POSTPHEOCHROMOCYTOMA ERYTHROPOIETIN-DEPENDENT ERYTHROCYTOSIS - A POSTISCHEMIC MECHANISM

Purpose: Erythropoietin-dependent pure erythrocytosis (EDPE) is a rare disorder caused by idiopathic hypererythropoietinemia. We describe a 13-year-oId girl who developed an EDPE-like erythrocytosis after remov al of an adrenal pheochromocytoma. Patients and Methods: As occurs in EDPE, this post-pheochromocytoma erythrocytosis was associated with a high serum erythropoietin (s-Epo) level that maintained physiological regulation. Results: Phlebotomies produced a three- to sixfold increas e of s-Epo, and a 6-week course of theophylline caused a decrease of b oth s-Epo and hemoglobin. Conclusions: We hypothesize that the intense and prolonged pheochromocytoma-induced renal ischemia before surgery could be the cause of this unique case of erythrocytosis.