Erythrocytosis (an increase in the total red cell mass)* in childhood is usually secondary to cyanotic congenital heart disease. Occasionally it occurs with pulmonary disease, methemoglobinemia, brain tumors, Cushing's disease, or renal adenoma. When there is an accompanying increase of white blood cells and platelets, the condition is termed polycythemia vera; this is extremely rare in children. Occasionally the condition may be familial with no elevation of white blood cell or platelet count and with no underlying cause or associated condition discernable. It is the purpose of this paper to present a case of primary erythrocytosis in a 6-year-old child whom we have been able to investigate.
Report of Case
The patient, a 6-year-old boy, was first seen in the Congenital Heart Disease Clinic of the University of Colorado Medical Center on Jan. 22, 1957. He had been referred because of cyanosis and decreasing tolerance for exercise. Pregnancy and delivery
KNOCK HL, GITHENS JH. Primary Erythrocytosis of Childhood. Am J Dis Child. 1960;100(2):189-195. doi:10.1001/archpedi.1960.04020040191006