Haematology

Question 1

Identify the possible complications of polycythaemia rubra vera

Answer 1

Immediate/Early: pruritus, haemorrhage, thrombosis (CVA, MI, DVT), erythromelalgia

Late: postpolycythaemic myelofibrosis (–> anaemia, pancytopenia), transformation to acute myeloid leukaemia

(BMJ)

Question 2

Generate a management plan for polycythaemia

Answer 2

Tx: phlebotomy, hydroxycarbamide (‘cytoreductive’), aspirin

(path)

Question 3

Identify appropriate investigations for polycythaemia

Answer 3

Confirm diagnosis

• 1st: JAK2 mutation (95%)
• raised Hb, raised HCT, low serum EPO. Normal/raised platelets/WCC

(path, passmedicine)

Question 4

Define polycythaemia rubra vera

Answer 4

Polycythaemia rubra vera (PRV) is a myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets.

(passmedicine)