BOF: Malignant (Myeloproliferative Neoplasm)

A 59 year old man is known to have a JAK2 V617F mutated polycythaemia. He has been maintained on a venesection programme for the last 6 years and has had no thrombotic complications. He is now complaining of increasing tiredness and some night sweats. His current FBC is Hb 9.9g/dL, MCV 70 fl, Neutrophils 5.3 x 10⁹/L, Platelets 712 x 10⁹/L, Ferritin 9

Which of the following options would be the most appropriate next step?

a) Bone marrow biopsy

b) Iron supplementation

c) Hydroxycarbamide

d) Recombinant human erythropoietic proteins (rHuEpo)

e) Top-up transfusion

Answer:

Bone marrow biopsy

Explanation:

Although excess venesection may explain the anaemia and iron deficiency, a new thrombocytosis and anaemia requires investigation for myelofibrosis. Although the treatment would likely be hydroxyurea in the first instance, an assessment bone marrow would be helpful to rule out disease evolution before commencing therapy, and cytoreduction is not mandated by criteria for essential thrombocythemia, since he is under 60, has no prior complications, an hydroxyurea may exacerbate his anaemia.

Reference:

Beer PA, Erber WN, Campbell PJ, Green AR. How I treat essential thrombocythemia. Blood 2011;117(5):1472-1482