splenectomy. The rationale for splenectomy is to reduce the rate of haemolysis and improve red cell survival, therefore improving symptoms. According to the British Society of Haematology's 2011 guidelines on the management of hereditary spherocytosis, splenectomy should be offered to those with moderate-to-severe disease severity eg. gallstones, reduced exercise tolerance. This patient would meet these criteria and therefore should be considered for splenectomy.

Question 39

A 65-year-old man is receiving chemotherapy for a high-grade non-Hodgkin lymphoma. He has a past medical history of hypertension and gout, for which he takes amlodipine and allopurinol. Since starting chemotherapy, he has been prescribed a higher dose of allopurinol compared to what he usually takes. What is the indication for allopurinol here? To treat gout To prevent calcium oxalate renal stones Prescribing error To prevent gout To prevent tumour lysis syndrome

Answer 39

To prevent tumour lysis syndrome Tumour lysis syndrome is characterised by metabolic and electrolyte abnormalities that occur when a large number of cancer cells die within a short period. These abnormalities include hyperkalaemia, hyperphosphataemia and hyperuricaemia. Hyperuricaemia associated with chemotherapy can be prevented by taking allopurinol.

Question 40

A 43 year old male presents to the Emergency Department because he has been feeling tired all the time and because he is unsteady on his feet. This has been progressing over six months. He has a history of alcohol excess. The foundation doctor performs a neurological examination, which shows normal power throughout but impaired vibration and pinprick sensation in his feet, only. His plantars are mute. His blood results show a macrocytic anaemia. What is the most likely diagnosis? Iron deficiency anaemia Thalassaemia Vitamin B1 deficiency Lead poisoning Vitamin B12 deficiency

Answer 40

Vitamin B12 deficiency Sub-acute combined degeneration of the cord is a neurological complication of vitamin B12 deficiency caused by degeneration both the dorsal columns and corticospinal tracts (pain and temperature sensation are typically preserved). It has a wide variety of presentations including distal sensory loss, ataxia, diminished vibration/propprioception sense and variable absent/exaggerated limb reflexes.

Question 41

A 58 year old lady presents to her GP with generalised fatigue and night sweats which is investigated. She is diagnosed with chronic myeloid leukaemia (CML). What is the mainstay of pharmacotherapy for Philadelphia chromosome positive CML? Methotrexate Rituximab Lenalidomide Imatinib Prednisolone

Answer 41

Imatinib This is correct. Imatinib is a BCL-ABR tyrosine kinase inhibitor which works to inhibit the tyrosine kinase activity induced by the Philadelphia chromosome. The Philadelphia chromosome is a hybrid chromosome of chromosome 9 and 22 - t(9;22) - forming the fusion gene BCR/ABL. This fusion gene, through its tyrosine kinase activity, potentiates uncontrolled proliferation of the myeloid cells, leading to CML

Question 42

A 75 year old asymptomatic man is found to be mildly anaemic on routine blood tests. He past medical history includes hypertension, and atrial fibrillation secondary to valvular heart disease (he received a metallic aortic valve replacement 6 years earlier). He currently takes amlodipine, lisinopril, bisoprolol, simvastatin and warfarin. On examination there is an irregular pulse with a loud second heart sound, but nil else of note. First line blood tests demonstrate: normocytic anaemia (106 g/L); normal white cell and platelet counts; normal urea and electrolytes; unconjugated hyperbilirubinaemia (60 mmol/L), but otherwise normal liver function; normal C-reactive protein. Second line blood tests reveal that iron studies and haematinics are normal, but The LDH level is raised. A few reticulocytes are noted on the peripheral blood film. What is the single most likely causes of this patient’s anaemia? Drug-induced anaemia Haemolytic anaemia Acute blood loss Chronic myeloid leukaemia Iron deficiency

Answer 42

Haemolytic anaemia Metallic aortic valves are known to cause non-immune haemolytic anaemia in some cases (probably due to shear stress against the foreign material of the valve)

Question 43

A 2 year old child is investigated due to failure to walk. His knees and hands are swollen and he cries with pain on examination of his joints. An X-ray of his knees shows lipohaemarthrosis. What is the most likely underlying diagnosis? Hereditary Haemochromatosis Juvenile Idiopathic Arthritis Sickle Cell Disease Trauma Haemophilia

Answer 43

Haemophilia Haemophilia often presents as failure to walk in toddlers due to bleeding into joints (lipohaemarthrosis)

Question 44

A 53 year old man with terminal pancreatic cancer is followed up in the outpatient oncology clinic. He complains of multiple episodes of epistaxis in recent weeks. In addition, he has noticed that his blood takes longer to clot following blood tests. On examination, the patient was profoundly jaundiced with a hard mass in the epigastrium. Inspection of a venepuncture site from earlier that day revealed a slow oozing of blood. Point of care testing revealed the patient's INR was elevated. What is the single most likely cause of this patient’s symptoms? Factor V leiden Trousseau syndrome Chemotherapy-related thrombocytopenia von Willebrand’s disease Vitamin K deficiency

Answer 44

Vitamin K deficiency This is the correct answer. This patient’s pancreatic cancer is causing total biliary obstruction, resulting in fat malabsorption and deficiency of the fat-soluble vitamins D, E, A and K. Deficiency in vitamin K may cause this pattern of bleeding, and is associated with a prolonged INR

Question 45

A patient with a previous history of heart attack is prescribed Warfarin to prevent clot formation. Which of the following factors would have their production inhibited by Warfarin? Factors II, VII, IX and X Factors I, IX, XI and XII Factors V, VIII, XI and XII Factors II, VII, X and XII Factors III, IV, VIII and X

Answer 45

Factors II, VII, IX and X This is the correct answer. Factors II, VII, IX and X are all synthesised in the liver and require vitamin K to be activated in vivo. Vitamin K is required for gamma-carboxylation of terminal amino acid residues for these precursors to become functional. Warfarin is an inhibitor of reductase which reduces the levels of vitamin K available to activate factors II, VII, IX and X

Question 46

A 17-year-old girl presents to her general practitioner with a history of mucosal bleeding. She had an episode of prolonged bleeding after a tooth extraction a few weeks ago and has had heavy periods since menarche. She is otherwise fit and well. She also mentions that her father has a history of prolonged bleeding. Which of the following is the most likely diagnosis? Haemophilia A von Willebrand disease (vWD) History of anticoagulant use Haemophilia B Immune thrombocytopenic purpura (ITP)

Answer 46

von Willebrand disease (vWD) vWD is an autosomal dominant condition that typically presents with mucosal bleeding (eg. epistaxis, gum bleeding) and menorrhagia. These patients will typically have a personal history of prolonged bleeding, with a family history of vWD/prolonged bleeding. A personal history of persistent, episodic prolonged bleeding with a positive family history makes vWD the most likely diagnosis.

Question 47

blood test findinfs for von willebrand

Answer 47

Investigation results In VWD there is decreased factor VIII activity, so the PT time is normal but APTT is prolonged (if factor VII activity is <35% of normal). Bleeding time is prolonged.

Question 48

management of von willebrand disease

Answer 48

Von Willebrand disease does not require day to day treatment. Management is required either in response to major bleeding or trauma (to stop bleeding) or in preparation for operations (to prevent bleeding): * Desmopressin can be used to stimulates the release of VWF * VWF can be infused * Factor VIII is often infused along with plasma-derived VWF

Question 49

A 28 year old man presents to his GP complaining of fatigue and breathlessness. He has recently recovered (2-3 weeks ago) from pneumonia, likely caused by mycoplasma pneumoniae. He also reports a recent appearance of red-brown urine discolouration. On examination he is mildly icteric, but otherwise normal with no evidence of organomegaly. Blood tests reveal anaemia (98 g/L), normal white cell counts and platelets, pre-hepatic hyperbilirubinaemia and raised LDH. Which single process best describes this patient's findings? Extravascular haemolysis Autoimmune intravascular haemolysis Non-immune intravascular haemolysis Acute blood loss Bone marrow suppression

Answer 49

Autoimmune intravascular haemolysis This is the correct answer. The patient is exhibiting features of intravascular haemolysis (new onset anaemia, jaundice, haemoglobinuria, absent organomegaly) 2-3 weeks after infection with mycoplasma (known to cause a post-infective cold-agglutinin (IgM) mediated autoimmune haemolysis)

Question 50

A 75-year-old man presents to his GP with a six week history of generalised weakness and fatigue. He also reports recurrent nosebleeds and bleeding gums. He has urgent blood tests which reveal anaemia, thrombocytopenia and leucocytosis. A diagnosis of leukaemia is suspected. Which of the following is a hallmark of acute myeloid leukaemia (AML)? Reed-Sternberg cells Auer rods Philadelphia chromosome Blast cells Rouleaux formation

Answer 50

Auer rods Characteristic blood film findings in AML include Auer rods. Auer rods are needle-shaped structures which are present in the cytoplasm and contain peroxidase.

Question 51

Reed-sternberg cells

Answer 51

Hodgkins lymphoma owl sign

Question 52

A 30-year-old woman with a history of Graves' disease presents to her doctor with tiredness, a sore tongue and a feeling of "pins and needles" in her feet. These symptoms were first noticed one month ago. A full blood count reveals a macrocytic anaemia, along with hyper-segmented neutrophils on her blood film. On further questioning, she says she has a varied diet which includes meat and she drinks alcohol only very occasionally at social events. Considering the most likely diagnosis, which of the following is the most appropriate initial treatment? Ferrous sulfate 200mg twice daily Hydroxocobalamin 1mg on alternate days for two weeks followed by lifelong injections every two months Thiamine 100mg daily Cyanocobalamin 50 micrograms daily Folic acid 5mg daily

Answer 52

Hydroxocobalamin 1mg on alternate days for two weeks followed by lifelong injections every two months This patient's symptoms, megaloblastic macrocytic anaemia and history of another autoimmune condition are suggestive of pernicious anaemia. This is an autoimmune condition which is characterised by vitamin B12 deficiency due to a lack of intrinsic factor secretion by the gastric parietal cells. The treatment is B12 replacement. For patients with neurological symptoms, this is usually with hydroxocobalamin IM injections on alternate days for two weeks, followed by regular long-term injections every two months.

Question 53

A 30-year-old woman with a history of Graves' disease presents to her doctor with tiredness, a sore tongue and a feeling of "pins and needles" in her feet. These symptoms were first noticed one month ago. A full blood count reveals a macrocytic anaemia, along with hyper-segmented neutrophils on her blood film. On further questioning, she says she has a varied diet which includes meat and she drinks alcohol only very occasionally at social events. Considering the most likely diagnosis, which of the following is the most appropriate initial treatment? Ferrous sulfate 200mg twice daily Hydroxocobalamin 1mg on alternate days for two weeks followed by lifelong injections every two months Thiamine 100mg daily Cyanocobalamin 50 micrograms daily Folic acid 5mg daily

Answer 53

Hydroxocobalamin 1mg on alternate days for two weeks followed by lifelong injections every two months This patient's symptoms, megaloblastic macrocytic anaemia and history of another autoimmune condition are suggestive of pernicious anaemia. This is an autoimmune condition which is characterised by vitamin B12 deficiency due to a lack of intrinsic factor secretion by the gastric parietal cells. The treatment is B12 replacement. For patients with neurological symptoms, this is usually with hydroxocobalamin IM injections on alternate days for two weeks, followed by regular long-term injections every two months.

Question 54

A 70 year old male patient presents to the general practitioner for annual review of his diabetes. He is asymptomatic at present and his diabetes is well controlled. The GP orders some routine blood tests and the white cell count is found to be 128 x 10^9/L, with 85% lymphocytes. Which of the following blood film findings is consistent with the most likely diagnosis? Rouleaux formation Smudge cells Auer rods Leukaemic lymphoblastic cells Mature myeloid cells

Answer 54

Smudge cells This is the correct answer. Smudge cells are seen in chronic lymphocytic leukaemia (CLL). In CLL lymphocytes are fragile so may be damaged during the slide preparation, resulting in smudge/smear cells. CLL typically presents in male patients over the age of 60, and is often picked up incidentally as a raised white cell count

Question 55

A 36-year-old man has been diagnosed with rheumatoid arthritis and is being prescribed methotrexate. Which vitamin may be prescribed alongside methotrexate to help to reduce the side effects of this drug? Vitamin B9 (folate) Vitamin B6 (pyridoxine) Vitamin B1 (thiamine) Vitamin B3 (niacin) Vitamin B12 (cobalamin)

Answer 55

Vitamin B9 (folate) Patients who are taking methotrexate may be prescribed folic acid while on treatment. This is because methotrexate inhibits dihydrofolate reductase and may cause folate deficiency.

Question 56

A 65-year-old female presents to her GP with a 1 month history of weight loss and night sweats. She has been generally lethargic and noted early satiety at meal times and abdominal discomfort. On abdominal examination a firm mass in the left upper quadrant extending towards the umbilicus is palpated. Blood results show a WCC 100 X10^9 (4-11) Blood film revealed granulocytes at different stages of differentiation. Given the likely diagnosis what is the most appropriate treatment? All trans retinoic acid (ATRA) Imatinib R-CHOP regimen Daunorubicin VAD Chemotherapy Regimen

Answer 56

Imatinib This patient has massive splenomegaly and a raised white cell count which is suggestive of CML. The Philadelphia chromosome translocation gives rise to the BCR-ABL tyrosine kinase and this can be targeted by Imatinib. This translocation is present in >95% of cases of CML and confers a better prognosis due to the ability to use Imatinib

Question 57

A 40 year old male presents to the GP with a swollen neck, night sweats and weight loss. He has a past medical history of hyperthyroidism, gout, and HIV for which he takes carbimazole, allopurinol, and antiretrovirals, respectively. Several years ago he was involved in a road traffic accident where he received two units of packed red blood cells for treatment of blood loss. His sister has just received a diagnosis of breast cancer. He is investigated and later receives a diagnosis of non-Hodgkin lymphoma. Which of the following predisposed this patient to developing non-Hodgkin's lymphoma? Carbimazole treatment Allopurinol treatment Previous blood transfusion HIV Family history of breast cancer

Answer 57

HIV

Question 58

A 25 year old male patient presents to the general practitioner with a 4 week history of an enlarging left neck mass. He reports the mass is generally painless, but he notices it is sometimes painful after he drinks alcohol. He denies any other symptoms. He has epilepsy and takes carbamazepine. Physical examination reveals non-tender cervical lymphadenopathy. Excisional lymph node biopsy reveals large cells with bilobed nuclei and eosinophilic nucleoli. Which of the following is a risk factor for the most likely diagnosis? African ancestry Low socio-economic class History of Epstein-Barr virus (EBV) infection History of epilepsy Middle age

Answer 58

History of Epstein-Barr virus (EBV) infection

Question 59

A 32 year old woman with transfusion-dependent beta thalassaemia attends haematology outpatients for disease monitoring. On questioning she reports worsening joint pain, most pronounced in the small joints of the hand. On examination the hands are metacarpophalangeal joints subtly swollen bilaterally and the haematologist notices brown hyper-pigmentation of his palmar creases. What is the single best explanation for these findings? Progression of thalassaemia Rheumatoid arthritis Hereditary haemochromatosis Addison’s disease Transfusion-related iron overload

Answer 59

Transfusion-related iron overload This is the correct answer. The joint symptoms (focused on the metacarpophalangeal joints) and hyper-pigmented appearance are typical of iron overload, and the patient’s history of repeated transfusions makes this diagnosis likely. It would be sensible to also check liver function tests, oral glucose tolerance tests, and an ECG/echo; and to measure iron status with blood tests and imaging

Question 60

A 60 year old male presents with weight loss, night sweats and generalised fatigue. He reports a number of infections over the last few months, including a number of upper respiratory tract infections and a urinary tract infection, which he has never had before. On examination, massive hepatosplenomegaly is observed. There are no other significant abnormalities. Blood investigations suggest bone marrow failure. A peripheral blood film is ordered, which is shown below. Blood film report: tear drop poikilocytes (dacrocytes) noted. Which of these myeloproliferative disorders is this patient most likely to be suffering from? Myelofibrosis Essential thrombocythaemia Chronic eosinophilic leukaemia Polycythaemia rubra vera Chronic myeloid leukaemia (CML)

Answer 60

Myelofibrosis This is correct. Myelofibrosis presents with constitutional symptoms as described and is associated with tear drop cells on the peripheral blood film. It occurs when there is abnormal proliferation of fibroblasts which produce platelet-derived growth factor, leading to significant bone marrow fibrosis. This leads to bone marrow failure which causes anaemia, low platelets and low white cell count. The low white cell count may make the patient more susceptible to infections, as suggested by the multiple URTIs and new onset UTI

Question 61

An 80-year-old woman is recovering on the orthogeriatric ward after surgery for a neck of femur fracture. Her past medical history includes rheumatoid arthritis and hypertension, for which she takes methotrexate, folic acid and amlodipine. She has no known drug allergies. Over the past two days she has developed urinary frequency and dysuria. On examination, the patient has suprapubic tenderness but no renal angle tenderness. Her observations are stable and a mid-stream urine sample is sent for analysis. She is started on antibiotics for a urinary tract infection (UTI). Which of the following antibiotics is contraindicated due to the risk of bone marrow suppression? Amoxicillin Gentamicin Nitrofurantoin Fosfomycin Trimethoprim

Answer 61

Trimethoprim Whilst trimethoprim is a common first-line antibiotic for UTIs, methotrexate and trimethoprim are both folate antagonists. They should not be prescribed together due to the increased risk of bone marrow suppression.

Question 62

A 37 year old female patient presents to the emergency department with sudden onset pleuritic chest pain and shortness of breath. The patient has a heart rate of 102 bpm and oxygen saturations of 90% on room air, but physical examination reveals no abnormalities. Chest x-ray is normal. Electrocardiogram (ECG) reveals sinus tachycardia, but is otherwise normal. Arterial blood gas shows: pH = 7.49, pO2 7.6 kPa, pCO2 3.8 kPa, HCO3 23 mol/L. Which of the following is the strongest risk factor for the most likely diagnosis? Factor VIII mutation BMI 26 kg/m^2 Recent history of 2 day bed rest for influenza Open cholecystectomy 4 weeks ago Cigarette smoking

Answer 62

This is the correct answer. The patient presents with clinical features and investigation findings consistent with a pulmonary embolism (PE). Surgery within the last 8 weeks is a strong risk factor for venous thromboembolism (VTE), due to alteration of the 3 components of Virchow's triad (endothelial damage, hyper-coagulability, and blood stasis)

Question 63

Haemophilia A

Answer 63

Inheritance and pathophysiology - Haemophilia A is an X-linked recessive inherited bleeding disorder caused by deficiency in clotting factor VIII – an integral part of the intrinsic component of the coagulation cascade (measured using the APTT). Clinical features - It typically presents early in life with deep and severe bleeding into soft tissues, joints and muscles. Diagnosis - Diagnosis is with a factor VIII assay, and severity depends on the factor VIII level (severe disease occurs if factor VIII is <1% of normal). Management - Minor bleeds can be managed with Desmopressin and major bleeds with recombinant factor VIII.

Question 64

Haemophilia B

Answer 64

Inheritance and pathophysiology - Haemophilia B is an x-linked recessive inherited bleeding disorder caused by deficiency in clotting factor IX – an integral part of the intrinsic component of the coagulation cascade (measured using the APTT). Clinical features - It typically presents early in life with deep and severe bleeding into soft tissues, joints and muscles. Diagnosis - Diagnosis is with a factor IX assay. Management - Major bleeds with recombinant factor IX.

Question 65

A 30 year old female is seen in anticoagulation clinic. She has had 4 episodes of venous thromboembolism (VTE). Two of these episodes occurred during pregnancy. All of her three pregnancies have survived to term. She has a family history of recurrent VTE on her maternal side although not all family members are affected. She is currently on long-term warfarin and her INR is currently in range. What is the most likely cause of her venous thromboembolisms? Anti-thrombin deficiency Pregancy induced venous thromboembolism Combined oral contraceptive pill Anti-phospholipid Syndrome Factor V Leiden/ Activated Protein C resistance

Answer 65

Factor V Leiden/ Activated Protein C resistance This patient clearly has a thrombophilia and Factor V Leiden is the most common cause of inherited thrombophilia. The mutation is carried in about 5% of the UK population. Although it is autosomal dominant it has incomplete penetrance therefore not all those with the mutation are affected which fits with the history provided

Question 66

poor prognostic markers for lymphoma

Answer 66

Poor prognostic markers include: - the presence of B symptoms (weight loss, night sweats, and fever) - lymphadenopathy/organomegaly - advanced tumour stage - advanced age - anaemia - raised serum LDH. Raised LDH is an indirect indicator of the rate of proliferation of the lymphoma

Question 67

Provoked DVT/PE treatment duration

Answer 67

Patients with a provoked DVT/PE should be treated with **3 months of DOAC therapy**. A provoked DVT/PE is a DVT/PE occurring in a patient with antecedent (within 3 months) and major risk factors for a DVT/PE e.g. surgery, trauma, significant immobility, puerperium, or hormonal therapy.

Question 68

Unprovoked DVT/PE treatment duration

Answer 68

Patients with an unprovoked DVT/PE should be treated with 6 months of DOAC therapy. An unprovoked DVT/PE is a DVT/PE occurring in a patient with no antecedent major risk factors for a DVT/PE.

Question 69

DVT/PE in patients with active cancer treatment duration

Answer 69

Patients with an active cancer presenting with a DVT/PE should be offered a DOAC or low-molecular weight heparin (eg. treatment-dose Dalteparin), not warfarin, for 3-6 months.

Question 70

A 60-year-old man presents to his GP with a flushed complexion, headaches and itching which is especially worse after taking hot baths. On examination, he has a palpable mass in the left upper quadrant. The GP arranges urgent blood tests which reveal a raised haemoglobin (195), raised haematocrit (0.64) and a mild thrombophilia (490). Based on the suspected underlying cause, which gene is most likely to have a mutation? BCR ANK1 HBB TET2 JAK2

Answer 70

JAK2

Question 71

A 10-year-old is brought into the emergency department unwell with purpura. Blood abnormalities are as follows: Platelets 67 x109/L (normal 150-400x109/L) Prothrombin time (PT): 20.1 (normal 12.0-14.8s) Activated partial thromboplastin time (APTT): 52.0 s (normal 27.0-41.0s) What is the most likely diagnosis? Acute lymphoblastic leukaemia (ALL) Haemophilia A Disseminated intravascular coagulation (DIC) Thrombotic thrombocytopenic purpura (TTP) Von Willebrand's disease

Answer 71

Disseminated intravascular coagulation (DIC) Prolonged PT and APTT with low platelets point to DIC. Fibrinogen and D-dimer levels should be performed next for scoring of severity.

Question 72

A 40 year old woman presents to the emergency department with vomiting and abdominal pain. She has a past medical history of lymphoma and had her chemotherapy regimen 3 days ago. Her heart rate is 72 beats per minute, blood pressure is 110/80 mmHg, temperature 37.0 degrees Celsius, respiration rate 14 breaths/min and SpO2 99% on air. An ECG done shows: What would be your immediate management? IV fluids Ondansetron Salbutamol nebuliser and insulin-glucose infusion Allopurinol 10% Calcium gluconate

Answer 72

10% Calcium gluconate The ECG shows features of hyperkalaemia, shown by PR prolongation, P wave flattening and peaked T waves. The patient is at risk of a life threatening arrhythmia, hence it is important stabilise the heart with calcium gluconate. Tumour lysis syndrome leads to an increase in serum urate, potassium and phosphate.

Question 73

Allopurinol and tumour lysis syndrome

Answer 73

This is used for prophylaxis of tumour lysis syndrome.

Question 74

A 65-year-old woman with a history of polycythaemia rubra vera presents to her general practitioner with a 6-month history of weight loss, night sweats and early satiety. On examination, she has hepatosplenomegaly and a purpuric rash affecting their legs. She has some blood tests, which are shown below: Haemoglobin (Hb) 100 g/L (115–155 g/L) Mean cell volume (MCV) 92 fL (80–96 fL) White cell count 2.6 × 109/L (3.0–10.0 × 109/L) Platelets 100 × 109/L (150–400 × 109/L) A blood film shows teardrop poikilocytosis. Which of the following investigation results support the most likely diagnosis? JAK2 mutation positive BCR-ABL t(9,22) positive Interferon-gamma release assay positive Reed–Sternberg cells on excisional lymph node biopsy A dry tap on bone marrow aspiration

Answer 74

A dry tap on bone marrow aspiration Myelofibrosis is a haematological condition associated with bone marrow fibrosis. Risk factors include myeloproliferative disorders (eg. polycythaemia rubra vera, essential thrombocytosis), radiation and industrial solvents. Symptoms include constitutional symptoms (weight loss, night sweats, fever), hepatosplenomegaly (which may cause abdominal discomfort and early satiety) and bone marrow failure (causing anaemia, leukopenia and thrombocytopenia). A blood film will typically show teardrop poikilocytosis. A bone marrow aspiration will typically present with a 'dry tap', where bone marrow cannot be aspirated owing to the fibrosis.

Question 75

A 50-year-old man is admitted to hospital with community-acquired pneumonia. He has a CURB-65 score of 3 and is requiring 1L of oxygen to maintain target oxygen saturations. His regular medications have been added to his drug chart and include warfarin 5 mg daily with a target INR of 3.5. The patient's nurse is concerned that this is an error and says most patients have a target INR of 2.5. Which of the following is an indication for a target INR of 3.5? Atrial fibrillation Mechanical mitral valve Antiphospholipid syndrome Provoked pulmonary embolism Provoked deep vein thrombosis

Answer 75

mechanical mitral valve The target INR for patients prescribed warfarin for a mechanical mitral valve is 3.5 due to the high risk of thromboembolic events.

Question 76

target INR for AF

Answer 76

Atrial fibrillation The target INR for patients prescribed warfarin for atrial fibrillation is 2.5.

Question 77

target INR for VTE

Answer 77

2.5

Question 78

A 30 year old woman presents to the GP with a history of menorrhagia that has been ongoing for many years. Clotting studies are ordered. Activated partial thromboplastin time (APTT) was significantly raised, but prothrombin time (PT) and platelet count were both in the normal range. Factor VIII assay was also returned as normal. What is the most likely cause of her menorrhagia? Von Willebrand's disease Vitamin K deficiency Haemophilia A Heparin treatment Disseminated intravascular coagulation (DIC)

Answer 78

Von Willebrand's disease is the most common inherited bleeding disorder and most commonly presents with recurrent epistaxis or menorrhagia in women. It is caused by a deficiency in von Willebrand's factor which binds to factor VIII in the clotting cascade. It causes an isolated rise in APTT (which tests the intrinsic system, which factor VIII/vWF is part of) but has no affect on PT (which tests the extrinsic system). It has no affect on platelets. The level of factor VIII is normal in von Willebrand's disease

Question 79

A 50 year old man presents to the GP with a 6 month history of progressive weight loss, fatigue and night sweats. He also reports abdominal discomfort. On examination, he has splenomegaly and is cachectic but there are no other abnormalities. Blood tests show a raised WCC and anaemia. Cytogenetic analysis of the patient's blood shows that he is positive for the Philadelphia chromosome. What is the most likely diagnosis? Chronic lymphocytic leukaemia (CLL) Chronic myeloid leukaemia (CML) Acute haemolytic anaemia Hodgkin's lymphoma Non-Hodgkin's lymphoma

Answer 79

Chronic myeloid leukaemia (CML) This is correct. CML classically presents with massive splenomegaly and insidious onset of constitutional symptoms such as weight loss, fatigue and night sweats (both of which are red flags for cancer). It is classically associated with the Philadelphia chromosome (present in >80% of those with CML). The Philadelphia chromosome is a hybrid chromosome of chromosome 9 and 22 - t(9;22) - forming the fusion gene BCR/ABL which has tyrosine kinase activity. This can be treated with the specifically targeted drug imatinib

Question 80

A 66-year-old man presents to the emergency department with nausea, vomiting and lethargy. He had a seizure at home lasting 2 minutes and a further seizure in the emergency department. He has a diagnosis of Burkitt lymphoma and received his first dose of chemotherapy 5 days ago. His blood results are shown below: Haemoglobin (Hb) 110 g/L (130–170 g/L) Mean cell volume (MCV) 92 fL (80–96 fL) White cell count 2.5 × 109/L (3.0–10.0 × 109/L) Platelets 110 × 109/L (150–400 × 109/L) Lactate dehydrogenase 400 IU/L (70–250 IU/L) Sodium 142 mmol/L (135–146 mmol/L) Potassium 6.3 mmol/L (3.5–5.3 mmol/L) Urea 13 mmol/L (2.5–7.8 mmol/L) Creatinine 167 mmol/L (60–120 mmol/L) Calcium 1.6 mmol/L (2.2–2.6 mmol/L) Phosphate 1.8 mmol/L (0.8–1.5 mmol/L) Which of the following is the best next step in the management of this patient? Rasburicase Intravenous zolendronic acid Allopurinol Intravenous hydrocortisone Intravenous broad-spectrum intravenous antibiotics

Answer 80

Rasburicase Tumour lysis syndrome is a life-threatening condition associated with the rapid lysis of cancer cells, generally following chemotherapy. It is associated with many malignancies, but high-grade lymphoma and leukaemia pose the highest risk. This condition is characterised by acute kidney injury and electrolyte disturbance (high uric acid, potassium and phosphate; low calcium). Clinically, patients present with nonspecific features (eg. nausea and vomiting, diarrhoea, lethargy), tetany, cardiac arrhythmias and seizures. Rasburicase is a recombinant urate oxidase, meaning it metabolises uric acid to allantoin. It is the first-line treatment for tumour lysis syndrome. Note: the low Hb, white cell count and platelets in this case are likely to be related to either bone marrow infiltration (stage 4 lymphoma) or the patient's recent chemotherapy.

Question 81

A 25 year old woman presents to her GP with recently worsening menorrhagia and recurrent epistaxis. The clinical examination is normal, with the exception of scattered cutaneous purpuric and petechial haemorrhages at sites of mild trauma. A full blood count reveals a raised white cell count with normocytic anaemia (80 g/L) and reduced platelet count (45 x10^9/L). The blood film below shows abnormal white cells: What is the single most likely underlying abnormality? Von Willebrand’s disease Vitamin K deficiency Thrombocytopenia due to immune thrombocytopenia purpura (ITP) Thrombocytopenia due to acute leukaemia Haemophilia A

Answer 81

Thrombocytopenia due to acute leukaemia This is the correct answer. New onset, abnormal bleeding should never be ignored and ad blood count sent promptly. The myelosuppression exhibited here in association with blast cells on the peripheral film strongly indicate acutre leukaemia.

Question 82

A 30 year old woman presents with a history of shortness of breath, fatigue and pale skin. Which of the following would confirm a diagnosis of pernicious anaemia? Vitamin D deficiency Autoantibodies against intrinsic factor (IF) Vitamin C deficiency Impaired RNA synthesis Auto-antibodies against vitamin B12

Answer 82

Autoantibodies against intrinsic factor (IF) This is the correct answer. Typically, pernicious anaemia (a form of megaloblastic anaemia) is caused by autoantibodies against intrinsic factor or gastric parietal cells (secrete intrinsic factor). Intrinsic factor is required to protect vitamin B12 from degradation and aids in the absorption of vitamin B12 at the terminal ileum

Question 83

A 6 year old boy presents to the GP with large bruises on his knees. His mother says that he is very active and enjoys playing outside but his bruises appear to be a lot bigger and last a lot longer than his older sister's do. She also reports that he frequently has spontaneous nose bleeds and has bleeding gums despite good dental hygiene. Haemophilia A is suspected as the cause of his symptoms. Which of the following would confirm this diagnosis? Haemoglobin electrophoresis Blood film Platelet count Factor VIII assay Prothrombin time (PT)

Answer 83

Factor VIII assay This is correct. Haemophilia A is caused by an inherited deficiency in factor VIII. A factor VIII assay would show how much factor VIII is present in the patient's body and hence a reduction in factor VIII will aid diagnosis of Haemophilia A. It also helps to differentiate from Haemophilia B, which is caused by a deficiency in factor IX

Question 84

A 28-year-old female attends the cardiology clinic for a routine follow-up. She suffered from severe infective endocarditis aged 24, requiring mitral valve replacement with a mechanical valve. Routine bloods, including a peripheral blood film, is taken. Which finding would be expected based on her history? Schistocytes Spherocytes Target cells Anisocytosis Howell-Jolly bodies

Answer 84

Schistocytes Schistocytes are fragmented red cells seen in intravascular haemolysis, such as haemolytic anaemia. They are also seen in patients with mechanical heart valves due to mechanical haemolysis of the cells.

Question 85

An 24 year old medical student presents to the emergency department with bloody diarrhoea, 1 week after returning from her elective in Zambia. She was well until 72 hours before admission, when she began to notice cramping abdominal pain associated with watery diarrhoea which has since progressed. On examination she appears pale, confused and dyspnoeic. Scattered purpuric lesion are noted on her limbs. She is resuscitated and initial blood tests are sent revealing evidence of intravascular haemolysis, thrombocytopenia and acute kidney injury. Haematology and infectious diseases input is sought. What is the single most likely causative pathogen? Mycoplasma pneumoniae E. Coli Plasmodium falciparum Babesia species Hepatitis B virus

Answer 85

E. Coli This is the correct answer. This patient is suffering from haemolytic uraemic syndrome. A vasculopathy caused by direct toxin-related injury to the vascular endothelium. The toxin is usually produced by an invasive variant of E. coli (strain 0157:H7) producing shiga-like toxin. Bloody diarrhoeal illness preceding microangiopathic intravascular haemolysis and thrombocytopenia are typical, along with renal and cerebrovascular involvement in severe cases

Question 86

A 52 year old male patient presents to the general practitioner with a 4 week history of weight loss and malaise. He also reports discomfort in the left upper abdomen and abdominal fullness. There is no past medical history of note. He denies any recent travel. On physical examination there is a mass arising from the left upper quadrant, extending to the right iliac fossa. The mass has a palpable notch and a dull percussion note. Which of the following blood film findings is consistent with the most likely diagnosis? Smudge cells Mature myeloid cells Leukaemic lymphoblastic cells Auer rods Rouleaux formation

Answer 86

Mature myeloid cells This is the correct answer. Mature myeloid cells are a feature of chronic myeloid leukaemia (CML). There may also be multiple basophils and eosinophils. CML typically presents in middle-aged patients with massive splenomegaly

Question 87

A 19 year old woman is referred to haematology outpatients with recurrent and persistent nosebleeds and severe bruising. There is a dominant family history of Von Willebrand disease. What is the expected pattern on routine clotting tests? Normal platelets; long bleeding time; normal APTT, PT and TT Low platelets; normal bleeding time; prolonged APTT, normal PT and TT Normal platelets; normal bleeding time; normal APTT, PT and TT Normal platelets; prolonged bleeding time; prolonged PT, normal APTT and TT Normal platelets; prolonged bleeding time; prolonged APTT, normal PT and TT

Answer 87

Normal platelets; prolonged bleeding time; prolonged APTT, normal PT and TT This is the correct answer. Von Willebrand factors transports factor 8 in the plasma, so the intrinsic pathway is compromised in Von Willebrand disease leading to prolongation of the APTT (Note: this may not be seen in very mild cases)

Question 88

A 51-year-old man is seen by his GP after having an Hb A1c of 58 mmol/mol (20–42 mmol/mol) on routine blood testing. He has no polyuria or polydipsia and feels well. His random blood glucose at the GP surgery today was 6.9 mmol/l. Which of the following is the most likely cause of this patient's high Hb A1c? Type 2 diabetes mellitus Vitamin E supplementation Glucose-6-phosphate dehydrogenase (G6PD) deficiency Previous splenectomy Acute blood loss

Answer 88

Previous splenectomy Patients who are post-splenectomy have a higher red cell lifespan, meaning that haemoglobin has more time to become glycosylated. As a result, this may present with a falsely high Hb A1c that is out of keeping with glucose readings. Other causes include iron, B12 and folate deficiency, which is due to prolongation of the red cell lifespan.

Question 89

A 5 year old male patient presents to the emergency department with fever, headache, and vomiting. On physical examination he is pyrexial, with neck stiffness and a petechial skin rash. He is admitted to the intensive care unit but deteriorates, with evidence of blood oozing from the cannula sites, gingival bleeding, and hypotension. Which of the following coagulation profiles is consistent with the most likely diagnosis? Elevated fibrinogen, decreased platelets, elevated D-dimer Decreased fibrinogen, decreased platelets, reduced prothrombin time Decreased fibrinogen, decreased platelets, decreased D-dimer Decreased fibrinogen, elevated platelets, elevated D-dimer Decreased fibrinogen, decreased platelets, elevated D-dimer

Answer 89

Decreased fibrinogen, decreased platelets, elevated D-dimer This is the correct answer. The patient presents with clinical features consistent with disseminated intravascular coagulation (DIC) secondary to meningococcal sepsis. DIC is caused by activation of coagulation pathways, resulting in thrombus formation and consumption of platelets and coagulation factors. Fibrin degradation products (such as D-dimer) will be raised

Question 90

A 46 year old female presents to the emergency department with a painful calf. She is diagnosed with a deep vein thrombosis (DVT). There does not appear to be any risk factors and so this appears to be an unprovoked venous thromboembolism (VTE). She is otherwise well and does not take any medication. She reports that her mother and sister have both had unprovoked pulmonary embolisms in the past. Aside from the DVT, there are no abnormal findings on examination. What is the most likely underlying diagnosis that made her more susceptible to developing the DVT? Factor V Leiden Antiphospholipid syndrome Heart failure Liver disease Von Willebrand's disease

Answer 90

Factor V Leiden This is correct. Factor V Leiden is the most common inherited thrombophilia (present in around 5% of the population) and increases the risk of VTE significantly. It occurs due to an inherited point mutation in factor V, meaning that this factor is not broken down by activated protein C, leading to higher risk of formation of thromboses

Question 91

A 26 year old man with a long-standing history of sickle cell anaemia presents to the emergency department with worsening shortness of breath, wheeze and a new wet cough. On examination he tachypnoeic, de-saturating to 91% on air and has a widespread polyphonic wheeze on auscultation. He had been well until 7 days previously, when he developed a low-grade flu-like illness which has since worsened. He is not on any regular medications. Given the likely cause of this mans symptoms, what is the single best course of action? High-flow oxygen, antibiotics and set up for an urgent exchange transfusion High-dose oral hydroxyurea Back-to-back salbutamol nebulisers (driven with oxygen) and oral prednisolone High-flow oxygen and oral furosemide Antibiotics, with atypical cover, and discharge to GP follow up

Answer 91

High-flow oxygen, antibiotics and set up for an urgent exchange transfusion This is the correct answer. The core triad of acute chest crisis management is high-flow oxygen, antibiotics (with atypical cover) and exchange transfusion. Long-term this patient should be started on hydroxyurea - there is good evidence of benefit in preventing subsequent episodes

Question 92

A 20 year old male presents to his General Practice with persistent cervical lymphadenopathy and fever weight loss and night sweats. He is referred for a biopsy which show Reed-Sterberg cells. On further investigation it is noted that he has inguinal lymphadenopathy that also show Reed-Sterberg cells on biopsy. There is no evidence of liver, spleen or marrow involvement. Given his likely diagnosis what stage is his disease? 2 A 1 B 3 A 3 B 4 B

Answer 92

3 B The Ann-Arbor staging system is important in guiding further management of patients with Hodgkin's Lymphoma. This patient has B symptoms and has nodes present either side of the diaphragm therefore is staged as 3 B( for B symptoms)

Question 93

A 46 year old female is admitted to the gynaecology ward with a history of heavy menstrual bleeding secondary to uterine fibroids. She has no other significant past medical history. A few minutes after a blood transfusion is started the patient complains of abdominal pain, dizziness and feeling generally unwell. On examination her temperature is 38.4 degrees, heart rate 102bpm, blood pressure 86/62 mm/Hg, oxygen saturations 95% on room air and respiratory rate 20 bpm. On examination there is no notable oedema or urticarial rash and her airway is patent. Given the likely diagnosis after stopping the transfusion which of the following is the most appropriate next step in the management of this patient? Intramuscular adrenaline Intravenous fluid resuscitation Oral chlorphenamine Intravenous furosemide Intravenous paracetamol

Answer 93

Intravenous fluid resuscitation This is an acute haemolytic transfusion reaction. It occurs due to ABO incompatibility resulting in IgM mediated intravascular haemolysis. Patients present with features of shock, abdominal and chest pain and feeling generally unwell within minutes of the transfusion starting. The most important management step is to stop the transfusion and then begin fluid resuscitation with intravenous saline. It is also important to send a sample of the patient's blood for direct Coombs test (which will confirm antibodies have bound to the surface of the transfused red blood cells) as well as repeat blood typing and cross-matching. The laboratory should be alerted and a Datix completed

Question 94

A 30 year old female attends the GP surgery complaining of heavy periods and lethargy. Lab results confirm the patient is suffering from iron deficiency anaemia. Which of the following lab results would be expected in an individual with iron deficiency anaemia? High total iron binding capacity (TIBC) with low ferritin 80 < MCV < 100 High total iron binding capacity (TIBC) with high ferritin MCV > 100fl Low total iron binding capacity (TIBC) with normal to high ferritin

Answer 94

High total iron binding capacity (TIBC) with low ferritin Individuals with an MCV greater than 80fl but lower than 100fl will have normocytic anaemia. A reticulocyte count would be conducted to see if the condition is hypo-proliferative or hyper-proliferative

Question 94

A 30 year old female attends the GP surgery complaining of heavy periods and lethargy. Lab results confirm the patient is suffering from iron deficiency anaemia. Which of the following lab results would be expected in an individual with iron deficiency anaemia? High total iron binding capacity (TIBC) with low ferritin 80 < MCV < 100 High total iron binding capacity (TIBC) with high ferritin MCV > 100fl Low total iron binding capacity (TIBC) with normal to high ferritin

Answer 94

High total iron binding capacity (TIBC) with low ferritin Individuals with an MCV greater than 80fl but lower than 100fl will have normocytic anaemia. A reticulocyte count would be conducted to see if the condition is hypo-proliferative or hyper-proliferative

Question 95

A 23-year-old female is receiving a blood transfusion in the respiratory ward. After 10 minutes, she reports that she feels cold. Her observations are as follows: heart rate 70/min, blood pressure 123/81 mmHg, respiratory rate 13/min, oxygen saturations 97% and temperature 38.6ºC. What is the most likely diagnosis? Transfusion-related acute lung injury Transfusion-associated circulatory overload Acute haemolytic transfusion reaction Febrile non-haemolytic transfusion reaction

Answer 95

Febrile non-haemolytic transfusion reaction Febrile non-haemolytic transfusion reaction typically presents with fever (with or without chills and rigors) only, with otherwise normal observations. This is the case with this patient. Typical management would be to stop transfusion, give paracetamol and reintroduce the transfusion at a slower rate

Question 96

A 21-year-old man attends the haematology clinic. He has a history of sickle cell disease, with infrequent sickle cell crises that can be managed at home with hydration and simple analgesia. He takes no regular medications. Which of the following combinations of haemoglobin genes is this patient most likely to have? Hb SS Hb SC Hb SN20 Hb AS Hb AA

Answer 96

Hb SC This is a milder form of sickle cell disease that is caused by the inheritance of one sickle haemoglobin gene and one abnormal C haemoglobin gene. This usually presents as a milder form of sickle cell disease.

Question 97

A 21 year old male patient presents to the emergency department following blunt abdominal trauma following a road traffic accident. CT scan reveals traumatic rupture of the spleen. The patient undergoes emergency splenectomy. The patient has no past medical history of note. The patient is allergic to penicillin. Which of the following long-term management plans is most suitable for this patient? Daily erythromycin, vaccination against pneumococcus (every 5 years), influenza (yearly), HiB, and MenC Daily penicillin V, vaccination against pneumococcus (every 5 years), influenza (yearly), HiB, and MenC (one off) Daily erythromycin, vaccination against influenza (yearly), pneumococcus, HiB, and MenC (one off) Daily penicillin V, vaccination against influenza (yearly), pneumococcus, HiB, and MenC (one off) Daily erythromycin, vaccination against influenza, pneumococcus, HiB, and MenC (one off)

Answer 97

Daily erythromycin, vaccination against pneumococcus (every 5 years), influenza (yearly), HiB, and MenC This is the correct answer. Following splenectomy patients are at increased risk of infection against encapsulated organisms (pneumococcus, meningococcus, and haemophilus). Along with ensuring patients are up to date with routine childDaily erythromycin, vaccination against pneumococcus (every 5 years), influenza (yearly), HiB, and MenC This is the correct answer. Following splenectomy patients are at increased risk of infection against encapsulated organisms (pneumococcus, meningococcus, and haemophilus). Along with ensuring patients are up to date with routine childhood vaccinations, the above long-term management is required. Note that the patient is allergic to penicillin so requires an alternative antibiotic to penicillin Vhood vaccinations, the above long-term management is required. Note that the patient is allergic to penicillin so requires an alternative antibiotic to penicillin V

Question 98

A 28-year-old man presents to the emergency department with a 2-hour history of sudden onset dyspnoea and chest pain. He has not had any recent long-haul flights or surgical procedures. He has no past medical history, although he mentions that his mother and maternal grandmother both had blood clots at a young age. An ECG is performed, which shows a sinus tachycardia. A chest X-ray is performed, which shows no abnormalities. Which of the following is the most likely underlying diagnosis? Factor V Leiden Antithrombin III deficiency Protein C deficiency Marfan syndrome Antiphospholipid syndrome

Answer 98

Factor V Leiden Factor V Leiden (also known as activated protein C resistance) is the most common hereditary thrombophilia, affecting around 1 in 20 people. This patient is presenting with a history suggestive of pulmonary embolism and a significant family history for thromboembolism. As factor V Leiden is the most common hereditary thrombophilia, this is the most likely underlying cause for this patient's suspected pulmonary embolism.

Question 99

A 62 year old female has recurrent deep vein thromboses (DVT) for which she takes rivaroxaban 20mg once a day. Unfortunately she develops a new unilateral leg swelling and ultrasound investigation confirms a further deep vein thrombosis. She has been investigated previously for underlying hypercoagulable conditions and all the tests have been negative. How should you treat this patient? Regular venesection Commence apixaban Insert an inferior vena cava filter Increase her rivaroxaban dose Commence warfarin

Answer 99

Commence warfarin This patient has failed anticoagulation with a direct oral anticoagulant (DOAC). The National Institute for Health Care Excellence (NICE) guidance therefore suggests confirming her adherence to the medication and then either increasing the dosage of the medication or changing for an anticoagulant with a different mode of action. As she is already on the maximum dose of rivaroxaban changing her anticoagulation to warfarin is reasonable

Question 100

A 69-year-old male requires a 3 unit blood transfusion following a road traffic accident. He has no past medical history but appears physically very frail, weighing 49 kilograms with a body mass index of 17. Two hours after the transfusion, he becomes increasingly short of breath and develops an oxygen requirement, needing 2 litres to maintain saturations of 96%. What is the most likely cause of his respiratory symptoms? Persistent anaemia requiring further transfusion Anaphylactic reaction Transfusion associated circulatory overload Transfusion associated sepsis Delayed haemolytic transfusion reaction

Answer 100

Transfusion associated circulatory overload In older, more frail patients, extra care and caution must be taken to avoid fluid overload when giving any infusion. We are used to considering this and adapting our prescriptions accordingly when giving simple fluids, but this can be overlooked when giving blood products. It is important to remember that blood products can have similar effects to fluids, particularly when multiple units are required. You can reduce the risk of transfusion associated circulatory overload by giving the blood products more slowly (i.e. giving 1 unit over 4 or 5 hours, instead of 2 or 3) or by giving a small dose of furosemide between each unit

Question 101

A 62 year old female patient presents to the general practitioner with dizziness and a burning pain in her hands. The patient has no past medical or surgical history of note. On physical examination there is moderate splenomegaly and a dusky dis-colouration of the hands consistent with erythromelalgia. Full blood count reveals a haemoglobin of 13 g/dL, white cell count of 8 x 10^9/L, and platelet count of 500 x 10^9/L. Which of the following investigation findings is consistent with the most likely diagnosis? Peripheral blood film reveals megaloblasts JAK2 V617F mutation positive Serum ferritin 10 nanograms/mL C-reactive protein (CRP) 88 mg/dL Raised red cell mass

Answer 101

JAK2 V617F mutation positive This is the correct answer. The patient presents with clinical features consistent with essential thrombocytosis. JAK2 mutation is present in 50-60% of patients with essential thrombocytosis, and helps distinguish essential thrombocytosis from secondary (reactive) thrombocytosis

Question 102

A 21-year-old male presents to his general practice with a 4-week history of 'tingling' in his hands and fatigue. In addition he complains of falling over at night when walking in the dark. He has no other relevant past medical history however he does admit to regular consumption of alcohol and using nitrous oxide at parties. On general examination conjunctival pallor and areas of hyperpigmentation are noted on the oral mucosa and hands. On neurological examination in the upper limbs there is abnormal light touch, vibration sensation and loss of proprioception. The lower limb neurological examination shows absent reflexes and up going plantars. What is the most likely cause of his symptoms? B12 deficiency Stroke Motor Neuron Disease (MND) Freidrech's Ataxia Alcohol induced peripheral neuropathy

Answer 102

B12 deficiency The patient has a history of fatigue suggesting he is anaemic. The falling over at night refers to his sensory ataxia (a- loss, -taxia control). Recreational use of nitrous oxide (laughing gas) is known to deplete B12 reserves. Neurological examination shows mixed upper and lower motor neuron signs and sensory disturbance consistent with dorsal column pathology which can be caused by B12 deficiency

Question 103

A 65 year old male patient presents to the general practitioner with a 1 month history of malaise, night sweats, and weight loss. On physical examination he appears cachectic and there is conjunctival pallor and marked splenomegaly. Full blood count reveals a white cell count of 5.5 x 10^9/L, a haemoglobin of 7.5 g/dL, platelet count of 88 x 10^9/L, and absolute reticulocyte count of 0.4%. Peripheral blood film reveals poikilocytes. Which of the following investigation findings is consistent with the most likely diagnosis? BCR-ABL polymerase chain reaction positive Bone marrow aspiration reveals 20% blasts and Auer rods 'Dry tap' on attempt of bone marrow aspiration Bone marrow aspiration reveals lymphoblastic infiltration and marrow hypercellularity Serum protein electrophoresis reveals IgG paraprotein spike

Answer 103

'Dry tap' on attempt of bone marrow aspiration This is the correct answer. The patient presents with clinical features consistent with myelofibrosis, a myeloproliferative disorder associated with marrow fibrosis. Because of the marrow fibrosis it is difficult to aspirate the bone marrow, resulting in a 'dry tap'

Question 104

A 71-year-old man presents to the haematology clinic after having some blood tests at his general practice. He has a 3-month history of cervical lymphadenopathy, but feels well and is otherwise asymptomatic. He has no organomegaly. His recent blood tests are shown below: Haemoglobin (Hb) 145 g/L (130–170 g/L) White cell count 26.0 × 109/L (3.0-10.0 × 109/L) Neutrophils 6.3 × 109/L (2.0-7.5 × 109/L) Lymphocytes 11.0 × 109/L (1.5-4.0 × 109/L) Monocytes 0.7 × 109/L (0.2-1.0 × 109/L) Eosinophils 0.3 × 109/L (0-0.4 × 109/L) Basophils 0.08 × 109/L (0-0.1 × 109/L) Platelets 230 × 109/L (150-400 × 109/L) Urea 6.2 mmol/L (2.5-7.8 mmol/L) Creatinine 103 mmol/L (60-120 mmol/L) He also has a blood film showing smudge cells. Which of the following complications is most commonly associated with the most likely diagnosis? Diffuse large B-cell lymphoma Acute lymphoblastic leukaemia (ALL) Burkitt lymphoma Hodgkin's lymphoma Myelofibrosis

Answer 104

Diffuse large B-cell lymphoma The most likely diagnosis in this case is chronic lymphocytic leukaemia (CLL). An elevated white cell count with an absolute lymphocytosis is required for diagnosis; this condition is often diagnosed incidentally in asymptomatic patients or in patients with lymphadenopathy as a presenting complaint. It typically occurs in those aged over 60 years, with a median age at diagnosis of 70 years. Anaemia and thrombocytopenia are late features that confer a poor prognosis. Smudge cells (fragile lymphocytes that are lysed in the preparation of the blood film slide) are pathognomonic for CLL. Diffuse large B-cell lymphoma is a complication of CLL; the conversion of CLL to Non-Hodgkin's lymphoma (most commonly diffuse large B-cell lymphoma) is known as Richter transformation.

Question 105

A 50 year old female presents to the GP for a review. She has a blood test which shows that she has low neutrophils at 0.5x109/L. Her drug history is as follows; Aspirin 75mg PO daily Carbimazole 30mg PO daily Metformin MR 500mg PO daily Simvastatin 20mg PO daily Salbutamol 200micrograms INH as required, up to 4 times daily Which of the above drugs are the most likely culprit for her blood result? Metformin Salbutamol Carbimazole Simvastatin Aspirin

Answer 105

Carbimazole This is correct. Carbimazole is well known for its association with neutropenia and agranulocytosis. For this reason, patients should be advised to report any signs or symptoms indicative of an infection, especially a sore throat

Question 106

A 50 year old female presents to the general practitioner complaining of pain and swelling in her left leg. The patient is has a Body Mass Index (BMI) of 37 and has a 20 pack year smoking history, but is otherwise well. She has recently returned from a trip to Australia. On physical examination the left calf is swollen, with pitting oedema to the mid shins and tenderness along the deep venous system. Venous duplex ultrasound confirms the likely diagnosis. Which of the following is the most appropriate long-term management plan? Apixaban for 3 months Low molecular weight heparin for 3 months Warfarin for 3 months, target INR 3-4 Low molecular weight heparin (LMWH) for 6 months Warfarin for 6 months, target INR 2-3

Answer 106

Apixaban for 3 months This is the correct answer. The patient presents with a 'provoked' distal deep vein thrombosis (DVT), where there is an identifiable risk factor of recent long-haul travel. Therapy is initially for 3 months, with re-evaluation at 3 months for consideration of extended therapy if ongoing risk factors are identified. According to NICE guidelines, first line therapy should be a DOAC such as a Apixaban or Rivaroxaban

Question 107

which anticoagulant preferred in opeople with active cancer with a blood clot

Answer 107

LWMH

Question 108

A 39 year old female is under investigation by Haematology. In the last year she has had a stroke, a deep vein thrombosis and a pulmonary embolism. She has noticed that her gums are bleeding and her periods are heavier. In addition to this she reports headaches and a burning sensation in her hands. Her full blood count shows the following: Haemoglobin: 120g/L (115-165) Mean cell volume: 90 (80-100) White cell count: 5 x10^9/L ( 4-11) Platelets: 1100 x10^9/L (150-450) Given the most likely diagnosis what is the best treatment? Clopidogrel R-CHOP chemotherapy regimen Warfarin Hydroxycarbamide Heparin

Answer 108

Hydroxycarbamide This patient has features consistent with essential thrombocythaemia. The stroke, venous thrombosis and bleeding are due to dysfunctional platelets and erythromelalgia. The treatment for this is hydroxycarbamide to reduce the platelet count

Question 109

A 2 year old Turkish boy presents to his GP. His parents say that he has not been himself. In particular, he seems to be less fit than his peers and appears pale. His height and weight are much lower than expected. On examination he has signs of an enlarged liver and spleen. His parents state that his cousin has experienced similar symptoms. His birth was at term and there were no complication. His immunisations are up to date. What investigation will confirm the diagnosis? Iron studies Blood film Echocardiogram Full blood count Haemoglobin electrophoresis

Answer 109

Haemoglobin electrophoresis This is a presentation of thalassaemia. Typically, patients present younger than the age of two and is more common in those of Mediterranean origin. He has clinical symptoms of anaemia and there is a failure to thrive. Hepatosplenomegaly is also common in these patients. Haemoglobin electrophoresis will show an increase in HbF or HbA2

Question 110

A 43 year old woman with systemic lupus erythematosus is found by their GP to be haemolysing with a haemoglobin concentration of 97 g/L and unconjugated hyperbilirubinaemia of 40 mmol/L. The cause of this haemolysis is investigated by her rheumatologist who sends her for some initial blood tests. There is no social, travel or family history of relevance. The blood film shows spherocytes and reticulocytosis, and no evidence of intracellular parasites or red cell fragments. A direct Coombs test is positive. What is the single most likely causes of this patient’s haemolysis? Hereditary spherocytosis Microangiopathic haemolytic anaemia Anaemia of chronic disease Warm auto-immune haemolytic anaemia Malaria

Answer 110

Warm auto-immune haemolytic anaemia This is the correct answer. SLE is associated with a warm autoimmune haemolytic anaemia. The presence of spherocytes on the blood film and a positive direct Coombs test confirm that this is the case here. Reticulocytosis is consistent with ongoing haemolysis of any cause