polycythemia vera
A rare myeloproliferative disorder characterized by increased red blood cell production, occasionally seen in adolescents.
Full Definition
Polycythemia vera is a clonal myeloproliferative neoplasm characterized by increased production of red blood cells, and often white blood cells and platelets as well. While predominantly a disease of older adults, polycythemia vera can rarely occur in children and adolescents. The condition is associated with mutations in the JAK2 gene in most cases. Pediatric presentation may include headaches, dizziness, visual disturbances, and thrombotic complications. Diagnosis requires demonstration of increased red cell mass along with molecular or bone marrow evidence of a clonal disorder. Treatment focuses on reducing blood viscosity through phlebotomy and cytoreductive therapy when indicated.
Usage
Usage note: May be abbreviated as PV; distinguish from secondary polycythemia in documentation.
In Context
- "The 16-year-old athlete's elevated hemoglobin and hematocrit levels led to investigation for polycythemia vera versus secondary causes." — Hematology consultation note