Platelet Problems

Question 1

What’s the most common cause of thrombophillia?

Answer 1

Factor V Leiden (activated protein C resistance)

Question 2

What’s the commonest bleeding disorder?

Answer 2

Von willebrands disease

Question 3

What are the treatments for Von willebrands disease?

Answer 3

• tranexamic acid for mild bleeding
• desmopressin (adh) - reduced bleeding by stimulating release on VWF from weibel palade bodies in the endothelial cells,
• factor VIII concentrate

Question 4

What’s the pathogensis in idiopAthic thrombocytopenia purpura?

Answer 4

Antibodies (IgG)against glycoprotein IIb/IIIa or Ib-V-IX complex

Question 5

What would clotting blood tests show on someone with Von willebrands?

Answer 5

Prolonged bleeding time

Prolonged APTT

Question 6

What are the different types of ITP and who gets them?

Answer 6

Acute: children, post infection or vaccine, self limiting. Purpura.

Chronic: young/middle aged women, relapsing-remitting course. Bruising and bleeding.

Evans syndrome: itp in association with autoimmune haemolytic anaemia. DAT positive

Question 7

What are the features of immune thrombocytopenic purpura?

Answer 7

Isolated low platelets (destruction by splenic macrophages but spleen is NOT enlarged)

Normal BM but increase in megakaryocytes

Self-limiting purpura and bleeding
NO Splenomegaly.

Question 8

What are the signs of thrombotic thrombocytopenic purpura?

Answer 8

Fever, florid purpura, AKI, CNS sings, jaundice (haemolytic anaemia),

Raised LDH
Emergency!

Question 9

What’s the treatment for TTP?

Answer 9

Urgent plasma exchange

Steriods and rituximab

Question 10

What’s the treatment for ITP?

Answer 10

1) if need Rx - steriods or IV Ig

2) splenectomy and or immunosuppressants

Question 11

What’s the cause of TTP?

Answer 11

ADAMTS 13 deficiency which is responsible for degradation of vWF.