Haem

Question 1

ALL:

Answer 1

• Most common in children less than 5 years old, associated with Down’s Syndrome

Question 2

CLL:

Answer 2

Smudge cells, Warm haemolytic anaemia, Richter’s transformation(from CLL to an aggressive high grade b-lymphoma

Question 3

AML:

Answer 3

Auer rods and high number of blast cells, transformation from a Myeloproliferative disorder(eg. polycythaemia vera)

Question 4

CML:

Answer 4

Philadelphia Chromosome, has 3 phase: long chronic(several years), accelerated(blast cells take up proportion of blood cells), blast phase

Question 5

Leukaemia presenting feature:

Answer 5

• Bleeding under the skin due to thrombocytopenia causing non-blanching lesions

Question 6

Leukaemia Investigations:

Answer 6

• FBC within 48 hours
• Bone marrow Biopsy= for definitive diagnosis (Gold standard)

Question 7

Leukaemia general mx:

Answer 7

• Chemotherapy
• Targeted therapy(mainly used in CLL- tyrosine kinase inhibitors eg. ibrutinib and monoclonal antibodies eg. rituximab)

Question 8

Haemophilia: (2 types, inherited X–linked recessive blood disorder therefore mainly affect Males as only have 1 chromosome

Answer 8

A- deficiency in Factor 8

B(Christmas Disease)- deficiency in Factor 9

Question 9

Haemophilia Presentation:

Answer 9

is a severe bleeding disorder; can result in spontaneous bleeding without trauma and bleeding from eg. internal organs, into joints, intracranial haemorrhage

Question 10

Haemophilia Inv:

Answer 10

Bleeding score, coagulation factor assays, genetic testing

Question 11

Haemophilia Mx:

Answer 11

• Give Clotting factors 8/9 depending on type as an IV infusion.

Question 12

Von Willebrand disease:

Answer 12

• Is the most common inherited cause of abnormal and prolonged bleeding
  -deficiency of Von-Willebrand factor(important in platelet adhesion and aggregation)
• 3 types: 1(partial deficiency), 2(reduced function), 3(complete deficiency)
  Mx: Tranexamic acid for mild bleeding, Desmopressin

Question 13

Thrombocytopenia= Low platelet count:

Answer 13

either:
- reduced platelet production
- increased platelet destruction

Question 14

ITP(Immune Thrombocytopenia Purpura):

TTP(Thrombotic Thrombocytopenia Purpura):

Answer 14

ITP: Antibodies are created against platelets, presents with Purpura (non-blanching reasons due to bleeding under the skin). Mx: Prednisolone(steroids), IV immunoglobulin, Rituximab(monoclonal antibody that targets B cells- good for autoimmune related conditions.

TTP:
- Tiny thrombus develop through blood vessels using up platelets. Causes: Thrombocytopenia, purpura, tissue ischaemia/end organ damage
(ADAMTS13 protein),

Question 15

Myelodysplastic syndromes:

Answer 15

• Form of cancer caused by mutation of myeloid cells and has the potential to transform into AML.
  -Presents with: Anaemia, Neutropenia(low neutrophils), Thrombocytopenia-bleeding and purpura (low platelets)
  Inv: Bone marrow biopsy is required to confirm diagnosis

Question 16

Myeloproliferative disorder:

Answer 16

-Uncontrolled proliferation of a single type of blood cell, could transform into AML
- JAK2 mutation
- Main ones to remember(3):
1) Primary Myelofibrosis- low Hb, Mx: JAK2 inhibitor, supportive mx, chemotherapy
2) Polycythaemia Vera- High Hb, gout and thrombosis is a complication of the condition
3) Essential thrombocythaemia High platelet

Blood film: Tear-dropped shaped RBC, Anisocytosis(varying sizes of RBCs), Blasts (immature red and white cells)

Question 17

Polycythaemia Vera (type of myeloproliferative disorder):

Answer 17

• Red face, conjunctival plethora(red insides of eye), splenomegaly, hypertension
• Bone marrow biopsy to confirm diagnosis
  Mx: venesection, aspirin, chemo(hydroxycarbamide)

Question 18

Myeloma:

Question 19

Reversal agent for Dabigratran(anticoagulant):

Answer 19

-Idarucizumab

-Other reversal agent: Protamine to reverse=heparin, Vitamin K=warfarin

Question 20

Sickle cell disease definitive diagnostic inv:

Answer 20

haemoglobin electrophoresis

Question 21

Transfusion reactions presentations:

Answer 21

Transfusion Associated Circularory Overload(TRACO): HYPERtension, raised jugular venous pulse, AFebrile, S3 present.

Transfusion Related Acute Lung Injury(TRALI): HYPOtension, pyrexia(Febrile), normal/unchanged JVP

Question 22

Tumour lysis syndrome reduce risk:

Answer 22

Allopurinol or Rasburicase= to lower uric acid in the blood.

Tumour lysis syndrome: Hyperuricaemia, Hyperkalaemia, Hyperphosphate, Hypocalcaemia

• Burkitt’s Lymphoma(fast growing b-cell Non-Hodgkin’s Lymphoma)= common cause of Tumour Lysis syndrome

Question 23

Post-thrombotic syndrome:

Answer 23

• slow progression of pruritus, pain and swelling are complications that can occur following a DVT.

Question 24

Why are irradiated blood products used in blood transfusions?

Answer 24

• they are depleted in T cell lymphocytes so it reduces the risk of graft vs. host disease

Question 25

Target cells, Howell Jowell bodies seen in what condition/presentation:

Answer 25

- coeliac disease= Hyposplenism

Question 26

Myeloma clinical presentation:

Answer 26

Hypercalcaemia, renal failure, high total protein(could be high proteinuria)

Question 27

Hodgkin's lymphoma staging:

Answer 27

1: single lymph node is involved 2: more than 1 lymph node, same side of diaphragm 3: more than 1, both sides of diaphragm 4: spread beyond lymph nodes

Question 28

Multiple Myeloma presentation: CRAB=

Answer 28

C- HyperCalcaemia R- Renal failure A- Anaemia B- Bone fractures/lytic lesions

Question 29

Sickle cell crisis: Painful (vaso occlusive: diagnostic Inv=

Answer 29

- Clinical diagnosis and no additional testing is required.

Question 30

Non-haemolytic febrile transfusion reaction:

Answer 30

Mx: Paracetamol can be used to reduce the pyrexia(fever), also need to stop the transfusion.

Question 31

Heinz bodies are seen in what condition?

Answer 31

- G6PD deficiency - Alpha Thalassaemia

Question 32

Target cells(Bull's eye spot):

Answer 32

- Iron deficiency anaemia - Post-Splenectomy

Question 33

Howell-Jolly bodies:

Answer 33

- Non-functioning spleen -severe anaemia

Question 34

Smudge cells (ruptured WBC) :

Answer 34

- Associated with CLL

Question 35

Spherocytes: (are sphere shaped RBC without bi-concave disk)

Answer 35

- Autoimmune Haemolytic Anaemia or -Hereditary Spherocytosis

Question 36

Anaemia:

Answer 36

- Is defined as low haemoglobin in the blood - MCV= means size of blood cells and is significant in relation to anaemia

Question 37

MCV levels in anaemia and type of anaemia correlation: - Low MCV - Normal MCV - High MCV

Answer 37

- Low MCV: Microcytic Anaemia - Normal MCV: Normocytic Anaemia - High MCV: Macrocytic Anaemia

Question 38

Causes of Microcytic Anaemia (TAILS mnemonic):

Answer 38

- T (Thalassaemia) - A (Anaemia of Chronic disease) - I (Iron deficiency Anaemia) - L (Lead Poisoning) - S (Sideroblastic Anaemia- body produces enough iron but is unable to put it into haemoglobin)

Question 39

Causes of Normocytic Anaemia (3 A's and 2 H's):

Answer 39

- Acute Blood loss - Anaemia of chronic disease (often occurs with CKD due to reduced production of erythropoietin) - Aplastic Anaemia - Haemolytic Anaemia - Hypothyroidism

Question 40

Causes of Megaloblastic Anaemia:

Answer 40

- B12 Deficiency - Folate Deficiency

Question 41

Generic symptoms and signs of Anaemia:

Answer 41

Symptoms: - Tiredness - SOB - Headaches - Dizziness - Palpitations - Worsening of other conditions eg. angina/heart failure Signs: - Pale Skin - Conjunctival Pallor - Tachycardia - Raised Respiratory Rate

Question 42

Symptoms and signs specific to Iron-deficiency anaemia:

Answer 42

- PICA (dietary cravings for abnormal things eg. metal, dirt, soil) - Hair Loss Signs: - Koilonychia (spoon shaped nails) - Angular Cheilitis -Atrophic Glossitis -Brittle hair and nails -

Question 43

what should you do for UNEXPLAINED iron-deficiency anaemia?

Answer 43

- OGD or Colonoscopy= to exclude GI cause of bleeding - Bone marrow biopsy also= to exclude unknown malignancy(leukaemia/myeloma)

Question 44

Main Haem conditions:

Answer 44

Iron deficiency (Microcytic Anaemia) Vitamin B12 deficiency (Macrocytic Anaemia) Folate deficiency (Macrocytic Anaemia) Haemophilia (Bleeding Disorder) Deep vein thrombosis Pulmonary embolism Complications of blood transfusion

Question 45

Myeloma: (including causes of Myeloma- CRAB mnemonic)

Answer 45

- Type of cancer effecting plasma cells in bone marrow, causes high amounts of M protein (M for Myeloma) - Presence of Bence Jones Proteins in: Urine protein electrophoresis - CRAB: Calcium (elevated), Renal failure, Anaemia, Bone lesions and bone pain - Most common complication= Anaemia -Other conditions that can be caused=Myeloma bone disease, Renal disease - Bone marrow biopsy= is required to confirm diagnosis - X-ray findings= Raindrop skull/pepper pot skull: refers to multiple lytic lesions seen on x-ray - Mx: will never be fully cured; disease is relapsing-remitting. Treatment: Chemotherapy/High-dose chemotherapy

Question 46

Lymphoma key symptom:

Answer 46

- Lymphadenopathy

Question 47

Hodgkin's Lymphoma:

Answer 47

- May experience lymph node pain after drinking alcohol + - B symptoms= Fever, weight loss and night sweats - Reed-Sternberg cells= key finding in biopsy - Diagnostic investigation: Lymph Node Biopsy - Additional non-specific symptoms: Fatigue, itching, cough,SOB, Abdominal pain, Recurrent infections Management: - Hodgkin's Lymphoma= Chemotherapy and Radiotherapy

Question 48

4 stages of Lymphoma (Lugano Classification):

Answer 48

Stage 1: Confined to ONE node/group of nodes Stage 2: More than group, but on the SAME SIDE of the diaphragm Stage 3: Lymph nodes ABOVE and BELOW diaphragm. Stage 4: Widespread involvement, including non-lymphatic organs.

Question 49

Management for: TACO (Transfusion Associated Circulatory Overload)=

Answer 49

- Should be treated with IV loop Diuretics - Eg. presentation= Patient had blood transfusion and then started getting SOB/reaction to the medication/blood given. - Is also indicated by crackles at the bilateral lung bases.