Haemolytic Anaemia and Transfusion Medicine

Question 1

What is the inheritance pattern of hereditary spherocytosis?

Answer 1

autosomal dominant

Question 2

What is the pathophysiology of hereditary sphrerocytosis?

Answer 2

a genetic abnormality causes a defect in the cytoskeleton of the RBC membrane leading to increased osmotic fragility and type I autohaemolysis

Question 3

What are the two main types of autoimmune haemolytic anaemia?

Answer 3

cold agglutinin haemolysis

warm AIHA

Question 4

What is the first line management of warm AIHA?

Answer 4

supportive care and transfusion
pred 1mg/kg
consider DVT prophylaxis