Haem Practice Q Flashcards

Question

Monoclonal Gammopathy of Undetermined Significance (MGUS) is a condition which carries a risk of developing into which condition? A. Acute Myeloid Leukaemia B. Chronic Lymphocytic leukaemia C. Myeloma/Multiple Myeloma D. Burkitt’s Lymphoma E. Sickle Cell anaemia

Answer 1

C- Myeloma/multiple myeloma MGUS is a precursor condition which has a 1% chance per year of developing into myeloma. It causes no end organ damage and has no symptoms. It is characterised by overproduction of a monoclonal immunoglobulin, generally found incidentally on blood tests. It has no relation to any of the other conditions.

Answer 2

D- Raised white cell count In Hodgkin’s Lymphoma white cells are not typically raised. It is counter intuitive that the WCC is not raised and it can be easy to be caught out. Pruritis can occasionally be the only presenting symptom in Hodgkin’s lymphoma. Reed-Sternberg cells are diagnostic if found on a blood film.

Answer 3

E- Multiple myeloma Myeloma symptoms can be remembered by the acronym CRAB – Calcium (raised due to bone resorption), Renal impairment (creatinine high), Anaemia and Bone (osteoporosis). The above history and investigations are indicative in derangement of all of these. In addition, Bence jones proteins in the urine and rouleaux formation in the blood are characteristic of Myeloma. While advanced Bronchial Ca could have metastasised into various organs to produce the spread of symptoms and investigative results we have here, it would be very advanced and the patient would be significantly more unwell with a very severe cough likely with haemoptysis. Acute lymphoblastic leukaemia presents typically with an anaemia/infection/bleeding picture and tends to develop more rapidly and do not typically cause renal impairment or calcium derangement until later in their disease course. CML tends to be much slower in bone erosion, and more typically causes fatigue, sweats and potentially gout over a chronic course. Follicular lymphoma is also a very slow developing (indolent) cancer which does not fit the presented symptoms or investigations.

Answer 4

D- Weight loss Weight loss (D) is not a known complication or feature of PCV. PCV is a condition in which the bone marrow over produces blood cells caused in 95% of cases by a JAK2 mutation. Dizziness (A) occurs because the blood is overly viscous causing various CNS abnormalities. Itching (B) occurs because the abnormal numbers of RBC’s stimulate histamine. Haemorrhage (C) can occur due to defective platelet function.

Answer 5

E- Tay-sachs disease You almost certainly will not know what Tay-Sachs disease is and you’re not meant to. It is a very rare neuro-degenerative disease of children. The point is that you should know that all of the other options are major risk factors for DVT – immobility, trauma and surgery because they all impair the body’s ability to move the blood from the legs back to the heart and malignancy because of cell wall damage in the blood vessels.

Answer 6

B- Heiz Bodies Heinz bodies are seen in G6PD deficiency not in IDA. The RBC’s will be pale due to insufficient haemoglobin and small for the same reason. They will also form in abnormal shapes as the normal structure requires a set amount of haemoglobin. The cells will vary wildly in size depending on the store of iron in the bone marrow which created them

Answer 7

A- AIDS infection Neutrophilia is a high neutrophil count. This often occurs in response to infection, inflammation or trauma. In contrast, neutropenia is a low neutrophil count and is often a result of immunosuppression by particular drugs and cancers. Despite AIDS (A) being an infection, it targets the immune system and can cause life-threatening neutropenia. In contrast, despite CML (C) being a cancer, it causes proliferation of neutrophils (as well as other cells such as basophils and eosinophils) and so is a cause of neutrophilia. Appendicitis (B), MI (D) and Strep pyogenes (E) infections all increase neutrophil count as the body responds to the acute event.

Answer 8

C- Hodgkin’s Lymphoma The Reed Sternberg cell is an abnormal lymphocyte which is classic of Hodgkin’s lymphoma. Other buzz words for haematological malignancies include the Philadelphia chromosome in CML and the Bence Jones Protein in Myeloma.

Answer 9

E- Vitamin B12 injections Pauline is likely suffering from Pernicious Anaemia, an autoimmune condition that prevent the uptake of vitamin B12/folate in the bowel. It is most commonly diagnosed in women around the age of 60. As well as ‘classic’ anaemia symptoms, pernicious anaemia can also cause peripheral neuropathy (hence the pins and needles), lemon tinged skin (due to a mix of mild jaundice and pallor), mouth ulcers, depression and dementia. Vitamin B12 injections can replace that which the body cannot absorb. Blood transfusions (A) and chemotherapy (B) are more commonly used in sickle cell anaemia Ferrous sulphate (C) is used in iron deficiency anaemia. Venesection is not a treatment for anaemia but for polycythaemia (D).

Answer 10

D- Iron deficiency Iron deficiency anaemia is a hypochromic, microcytic anaemia. The other options: B12/folate deficiency (A), bone marrow failure (B), hypothyroidism (C) and liver disease (E) all cause macrocytic anaemia.

Answer 11

A- Acute Lymphoblastic Leukaemia (ALL) ALL is more commonly found in children and is associated with Down’s syndrome (note the facies listed in the question- small chin, slanted eyes). It usually presents with anaemia, bleeding and infection alongside hepatosplenomegaly and peripheral lymphadenopathy. Lucy is suffering from two additional complications: CNS involvement causing facial nerve palsy (drooping), and Superior Vena Cava obstruction causing red face and dilated chest veins. These complications are rare but point towards a diagnosis of ALL.

Answer 12

D- Refer urgently for colonoscopy Mary is displaying red flag symptoms for bowel cancer, a concerning cause of iron deficiency anaemia in post-menopausal women and all men. An urgent (2 week wait) colonoscopy (D) is needed to rule out malignancy. Ferrous sulphate (A) and dietary advice (B) is appropriate management for pre-menopausal women with iron deficiency anaemia and no red flags. A referral to gynaecology (C) is appropriate for women with anaemia and unresponsive menorrhagia or post-menopausal bleeding.

Answer 13

D- Factor 9 Haemophilia B is an inherited condition in which Factor 9 is deficient, causing easy bruising and bleeding. It is less common that Haemophilia A, in which Factor 8 is deficient (memory trick: A comes before B, and 8 comes before 9, so A=8 and B=9)

Answer 14

D- Secondary polycythaemia Polycythaemia is an increase in RBC production. Primary polycythaemia (rubra vera) (C) is an issue with RBC overproduction, whereas secondary polycythaemia has an underlying cause, commonly chronic hypoxia as a result of lung disease, smoking, or low FiO2. In this case, we know Bill has a significant smoking history and COPD, making secondary polycythaemia more likely than primary. DIC (A) is a condition where many small clots form throughout the body, causing bleeding as clotting factors are used up. There is usually a distinct trigger such as sepsis, major surgery or trauma, making this less likely in Bill’s situation.

Answer 15

E- Parvovirus B19 Parvovirus B19 causes Fifth disease, a usually mild rash illness. However, in people with sickle cell, it can precipitate a painful crisis. Anti-malarial medications (A) containing quinine and green beans (B) are known to precipitate crises in people with G6PD. Both the Hepatitis B (C) and influencza vaccine (D) are recommended by NICE for people with sickle cell disease due to an increased risk of complications from infections and the increased likelihood of receiving a blood transfusion in their lifetime.

Answer 16

B- Calcium gluconate Robert has multiple myeloma causing back pain, anaemia, AKI and hypercalcaemia of malignancy. The presentation of myeloma can be remembered using CRAB – calcium, renal impairment, anaemia, bone lesions. Calcium gluconate (B) is the treatment for hypocalcaemia and so is unlikely to be prescribed in this context. Myeloma is incurable but often managed with a combination of chemotherapy, steroids (C) and thalidomide (E). Supplementary treatments include bisphosphonates (A) and radiotherapy (D) to address bony pain.

Answer 17

D) Folate deficiency Folate deficiency is a cause of macrocytic megaloblastic anaemia.

Answer 18

B) B12 binds to intrinsic factor produced by the parietal cells of the stomach it is then absorbed in the terminal ileum In pernicious anaemia (most common cause of vitamin B12 deficiency) the parietal cells of the stomach are attacked by the immune system, resulting in atrophic gastritis and the loss of intrinsic factor production

Answer 19

D) Splenectomy Management: * Relieves symptoms due to anaemia or splenomegaly, reverses growth failure and prevents recurrent gallstones * Best to postpone until after childhood due to infection risk post-op * After operation give appropriate immunisation and life-long penicillin prophylaxis

Answer 20

A) Large and small irregular hypochromic RBCs Beta thalassaemia is an example of a haemolytic anaemia. RBCs can either be normocytic or if there are many young RBCs (reticulocytes – which are larger) due to excessive destruction of old RBCs then macrocytic. Sickled erythrocytes (B) are seen in sickle cell anaemia, another type of haemolytic anaemia. Oval macrocytes (C) would be seen on the blood film of a patient who has megaloblastic. anaemia i.e. B12 or folate deficiency. Blast cells (D) are seen in acute lymphoblastic leukaemia. Plasmodium falciparum (E) is one of the causative organisms of malaria

Answer 21

C) Multiple Myeloma Multiple myeloma presents in adults aged over 70. The most prominent features on presentation of multiple myeloma can be remembered using CRAB. There are also non-specific presentations of tiredness/malaise or infection. C – calcium over 2.75mmol/L R – Renal impairment A – Anaemia B – Bone lesions -> pepper pot skull, cord compression, back pain The blood results confirm this diagnosis, the low Hb shows anaemia, WCC and platelets remain normal, high creatinine suggests renal impairment and high calcium CLL is a leukaemia affecting the B cells, it is the most common leukaemia normally affecting adults > 70-years-old. Often, those presenting with CLL are asymptomatic or have enlarged, rubbery nontender nodes – not the symptoms described in the question. As for the blood results, CLL causes a high WCC with high lymphocytes and a blood film would show small, mature lymphocytes. The blood results do not confirm this diagnosis as the WCC is not raised. AML tends to affect those aged over 40-years. It presents with anaemia, bleeding and infection. The blood results in a patient in AML would show anaemia, thrombocytopenia and neutropenia. On a bone marrow biopsy, you would expect to see Auer Rods to confirm a diagnosis. The blood results do not confirm this diagnosis as the patient has normal platelet levels not thrombocytopenia Hodgkin’s Lymphoma has a bimodal incidence affecting young adults and the elderly. The most common presentation of HL is fevers, sweating, enlarged rubbery non-tender nodes and systemic B symptoms (loss of appetite/night sweats). You would expect to see anaemia on the blood results however the remaining blood results do not fit with this diagnosis. Non-Hodgkin’s Lymphoma affects adults aged over 40 years. The presentation of NHL is similar to HL except B symptoms are less common and there may be some GI and skin involvement.

Answer 22

B) Rouleaux formation Rouleaux formation is seen on the blood film of patients with multiple myeloma You would expect to find Leukaemic blast cells on the blood film of a patient with acute lymphoblastic leukaemia (ALL) Auer Rods are seen on a bone marrow biopsy of someone with Acute Myeloid Leukaemia (AML) The Reed-Sternberg cell is a characteristic cell of Hodgkin’s Lymphoma Raised myeloid cells would be seen in chronic myeloid leukaemia (CML)

Answer 23

E) Plasmodium falciparum – causes the most severe form of malaria and has the highest rate of mortality. This species is mainly found in Africa, it is the most common type of malarial parasite. P. vivax can also cause severe disease and kill people; however, it contributes much less to the global burden of malaria than P. falciparum. Mainly found in Asia and South America. This species can stay in the liver for up to 3 years therefore there is the potential for relapses. P. ovale – this is very uncommon and usually is found in West Africa. This species can remain in your liver for several years without producing symptoms P malariae – this species is quite rare and usually only found in Africa P. knowlesi – this species is very rare and found in parts of southeast Asia.

Answer 24

A) Uncomplicated malaria – the key feature in this question was the feeling ‘warm and cold’ which represents the fever causing sweats and chills in an individual with malaria. Questions with fever and recent travel – THINK could it be malaria! TB – These symptoms this lady presented with are not typical of TB. A persistent cough, weight loss, night sweats and a fever are the symptoms expected in a TB infection Complicated malaria is characterised by vascular occlusion which can affect different organs therefore causing specific symptoms e.g., cerebral malaria leads to micro-infarcts and therefore symptoms include drowsiness, increased intracerebral pressure causing seizures and coma. Food poisoning – this would cause similar symptoms to uncomplicated malaria however the key in this question is the recent travel! Cholera is caused by the bacteria Vibrio cholerae and this releases a toxin which increases water release from intestinal cells resulting in severe diarrhoea. Infection occurs through ingestion of contaminated food or water.

Answer 25

D) Stage III B This patient’s lymphoma has spread to nodal areas on both sides of the diaphragm so it is Stage III. She also presents with a fever so has systemic symptoms, therefore her stage would include the suffix B. Stage II describes a cancer that is one a single side of the diaphragm, and stage IV has spread beyond the lymph nodes

Answer 26

B) Acute Lymphoblastic Leukaemia ALL is your paediatric cancer. It is most common in 2-4 year olds and can present with recurrent infection and general unwellness. You might also see hepatosplenomegaly, SVC obstruction and back pain (bone marrow failure) however these are not always present. AML and CML are seen in adults over 40, and CLL is seen in adults over 70.

Answer 27

C) Imatinib Imatinib is a tyrosine kinase inhibitor, which is given in CML alongside chemotherapy. Ibrutinib is a bruton kinase inhibitor, and is given in CLL. Rituximab is a monoclonal antibody given in CLL, Dexamethasone is a steroid, which is part of the treatment therapy in multiple myeloma.

Answer 28

A) Alpha thalassaemia is incompatible with life Alpha thalassaemia can be incompatible with life, however it is only the 4 gene deletion sub-group that this applies to . The 3 gene deletion and 2 gene deletion groups will survive. Beta thalassaemia minor patients do not require life-long transfusions, and it is diagnosed with a blood film. Alpha thalassaemia is diagnosed with haemoglobin electrophoresis, and results in gene deletion of one or both alpha chains.

Answer 29

B) Thrombotic thrombocytopenic purpura TTP is the answer – the deficiency in ADAMTS13 enzyme causes the vWF to form thromboses. To compensate, the body will break down these clots down to prevent ischaemic damage to organs hence haemolytic anaemia. Raised bilirubin levels. ITP affects platelet aggregation. Antibodies attach the GpIIB/IIIa receptors preventing platelet aggregation Haemophilia A and B are deficiencies in factors 8 and 9, respectively.

Answer 30

C) High altitude There are 2 types of secondary polycythaemia – compensatory and abnormal In hypoxic conditions, kidneys release high levels of EPO and hence more RBC are produced In abnormal conditions, it could be a tumour secreting EPO, GGWP Option A and B are causes of relative polycythaemia Option D is a cause of primary absolute polycythaemia

Answer 31

D) Lymphoma Myeloproliferative disorders are conditions affecting the myeloid pathway. Lymphomas affect the lymphoid pathway

Answer 32

D) Diameter of the blood vessel

Answer 33

Iron deficiency anaemia caused by colorectal cancer Blood results – low Hb (anaemia) low WCC (cancer), low MCV (microcytic anaemia) low ferritin (confirms iron deficiency anaemia although this could be raised in malignancy)

Answer 34

Easy bruising, epistaxis, menorrhagia, purpura (purple spots on the skin caused by bleeding underneath the skin), gum bleeding

Answer 35

1 in 4 // 25%

Answer 36

Brittle skin/nails, koilonychia, subconjunctival pallor, atrophic glossitis, angular stomatitis.

Answer 37

The signs you would expect include anaemia, jaundice, hepatosplenomegaly and ‘Black Water Fever’. Black water fever is a complication of malaria causing haemolysis of RBCs which results in Hb being released directly into the urine.

Answer 38

P. ovale and P. vivax can form hypnozoites in the liver which can lie dormant for years and cause relapses. The medication primaquine can be given to eliminate these – however be careful as this can cause haemolysis in those with G6PD. Primaquine is also contraindicated in pregnancy and breastfeeding.

Answer 39

Monoclonal protein in serum or urine Lytic bone lesions/ CRAB end organ damage Excess plasma cells in bone marrow

Answer 40

a reciprocal translocation between chromosome 11 and 14. An abnormality in chromosome 13q is associated with treatment resistance and poorer prognosis

Answer 41

a) Acute myeloid leukaemia b) Auer rods c) Down Syndrome Radiation d) Blood transfusion Allopurinol (to prevent tumour lysis) IV antibiotics Chemotherapy Steroids Bone marrow transplant

Answer 42

a) 70+ b) Enlarged, rubbery, non-tender lymph nodes Sweating, anorexia Commonly asymptomatic c) Smudge cells d) Chemotherapy Monoclonal antibodies (rituximab) Bruton kinase inhibitors (ibrutinib) e) Richter’s syndrome – transformation of CLL to an aggressive lymphoma

Answer 43

a) FBC – anaemia, high ESR CXR – wide mediastinum Blood film – Reed-Sternberg cells b) Hodgkin lymphoma c) Chemotherapy ABVD treatment Marrow transplant

Answer 44

a) It protects the RBCs against oxidative damage b) Fatigue, palpitations, shortness of breath, pallor c) Bite cells, reticulocytes d) Nitrofurantoin

Answer 45

a) DVT b) pain, pallor, perishingly cold, pulselessness, paralysis, paraesthesia c) PE, post-thrombotic syndrome, chronic venous insufficiency d) D-dimer (if positive then follow with Doppler US which is the gold standard) e) Well’s score f) clinical signs and symptoms of DVT no alternative diagnosis HR > 100 beats/min immobilisation > 3 days or surgery

Answer 46

a) Disseminated intravascular coagulation b) Tissue damage (from ARDS) will cause release and activation of tissue factor. This leads to widespread clot formation and the consumption of platelets and coagulation factors (thrombosis formation).A lso, the Tissue Plasminogen Activator is activated leading to increased fibrinolysis hence clotting is removed but increased risk of bleeding. DIC is a consumptive coagulopathy. Tissue Factor Release causes coagulation from both intrinsic & extrinsic pathways ➔ THROMBOSIS Thrombus are broken down by Fibrinolysis ➔ BLEEDING

Answer 47

Give: Fluids, Broad spectrum Abx, Administer O2 if required Take: Bloods, Urine Output, Lactate levels

Answer 48

Nitrofurantoin or trimethoprim

Answer 49

a) EPO levels b) Polycythaemia ruba vera c) Acute dehydration, chronic obesity/HTN/alcohol excess

Answer 50

Platelet count – level of platelets. A normal platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. Having more than 450,000 platelets is a condition called thrombocytosis; having less than 150,000 is known as thrombocytopenia. Bleeding time - Bleeding time is a laboratory test to assess platelet function and the body’s ability to form a clot. The test involves making a puncture wound in a superficial area of the skin and monitoring the time needed for bleeding to stop (ie, bleeding site turns "glassy"). PT – The prothrombin time is a measure of the integrity of the extrinsic and final common pathways of the coagulation cascade. This consists of tissue factor and factors VII, II (prothrombin), V, X, and fibrinogen. The test is performed by adding calcium and thromboplastin, an activator of the extrinsic pathway, to the blood sample then measuring the time (in seconds) required for fibrin clot formation. APTT – a measure of the functionality of the intrinsic and common pathways of the coagulation cascade. Thrombin time – Thrombin time is a screening coagulation test designed to assess fibrin formation from fibrinogen in plasma. The reference range for the thrombin time is usually less than 20 seconds Fibrin degradation products - Fibrin and fibrinogen-degradation product (FDP) testing is commonly used to diagnose disseminated intravascular coagulation (DIC). d-dimer – D-dimer is the degradation product of crosslinked (by factor XIII) fibrin. It reflects ongoing activation of the hemostatic system. The reference concentration of D-dimer is < 250 ng/mL, or < 0.4 mcg/mL.

Answer 51

Platelet Count – normal Bleeding Time – normal Partial Thromboplastin Time (PT) – normal Activated partial Thromboplastin Time (APTT) – increased Thrombin Time (TT) – normal or increased Fibrin Degradation Products – normal D-Dimer – normal

Answer 52

Deep bruising, pain and swelling in joints, epistaxis, other unexplained bleeding, bleeds into heads can cause headaches/double vision/weakness/vomiting

Answer 53

Replace the missing factor with plasma

Answer 54

C. Ferrous sulphate is the first line treatment for iron deficiency anaemia. This lady suffers from menorrhagia, which can cause this. A. Ferrous gluconate can be used to treat iron deficiency anaemia however is only given if the side effects of ferrous sulphate are too much for the patient. B. This is the treatment for normocytic anaemia, or anaemia of chronic disease. D. This is the treatment for B12 deficiency anaemia. E. This is the treatment for B12 deficiency anaemia.

Answer 55

D. G6PD deficiency is more common amongst males from African or SE Asian ethnicities. Nitrofurantoin is contra-indicated in this condition, and can cause a drug-induced oxidative crisis. This presents with symptoms commonly associated with anaemia, i.e. headaches. A. Iron deficiency anaemia would explain the blood result, however bite cells are not seen with this condition. B. An allergic reaction would normally present with a rash and itching in/around face/tongue/throat in addition to the breathlessness. C. Tuberculosis typically presents with a cough lasting more than 3 weeks, weight loss and night sweats. E. A sickle cell crisis would likely present with pain as well as breathlessness and anaemia.

Answer 56

E. A doppler ultrasound would be able to confirm a DVT, and therefore should be ordered in the event of a suspect one. Especially for a patient such as this one, who is elderly, has just undergone orthopaedic surgery, and is immobile. A. A D-dimer can be useful to exclude a DVT, however a positive D-dimer is not diagnostic so this test would not be able to confirm anything. B. An X-ray would not be useful for assessing vasculature. C. A CT is not indicated for DVT diagnosis. D. An FBC is not indicated for DVT diagnosis.

Answer 57

A. CKD is not a cause of thrombocytopenia\ B. Viral infection, such as HIV and TB, can result in thrombocytopenia C. Toxins, including alcohol, can result in a reduction in platelet production, and thus thrombocytopenia D. Myeloma (and other cancers such as leukaemia and lymphoma) can result in a reduction in platelet production, and thus thrombocytopenia E. Heparin can increase the destruction of platelets, leading to drug-induced thrombocytopenia

Answer 58

D. ALL is the most common paediatric cancer, therefore is the most likely diagnosis for this child. A. CML is most commonly seen between the ages of 40-60. B. CLL is more common in the elderly. C. AML is the most common adult leukaemia. E. The peak age for myeloma is 70+ (OLD CRAB)

Answer 59

B. Hb electrophoresis will confirm the diagnosis of sickle cell anaemia. A. A FBC will show a Hb in the range of 60-80 g/L and a raised reticulocyte count. These indicate sickle cell, however cannot diagnose it. C. A blood film is more commonly used to see infection, including malaria (thin blood film). D. Blood cultures would be used to identify an infection. E. There is a diagnostic test so this is incorrect.

Answer 60

E. Imatinib is a tyrosine kinase inhibitor and is the treatment for CML A. Adriamycin is part of the VAD treatment for myeloma, not CML. B. Allopurinol is prophylactic gout treatment, and can also be given to combat tumour lysis in ALL. C. Rituximab is the monoclonal antibody treatment for Non-Hodgkin's Lymphoma D. Vincristine is part of the VAD treatment for myeloma, not CML.

Answer 61

C. Stage IIb - 2 or more nodal areas on the same side of the diaphragm, with systemic symptoms A. Stage Ia - 1 nodal area with no systemic symptoms (e.g. weight loss, fever, night sweats) B. Stage IIa - 2 or more nodal areas on the same side of the diaphragm, with no systemic symptoms D. Stage IIIa - Involvement of nodal areas on both sides of the diaphragm, with no systemic symptoms E. Stage IIIb - Involvement of nodal areas on both sides of the diaphragm, with systemic symptoms

Answer 62

A. Spherocytosis is caused by a defect in the red cell membrane, which causes them to lose part of their membrane as they pass through the spleen. This abnormality is then associated with an increased permeability to sodium ions B. This statement says a ‘decreased permeability’ which is incorrect C. This statement says potassium rather than sodium D. This statement says potassium rather than sodium E. The haemoglobin is not affected in spherocytosis, only the shape

Answer 63

B. Sporozoites are the infective stage. These are transferred from mosquito to human through a mosquito bite. A. Oocysts sit in the gut wall of the anopheles mosquito, and contain sporozite, however they are not infective until they rupture C. Sporozoites are transported to the liver of their human host, and mature into schizonts. These then rupture in turn, releasing merozoites. D. Hypnozoites are the dormant stage of P. vivax and P. ovale. These 2 parasites can lay dormant in the liver for years before causing any symptoms. E. Trophozoites are found after the merozoites infect the red blood cells of its host.

Answer 64

This child is presenting with Acute Lymphoblastic Leukaemia. D. ‘Blast cells’ is the correct answer - these are present in Acute Lymphocytic Leukaemia, which is the most likely diagnosis in this case. ALL is the most common cancer of childhood. A. Auer Rods are characteristic of Acute Myeloid Leukaemia - AML is uncommon under the age of 45 - if it is an acute leukaemia picture in an adult, AML is likely the answer. B. Smudge cells are present in Chronic Lymphocytic Leukaemia; Chronic Leukaemias tend to affect adults, not children. C. RBCs in Rouleaux Formation are present in Multiple Myeloma; this tends to affect older men and women, rather than children. E. Sickled erythrocytes are characteristic of - you guessed it - sickle cell anaemia.

Answer 65

C. Chronic myeloid leukemia is associated with Philadelphia Chromosome A. Hodgkin's lymphoma would typically be seen in ages 15-35 and then less frequently in Px in their 70s. Although it would present w/ systemic features you would also expect (especially in SBA land) a ‘rubbery unilaterally enlarged lymph node’ B. Myeloma is a good differential for this case given the patient's age and symptoms of fatigue and weight loss. However it is not associated w/ philadelphia chromosome and there was no mention of hypercalcemia or renal impairment making it less likely than CML D. AML typically has less insidious or quicker onset of symptoms with evidence of pancytopenia and is associated which chromosome 5 and 7 deletions rather than philadelphia chromosome E. ALL typically effects children aged 2-10

Answer 66

C. Correct answer, by inhibition of tyrosine kinase you stop the excessive cellular replication of eosinophils, neutrophils and basophils A. This is the mechanism for EPO B. This is essentially the function/fault of the philadelphia chromosome, which is the underlying cause of CML D. A decrease in Leukocyte alkaline phosphatase is a clinical finding of CML E. This is essentially nonsense, CP450 inhibitors are drugs which inhibit CP450 enzymes in the liver which increases the time taken for certain drugs to be metabolised and therefore increases their effects

Answer 67

B. HypER rather than hypOcalcemia is seen in myeloma A. Anaemia is a classic finding in myeloma C. Raised creatinine indicates renal impairment, which is seen in myeloma D. Raised urea also suggests renal impairment, which is seen in myeloma E. This is a textbook finding that is part of the diagnostic criteria for myeloma

Answer 68

A. This is seen in myeloma B. Seen in CLL C. Seen in myelofibrosis D. Seen in hyposplenism E. Seen in G6PD deficiency

Answer 69

C. Right answer as DVT was unprovoked/no cause identified A. Correct duration in provoked DVT (this was unprovoked/no cause identified in the stem) B. Just wrong D. Would be a bit dramatic at this stage, some Px do end up on life long anticoag for repeat DVTs (e.g. in antiphospholipid syndrome) but in this case there is nil PMHx E. again, just wrong

Answer 70

D. Correct, back pain in elderly patients (especially in the presence of red flags such as thoracic level and waking up in the night) should always raise suspicion of myeloma. He is also anaemic and has symptoms of hypercalcemia which further suggests myeloma. A. ALL effects children aged 2-10, this is a 68 year old man B. Good differential, especially given the anaemia but would not explain the thirst, constipation or confusion (which is secondary to hypercalcemia) or the back pain (lytic lesions) so is not the best answer C. Would maybe explain the confusion but would be a very atypical presentation, does not explain the anaemia or other parts of the Hx E. Unusual for IBS to present for the first time in late 60s and multiple red flags in the Hx suggesting an underlying cause

Answer 71

D. Correct answer A. For this to be true, only one parent could be a carrier (or both parents non-carriers w/out the disease) B. For this to be true, both parents would have to have the disease C. This is the chance of AR disease being inherited if both parents are carriers E. This is the chance of AR disease not being inherited if both parents are carriers

Answer 72

C. Correct A. Typically seen in IDA B. Typically seen in blood loss and chronic disease D. Not associated alcohol intake E. Emergency needing senior review

Answer 73

D. Correct, a WCC of > 20 at time of diagnosis is associated with a poorer prognosis in ALL A. Poor prognosis in males not females B. < 2 or > 10 at time of diagnosis indicates poorer prognosis C. Non-caucasion has a poorer prognosis in ALL than being caucasion E. Hepatosplenomegaly is a clinical feature of ALL but does not influence prognosis

Answer 74

B. HTN is not caused by any of the drugs used in ABVD A. Infertility is a classic long term side effect of ABVD in SBA land although probably quite rare in clinical practice C. Doxorubicin (the A in ABVD, but literally do not worry about individual names, just knowing the regime is ABVD is probs enough) can cause cardiomyopathy D. Vinblastine can affect the nervous system E. Bleomycin can cause lung damage

Answer 75

B. MGUS - This is the correct answer. Doris has Multiple Myeloma - a cancer of the plasma cells that results in proliferation of these cells in the bone marrow, ‘crowding out’ normal blood cells. In terms of symptoms and signs, think ‘OLD CRAB’ - Myeloma often doesn’t present until it is quite advanced, so is often diagnosed in older people. ‘C’ stands for (raised) calcium, and it is mentioned that Doris has hypercalcaemia in the stem. ‘R’ stands for renal impairment, which Doris does not have symptoms of, but Renal involvement is mentioned in that she has recently had Kidney Stones (a symptom of the hypercalcaemia). ‘A’ stands for anaemia, and if you were to do an FBC on Doris, she would likely be anaemic, and ‘B’ stands for bony lytic lesions, which present as bone pain, usually in the back or ribs, sometimes in the hips. Doris does not necessarily have the MGUS gene, but this is the gene that is associated with a proportion of cases of myeloma. A. CFTR - Cystic Fibrosis Transmembrane conductance Regulator gene is defective in (you guessed it), Cystic Fibrosis. Popped in here as it’s a gene you’ll recognise. It is found on Chromosome 7. E. Trisomy 21, commonly known as Down’s syndrome, is associated with leukaemia (AML and ALL).

Answer 76

B. Acute Myeloid Leukaemia is the correct answer – it is the most common acute leukaemia in adulthood, and presents with pallor and fatigue (anaemia), easy bruising/bleeding (thrombocytopenia), breathlessness, frequent infections (neutropenia), lymphadenopathy, and sometimes bone/joint pain. Auer rods on a blood film are the distinguishing feature here. Generally, with an adult with an acute leukaemia picture, AML is a good guess (ALL is more common in children). A. ITP is an immune-mediated disorder characterised by very low levels of platelets. It is often asymptomatic, but can present with petechiae, multiple bruises, and excessive bleeding from the gums or nose. Jenny has systemic features, though, so it is unlikely ITP. C. Acute Lymphoblastic Leukaemia is the most common childhood cancer (and hence, the most common blood cancer of childhood). Its incidence peaks between 2 and 5 years of age. D. Chronic Myeloid Leukaemia presents similarly to AML, but with a more chronic picture (months/years, rather than days/weeks as in this case). Patients with CML will often have lost weight as this is chronic, and they can lose their appetite due to a feeling of fullness. If a question is looking for you to think about CML, it might mention the Philadelphia Chromosome, as this is a gene abnormality associated with more than 90% of patients with CML. E. Sideroblastic Anaemia is a type of anaemia in which the body has enough iron, but is unable to turn it into haemoglobin; so the iron accumulates in the mitochondria of the blood cells, causing ‘ringed sideroblasts’ to be formed. These stain with blue on a blood smear, with Prussian blue staining. Symptoms include weakness, fatigue, shortness of breath, and signs may include hepatosplenomegaly due to iron build-up, or abnormal heart rhythms. It is a rare disorder - so don’t worry if it's not your strong suit

Answer 77

D. Diet rich in meat and dairy – though not necessarily the healthiest for you, meat and dairy are rich in vitamin B12, so a high intake of this can help to improve symptoms of B12 deficiency. Poultry, shellfish and eggs are also high in B12 – but don’t worry fellow veggies, you can also get B12 in plenty of fortified soy / nut milks, and nutritional yeast! A. Sideroblastic anaemia is a type of anaemia that is caused by the body’s inability to make enough healthy blood cells due to a vitamin B12 deficiency. B12 is required for the normal production of red blood cells, as well as for the normal myelination and functioning of the central nervous system – so can present a bit weirdly: along with the classic symptoms of anaemia (fatigue, weakness, pallor, weight loss), a patient with pernicious anaemia may experience peripheral neuropathy, memory loss, confusion, constipation and nausea/vomiting. B. Intrinsic factor is required for the normal absorption of vitamin B12 in the terminal ileum; this is a substance produced by the parietal cells of the stomach. Who ever said GI isn’t relevant to haematology, eh? C. Coeliac disease, if untreated and the patient still consumes gluten, can result in damage to the small intestine, where B12 is absorbed. This can result in deficiencies in iron, folate, or B12. E. Crohn’s disease is a gastrointestinal disorder that causes transmural granulomatous inflammation anywhere within the GI tract. If Crohn’s affects the terminal ileum, this can prevent adequate B12 absorption.

Answer 78

B. This bimodal age incidence (2-5, and >50) is characteristic of acute lymphoblastic leukaemia; the peak incidence of CML begins around 60-65 years of age. Epidemiology and patient demographics can be super helpful in forming your differential diagnosis, especially in topics like haem where a lot of things present similarly. A. Philadelphia Chromosome is a gene abnormality involving Chromosomes 9 and 22, caused by a translocation resulting in an abnormally small chromosome 22. It is associated with >90% of cases of CML (as well as a small number of ALL cases) C. Fatigue, weight loss and night sweats are common symptoms of CML (and most blood cancers!) D. Bone pain can be a feature of CML, due to bone marrow expansion due to the accumulation of the cancer cells. E. Easy bleeding and bruising can be a feature of CML due to thrombocytopaenia (remember, in CML there is proliferation of abnormal myeloid cells, so there may not be enough room / nutrients to produce sufficient normal platelets.

Answer 79

C. Reed-Sternberg Cells is the correct answer – this patient has Hodgkin Lymphoma (the hint here is that the lump is painful after consuming alcohol) – but Non-Hodgkin’s is a good differential diagnosis (and even recognising this as lymphoma is good!) Reed-Sternberg cells have a characteristic ‘owl’s eye’ appearance on a blood smear. A. Auer rods are present in AML (and some other myeloproliferative disorders) B. Haemosiderin staining occurs when erythrocytes are broken down: haem iron + biliverdin, and the iron is stored as haemosiderin. This can cause areas of skin hyperpigmentation and can accumulate in tissues such as the liver or kidney. It’s not too important but a bit of niche knowledge is never a bad thing! D. Heinz Bodies are essentially inclusions of denatured haemoglobin found in red blood cells. It can be seen in G6PD deficiency (which causes haemolytic anaemia), thalassaemia – haematological disorders that cause damage to haemoglobin, essentially. Again, not at all high-yield but a good bit of niche knowledge. E. Kayser-Fleischer rings are actually found in the eyes (the surrounding the iris) and are characteristic of Wilson’s disease (where excess copper is stored in various tissues including liver, brain and corneas).

Answer 80

C. Factor VIII – this is the correct answer; Haemophilia A is a genetic disorder inherited via an X-linked recessive pattern (therefore, it is more common in males, as they only need one copy of the gene whereas females need 2 copies of the faulty gene). It is a deficiency in factor 8. A. Factor VII deficiency is not present in Haemophilia A. B. Vitamin K deficiency causes a deficiency in the Vitamin K-dependent clotting factors; 2, 7, 9 and 10. D. Factor V Leiden is a mutation of one of the clotting factors in the blood, resulting in an increased risk of developing blood clots. It is the most common inherited form of thrombophilia. E. Factor IX (9) deficiency is characteristic of Haemophilia B, not A!

Answer 81

A. This is the correct answer; this child has beta-thalassaemia major (characterised by jaundice – the dark urine), slow growth (she is small), chipmunk facies (due to hyperproliferation of bone marrow leading to facial bone deformities), swollen abdomen and fatigue/weakness. This is caused by a lack of production of beta-globin – therefore HbA cannot be produced (HbA = 2 alpha chains, 2 beta chains). As she is only 5 months old, she will still have some HbF (HbF = 2 alpha chains, 2 gamma chains) – but her symptoms begin around this age as the HbF will be replaced. Her HbA2 levels will be increased to compensate for the lack of HbA (HbA2 = 2 alpha chains, 2 delta chains). B. HbA cannot be increased due to the lack of production of beta-globin chains. C. As above; HbA cannot be created in beta-thalassaemia major; beta-thalassaemia minor tends to be asymptomatic, and intermedia (where the beta-globin is present but faulty) is somewhere in between the two presentations). D. Delta-globin and gamma-globin are unaffected; and are used to create HbF and HbA2 to compensate for the lack of HbA E. There is definitely not a decreased beta-globin in Beta-thalassaemia, by definition!

Answer 82

D. Iron-deficiency causes microcytic anaemia – this is the correct answer. Other causes of microcytic anaemia include anaemia of chronic disease (which can be both macro or microcytic), hypothyroidism (which can cause macro or microcytic), sideroblastic anaemia amongst other conditions. A. Folate deficiency causes macrocytic anaemia B. Alcohol abuse causes macrocytic anaemia C. B12 Deficiency causes macrocytic anaemia E. Fanconi anaemia causes an aplastic anaemia; it is a rare genetic disease resulting in an impaired response to DNA damage – it is a type of bone marrow failure resulting in a lack of production of sufficient functional blood cells.

Answer 83

B. Sickle cell anaemia is a type of haemolytic anaemia which presents with low haemoglobin, a high reticulocyte count and patients often present with acute pain in their hands and feet A. Iron deficiency anaemia presents with low haemoglobin and a ow reticulocyte count C. B12 deficiency presents with low haemoglobin and a low reticulocyte count D. Folate deficiency presents with low haemoglobin and a low reticulocyte count E. Patients with Crohns have low haemaglobin and a low reticulocyte count

Answer 84

E. JAK2 gene - A mutation in the JAK2 gene is responsible for polycythaemia rubra vera A. B globin gene mutations occur in sickle cell anaemia B. I just made the QR8 gene up C. EPO mutations occur in primary familial and congenital polycythaemia D. The philadelphia CHROMOSOME mutation is associated with Chronic Myeloid Leukaemia

Answer 85

A. Being underweight is not a risk factor for DVTs. However, obesity is a risk factor B. Long haul travel is a risk factor for DVTs C. Oral contraceptive pill use is a risk factor for DVTs D. Pregnancy is a risk factor for DVTs E. Immobility following a leg fracture is a risk factor for DVTs

Answer 86

D. Epstein-Barr Virus This patient has Hodgkin’s lymphoma as they have a painless lump in their neck that feels rubbery and they have B symptoms such as weight loss and night sweats. The blood film also confirms the presence of Reed Sternberg cells. Previous infection with EBV has been shown to have an association to developing Hodgkin’s Lymphoma

Answer 87

B. Haemolytic Uraemic Syndrome – this is the correct answer. E. Coli O157 produces Shiga-toxin, which can cause endothelial damage to the blood vessels, damaging nearby red blood cells and platelets, leading to thrombocytopenia and haemolytic anaemia. HUS presents with symptoms of thrombocytopenia (easy bleeding and bruising), anaemia (tiredness, weakness) and kidney failure (blockage of vessels within the kidney due to blood clots, resulting in decreased kidney function). A. Tumour lysis syndrome is a group of metabolic abnormalities (hyperuricaemia, hyperkalaemia, hyperphosphataemia and hypocalcaemia) that occur just before, or up to a week after beginning chemotherapy. It is caused by the rapid break down of cancer cells, resulting in the release of intracellular contents into the bloodstream, which is what causes the electrolyte abnormalities. C. Felty’s syndrome is a rare complication of rheumatoid arthritis, which is a triad of rheumatoid arthritis, neutropenia and splenomegaly. D. Enterocolitis is inflammation of both the small and large intestine – this wouldn’t explain the changes in kidney function or the symptoms of thrombocytopenia and haemolysis that Jeff is experiencing. E. ITP is a disorder of blood clotting in which the blood has an unusually low level of platelets. It would explain the bruises, but not the other symptoms. The cause is unknown (hence ‘idiopathic’)

Answer 88

C. Auer rods Bone marrow infiltration and failure will lead to: > Bruising / bleeding - thrombocytopenia > Infections - leukopenia > Breathless, fatigue, pale - Anaemia (pancytopenia if all 3 are present) Bite cells ==== Seen in G6PD Smudge cells ==== Seen in ALL Auer rods ==== Seen in AML Spherocytes ==== Seen in HS (hereditary spherocytosis) Tear drop cells ==== Seen in myelofibrosis DS is most commonly associated with AML, however there is also a higher prevalence of ALL in patients with DS. Age points more to AML.

Answer 89

FBC > ↓Hb > ↑MCV (macrocytic anaemia) Blood film > Hypersegmented neutrophils (>5 lobes) and presence of oval macrocytes Serum cobalamin ↓

Answer 90

A. Most common cause of B12 deficiency anaemia is pernicious anaemia. Vitamin B12 binds to intrinsic factor B. 7 years vegan - Stores of B12 are sufficient to last 2-5 years (so very short history of dietary deficiency would not cause significant B12 deficiency) C. Test for antibodies: parietal cell and intrinsic factor (specific) antibodies Vitamin B12 deficiency can result in subacute combined degeneration of the spinal cord. Can present with sensory deficits, paraesthesia, weakness, ataxia and gait disturbance. Learn the difference between specific and sensitive. Important in conditions such as pernicious anaemia/rheumatoid arthritis. N.B. If someone had folate and B12 deficiency, correct B12 first.

Answer 91

B. B lymphocytes Around 80% of NHL is of B cell origin, the remaining 20% are of T cells. Normal B cells mature in bone marrow. It is upon subsequent differentiation in secondary lymphoid tissues (lymph nodes and spleen). Thought during subsequent differentiation they undergo malignant transformation. Large B cell lymphoma is the most common. Hodgkin’s lymphoma is also a B-cell malignancy. It is the type of lymphoma where you see Reed-Sternberg cells.

Answer 92

C. Vitamin K Warfarin is a competitive inhibitor of vitamin K epoxide reductase complex 1 which is required for activating vitamin K. Giving vitamin K is the reversal agent for warfarin. Normal INR range is 2.0-3.0, if it is too high, the blood doesn’t clot properly and you get bleeding e.g. epistaxis (nose bleeds), gums bleeding, bruising for no reason, coffee ground vomit. If INR is too low you get clotting.

Answer 93

X, IX, VII, II → 1972 are vitamin K dependent clotting factors

Answer 94

B. Numbness around the mouth Myeloma = Old CRAB Hypercalcaemia - Groans (constipation), stones, bones, moans (psychic) Renal injury - AKI (high creatinine, high urea, low eGFR) Anaemia - breathless, fatigue (not reported here). Bone destruction - pathological fractures (vertebral most common). Symptoms of hypocalcaemia = CATs go numb Convulsions, arrhythmias, tetany and numbness. Also Chvostek’s and Trousseau’s signs

Answer 95

D. Signs of sickle cell usually manifest within 1-2 weeks of birth Blood test findings in haemolytic anaemia: normocytic, ↑LDH ↑unconjugated bilirubin ↓haptoglobin (particularly with intravascular haemolysis) ↑reticulocyte count. HbA affected in sickle cell: β-globin chain misshapen. Normally glutamic acid but in sickle cell it is valine. Results in HbS- when deoxygenated it sickles- aggregates with other HbS and the RBC sickles. Fetal Hb (2α + 2γ globin chains) → primary Hb at birth so no symptoms until a few months old.

Answer 96

E. G6PD Polycythaemia, or an increased number of red blood cells can occur via two mechanisms: Primary: JAK2 mutation = polycythaemia vera Secondary: Alcohol Chronic hypoxia Obstructive sleep apnoea Living at altitude Lung disease affecting oxygen transfer Smoking EPO secreting tumours G6PD is a hereditary enzyme defect. As far as i can tell there is no association with polycythaemia (more likely causes anaemia).

Answer 97

B. Haemolytic uraemic syndrome Haemolytic uraemic syndrome→ triad of microangiopathic haemolytic anaemia, thrombocytopenia and renal impairment. Most are diarrhoea-associated (caused by Shiga-toxin producing E.coli) in children Blood test results show: FBC: anaemia and thrombocytopenia Haemolysis: LDH, bilirubin Peripheral blood smear: schistocytes U&E: raised creatinine and electrolyte abnormalities Management is mainly supportive

Answer 98

D. Oral hydroxychloroquine He’s got quite mild malaria. Think of this when you have a traveller presenting with fevery illness and signs of haemolytic anaemia (jaundice, dark urine). Diagnosed on Giemsa stained thin/thick blood films Mild is treated with oral (hydroxy)chloroquine Severe is treated with IV artesunate. + Primaquine, notify to PHE.

Answer 99

A. Microcytic hypochromic RBCs, anisocytosis (variation in size of RBCs), poikilocytosis (irregular shaped RBCs) B. Sideroblastic anaemia, anaemia of chronic disease, thalassaemia C. Koilonychia (spoon shaped nails), angular stomatitis, atrophic glossitis, pallor

Haem Practice Q Flashcards

(124 cards)