A Polycythemia Vera Update: Diagnosis, Pathobiology, and Treatment
Open Access
- 1 January 2000
- journal article
- Published by American Society of Hematology in Hematology-American Society Hematology Education Program
- Vol. 2000 (1) , 51-68
- https://doi.org/10.1182/asheducation-2000.1.51
Abstract
This review focuses on polycythemia vera (PV)—its diagnosis, cellular and genetic pathology, and management. In Section I, Dr. Pearson, with Drs. Messinezy and Westwood, reviews the diagnostic challenge of the investigation of patients with a raised hematocrit. The suggested approach divides patients on their red cell mass (RCM) results into those with absolute (raised RCM) and apparent (normal RCM) erythrocytosis. A standardized series of investigations is proposed for those with an absolute erythrocytosis to confirm the presence of a primary (PV) or secondary erythrocytosis, with abnormal and normal erythropoietic compartments respectively, leaving a heterogenous group, idiopathic erythrocytosis, where the cause cannot be established. Since there is no single diagnostic test for PV, its presence is confirmed following the use of updated diagnostic criteria and confirmatory marrow histology.Keywords
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