Haem

Question 1

Poor prognostic factors for ALL?

Answer 1

age < 2 years or > 10 years,
WBC > 20 * 109/l at diagnosis,
T or B cell surface markers,
non-Caucasian,
male sex

Question 2

Role of von willebrand factor?

Answer 2

• promotes platelet adhesion to damaged endothelium

- carrier molecule for factor VIII

Question 3

Ix of von willebrand’s disease?

Answer 3

Prolonged bleeding time, APTT may be prolonged, fVIII levels may be reduced (Type 1/3), defective platelet aggregation with ristocetin

Question 4

Mx of Von Willebrand’s disease?

Answer 4

tranexamic acid for mild bleeding.

• Desmopressin: to raise levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
• fVIII concentrate