Virchows Archiv

, Volume 473, Issue 1, pp 131–135 | Cite as

Molecular evidence of JAK2 p.V617F mutated pure erythroid leukemia arising from polycythemia vera

  • Alisha D. Ware
  • Jacqueline Birkness
  • Amy S. Duffield
  • Christopher D. Gocke
Brief Report


Pure erythroid leukemia (PEL) is an uncommon subtype of acute myeloid leukemia (AML) [7, 8, 13, 15]. While AML is recognized as a complication of polycythemia vera (PV) [10, 11, 12], there are few reports of PV transformation to PEL [1, 2, 3, 4, 6, 8, 9]. Five reported cases of PEL arising from PV, diagnosed between 1956 and 1962, followed therapy with radioactive phosphorus (32P) [1, 4, 6, 9]. There were two case reports of transformation following therapy with hydroxyurea, in 1998 and 2012 [2, 3]. More recently, transformation to PEL and erythroid sarcoma in patients with long-standing PV treated with phlebotomy and hydroxyurea was described [5]. Here, we report molecular evidence of an unusual case of PEL with osseous involvement arising in a patient with known JAK2 V617F-positive PV.

Case report

A 68-year-old man with a history of JAK2 V617F-positive polycythemia vera (Fig.  1), managed with phlebotomy, hydroxyurea, and recent addition of ruxolitinib, presented with...


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PathologyThe Johns Hopkins University School of MedicineBaltimoreUSA

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