Haematology

Question 1

Post thrombotic Syndrome?

Answer 1

Following a DVT, venous outflow obstruction and venous insufficiency result in chronic venous hypertension. Tx is compression stockings

• painful, heavy calves
• pruritus
• swelling
• varicose veins
• venous ulceration

Question 2

most common inherited bleeding disorder

Answer 2

Von WIllibrand Disease. (VWF usually promotes platelet adhesion to damaged endothelium)

-AD inheritance and can appear normal on blood tests

Question 3

most common hereditary haemolytic anaemia? what genetic inheritance predisposes that?

Answer 3

Hereditary Spherocytosis: autosomal dominant defect of red blood cell cytoskeleton

Question 4

In hereditary spherocytosis what changes can you see in the RBC? What consequences on the lifespan of the RBC does this have?

Answer 4

• Normal biconcave disc shape is replaced by a sphere-shaped red blood cell
• Increased destruction of RBC by spleen

Question 5

Presentation of Hereditary Spherocytosis

Answer 5

• failure to thrive
• jaundice, gallstones
• splenomegaly
• aplastic crisis precipitated by parvovirus infection
• degree of haemolysis variable
• MCHC elevated

Question 6

Diagnosis and management of Hereditary Spherocytosis?

Answer 6

Dx: osmotic fragility test

Management: folate replacement and spleenectomy

Question 7

G6PD presentation, who does it occur in

Answer 7

• Neonatal jaundice
• Infection/drugs precipitate haemolysis
• Gallstones

Male (X-linked recessive)

Question 8

What would you see on blood films in G6PD deficiency?

Answer 8

Heinz Bodies

Question 9

What are the 3 main NOACs to be aware of?

Answer 9

1) Dabigatran (Pradaxa)
2) Rivaroxaban
3) Apixaban

Question 10

1) Dabigatran (Pradaxa)
2) Rivaroxaban
3) Apixaban

What are these, what do they target and how are they excreted?

Answer 10

1) Dabigatran (Pradaxa): direct thrombin inhibitor, excr via Renal
2) Rivaroxaban: Direct Xa inhibitor. excr via liver
3) Apixaban: Direct Xa inhibitor, excr via faecal

Question 11

triad of joint pain, spiking fevers, and a pink bumpy rash is very characteristic of …..

Answer 11

Still’s Disease

rare systemic autoinflammatory disease. RF and ANA will be negative

Question 12

The most common causes of inherited thrombophilia are?

Answer 12

• factor V Leiden (activated protein C resistance): most common cause of thrombophilia
• prothrombin gene mutation: second most common cause

Thrombophilia is due to a def in natural anticoags (protein C/S and antithrombin III)

Question 13

Difference between aplastic crisis and sequestration crisis?

Answer 13

Aplastic Crisis: Lowered retic and HB (often precipitated by exposure to parvovirus B19)

Sequestration Crisis: increased retic and lowered Hb (due to sickling within organs/blood pooling)

Question 14

What is Churg Strauss Syndrome??

Answer 14

small-medium vessel vasculitis (ANCA mediated)