Bosio M, Barosi G
Department of Pediatrics, Magenta Hospital, Italy.
Am J Pediatr Hematol Oncol. 1994 Nov;16(4):365-7.
Erythropoietin-dependent pure erythrocytosis (EDPE) is a rare disorder caused by idiopathic hypererythropoietinemia. We describe a 13-year-old girl who developed an EDPE-like erythrocytosis after removal of an adrenal pheochromocytoma.
As occurs in EDPE, this post-pheochromocytoma erythrocytosis was associated with a high serum erythropoietin (s-Epo) level that maintained physiological regulation.
Phlebotomies produced a three- to sixfold increase of s-Epo, and a 6-week course of theophylline caused a decrease of both s-Epo and hemoglobin.
We hypothesize that the intense and prolonged pheochromocytoma-induced renal ischemia before surgery could be the cause of this unique case of erythrocytosis.
