Polycythemia
Pronunciation: pol-ee-sy-THEE-mee-ah
Increased red blood cell mass and elevated hematocrit, commonly developing in patients with chronic cyanotic heart disease.
Full Definition
Polycythemia is a compensatory physiological response to chronic hypoxemia characterized by increased production of red blood cells, resulting in elevated hematocrit and hemoglobin levels. In congenital heart disease, secondary polycythemia develops in patients with cyanotic lesions as the body attempts to improve oxygen-carrying capacity. While initially beneficial, severe polycythemia can lead to hyperviscosity syndrome, increased risk of thrombosis, and bleeding complications. Management may include phlebotomy, iron replacement, and definitive surgical correction of the underlying cardiac defect.
Usage
Usage note: Distinguish from primary polycythemia vera. Document hematocrit values and associated symptoms.
In Context
- "The patient developed symptomatic polycythemia with a hematocrit of 65% and required therapeutic phlebotomy." — Hematology consultation
- "Iron deficiency was corrected to prevent relative polycythemia and its associated complications." — Laboratory follow-up note