Haem

Question 1

What is the most common inherited bleeding disorder?

Answer 1

Von Willebrand’s disease
Autosomal dominant inheritance, reduce platelet adhesion so more bleeding after mild injury

Question 2

What happens to platelet level in von Willebrands disease?

Answer 2

Stays the same
Reduced adhesion of platelets to form platelet plug hence more bleeding, but number of platelets stay the same

Question 3

Presence of Reed Steenberg cells on a blood film is indicative of which condition?

Answer 3

Hodgkin’s lymphoma

Question 4

What a B symptoms?

Answer 4

Fever, weight loss, night sweats
(associated with a poor prognosis for lymphoma)

Question 5

How does multiple myeloma present?

Answer 5

CRAB
HyperCa due to increased osteoclast activity
Renal damage (light chain deposits in renal tubules)
Anaemia (bone marrow crowding suppresses erythropoiesis)
Bony pain (increased osteoclast activity -> lytic bone lesions)

Question 6

What is von Willebrands disease treated with?

Answer 6

Desmopressin (increases release of von Willebrand factor)

Question 7

What is ITP (immune thrombocytopenic purpura)?

Answer 7

Immune mediated drop in platelets often following infection (presents with bleeding, bruising)
Treated with oral steroids

Question 8

Philadelphia chromosome is associated with which malignancy?

Answer 8

CML

Question 9

What is the inheritance pattern of sickle cell anaemia?

Answer 9

Autosomal recessive

Question 10

Hypothyroidism can present with which type of anaemia?

Answer 10

Macrocytic

Question 11

Which clotting factors are deficient in Haemophilia A and B?

Answer 11

Haemophilia A = factor 8 deficiency
Haemophilia B = factor 9 deficiency aka Christmas disease

Question 12

Schistocytes on a blood film are indicative of what?

Answer 12

Haemolytic anaemia

(They are fragmented RBCs seen in HUS, ITP, DIC)

Question 13

Anticogaulation for AF should be considered for CHADs-VASc scores of what?

Answer 13

Males with score >1
Females with score >2

Question 14

What findings on a blood film could be seen in multiple myeloma?

Answer 14

Rouleux formation (stacks of RBCs)

Question 15

What is the antidote for heparin overdose?

Answer 15

Protamine sulphate

Question 16

Which blood product is at most risk of bacterial contamination?

Answer 16

Platelets (as they are stored at room temperature)

Question 17

Normocytic anaemia with Heinz bodies on a blood film is characteristic of what?

Answer 17

G6PD deficiency

Question 18

What is the inheritance pattern of G6PD deficiency?

Answer 18

X-linked recessive

Question 19

What is the inheritance pattern of haemophilia’s?

Answer 19

X linked recessive

Question 20

What parameter is affected in coagulation screen in patients with haemophilia?

Answer 20

Prolonged APTT

Rest is normal

Question 21

What type of anaemia is seen in thalassaemia?

Answer 21

Microcytic anaemia

Question 22

What is the most common type of sickle cell crisis?

Answer 22

Vaso-occlusive crisis
(Triggered by infection/ dehydration/ altitude, sickled cells block capillaries and cause distal ischaemia)

Question 23

What commonly causes a sickle cell aplastic crisis?

Answer 23

Parvovirus B19 infection

(Causes fall in Hb and aplastic anaemia, supportive management as should self resolve in 1 wk)

Question 24

How does an aplastic sickle cell crisis affect reticulocyte count?

Answer 24

Reduces (due to bone marrow suppression)

Whereas sequestration crisis increases reticulocyte number

Question 25

What is the inheritance pattern of sickle cell anaemia?

Answer 25

Autosomal recessive

(Hence one copy of the gene leads to having the sickle cell trait only, which is usually asymptomatic)