Polycythemia Vera Differential Diagnoses

Updated: Aug 31, 2022
  • Author: Srikanth Nagalla, MD, MS, FACP; Chief Editor: Sara J Grethlein, MD, MBA, FACP  more...
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Diagnostic Considerations

Polycythemia is characterized by increased cell counts in all cell lines in the myeloid series (ie, red blood cells, white blood cells [preferentially granulocytes], and platelets). Thus, conditions that involve individual cell lines, such as the following, must be excluded:

  • Erythrocytosis - Causes include secondary polycythemia (an increase in red blood cell [RBC] counts, typically due to chronic hypoxemia), familial erythrocythemia, and relative polycythemia (a benign condition in which RBC numbers are normal but plasma volume is contracted, due to dehydration or to reduced venous compliance).

  • Granulocytosis from infections or mobilization by secondary causes, as in leukemoid reactions

  • Thrombocytosis from bleeding and iron deficiency

Diagnostic laboratory tests have been developed to increase the ability to identify primary myeloproliferative diseases (MPDs) and to differentiate them from reactive conditions associated with increased blood cell levels, which can mimic MPDs. Once an MPD (Philadelphia chromosome negative [Ph–]) is documented, it must be differentiated from the following conditions, which have manifestations that overlap with polycythemia vera (PV):

Differential Diagnoses