Myeloproliferative conditions

Question 1

Rf of polycythaemia vera

Answer 1

increasing age and Budd chart syndrome

Question 1

presentation of polycythaemia vera

Answer 1

symptoms of hyperviscosity –> headache, arterial/venous thrombus, visual disturbance
-pruritus (after hot shower)
-erythromelalgia (pain and redness in hands and feet)
-splenomegaly
-gout (high uric acid release due to high cell turnover)

Question 2

Ix for PV

Answer 2

raised Hb + red cell mass >25% above predicted
-serrum ferritin low due to increased demand for iron
-blood film
-U+E and LFT (look for secondary causes)
-ABG - is hypoxia driving it?
-JAK2 mutational analysis

Question 3

Mx of PV

Answer 3

venesection, CVS Rf optimisation, low dose aspirin

Question 4

what can PV become

Answer 4

myelofibrosis or AML

Question 5

what is myelofibrosis

Answer 5

an abnormal proliferation of a cell type (normally megakaryocyte), this then produces growth factors which causes bone marrow fibrosis

Question 6

how does myelofibrosis arise

Answer 6

it can arise de novo or from PV or ET

Question 7

what investigations are done for myelofibrosis and what do they show

Answer 7

FBC - pancytopenia (but may initially get a raised WCC or platelet depending on which cell line is proliferating)
Poikilocytes on blood film
Dry bone marrow tap
May find hepatomegaly and splenomegaly on examination

Question 8

MX options for myelofibrosis

Answer 8

SCT, targeted therapy (as linked to JAK2), EPO, transfusions

Question 9

What is the platelet count in essential thrombocytosis

Answer 9

> 600 x10(9)

Question 10

what are some features of essential thrombocytosis

Answer 10

burning sensation in hands, can present with thrombosis or haemorrhage

Question 11

MX of ET

Answer 11

hydroxycarbamide and aspirin

Question 12

causes for secondary thrombocytosis

Answer 12

iron deficiency anaemia, surgery, inflammatory state, underlying solid cancer