Practice Patterns in the Diagnosis and Treatment of Polycythemia Vera in the Post-JAK2 V617F Discovery Era.

ABSTRACT

Polycythemia vera (PV) is an acquired clonal hematopoietic stem cell disorder characterized by an overproduction of red blood cells, white blood cells, and platelets; thrombotic and hemorrhagic complications; and an increased risk of transformation to myelofibrosis and acute leukemia. In 1967, the Polycythemia Vera Study Group proposed the optimal approach to diagnosis and treatment of PV, and in 2002, investigators from Johns Hopkins University School of Medicine surveyed the practice patterns of hematologists as they pertained to PV. Since this survey, the JAK2 V617F mutation was discovered, leading to a new era of discovery in the disease pathogenesis, diagnosis, and classification and treatment of PV. Our objective was to survey hematologists in the diagnosis and treatment of PV in the modern, post-JAK2 V617F discovery era. An anonymous 17-question survey was emailed to members of the Myeloproliferative Neoplasm (MPN) Research Foundation database and Aplastic Anemia and MDS International Foundation. A total of 71 surveys were used in the analysis. Diagnostic testing varied according to the respondent's clinical experience and practice type. In addition, there were marked differences in target hematocrit and platelet count among those surveyed. There continue to be variations in diagnosis and treatment of PV despite WHO guidelines and the JAK2 discovery. US-based guidelines for MPNs are needed to create consistency in the management of PV and other MPNs.