PV and AML

 Q: 74 years old male with a known history of Polycythemia Vera (PV) who has previous treatments with chemotherapy is admitted to ICU with community-acquired pneumonia - requiring intermittent BiPAP. Patient is also found to be in Acute Myeloid Leukemia (AML). AML with the history of PV usually signifies a good prognosis.

A) True

B) False

Answer: B

PV's transformation to AML bears a very poor prognosis. Two major risk factors in this regard are age above 70 years and previous treatments with cytoreductive agents except for hydroxyurea and interferon. The median survival of these patients is less than six months despite complete remission with AML treatment. 

These patients should be considered very early for allogeneic hematopoietic cell transplantation to avoid the fatal outcome.

#oncology

References:

1. Finazzi G, Caruso V, Marchioli R, et al. Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study. Blood 2005; 105:2664. 

2. Passamonti F, Rumi E, Arcaini L, et al. Leukemic transformation of polycythemia vera: a single center study of 23 patients. Cancer 2005; 104:1032. 

3. Tefferi A, Guglielmelli P, Larson DR, et al. Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood 2014; 124:2507.

4. Gangat N, Strand J, Li CY, et al. Leucocytosis in polycythaemia vera predicts both inferior survival and leukaemic transformation. Br J Haematol 2007; 138:354.