Polycythemia vera

This code represents a chronic myeloproliferative neoplasm characterized by the overproduction of all three myeloid cell lines, predominantly red blood cells, in the bone marrow. It leads to an increased red blood cell mass, often resulting in elevated hemoglobin and hematocrit levels, and can cause symptoms related to hyperviscosity and thrombosis.

Use this code for documented cases of primary polycythemia vera, confirmed by bone marrow biopsy, genetic testing (e.g., JAK2 mutation), and clinical findings. This diagnosis is typically established by a hematologist based on World Health Organization (WHO) diagnostic criteria.

• Ensure documentation clearly distinguishes primary polycythemia vera from secondary causes of erythrocytosis.
• Do not code this with D75.0 (familial polycythemia) or D75.1 (secondary polycythemia) due to the Excludes1 note.
• Review for any associated complications, such as thrombosis or myelofibrosis, which may require additional coding.