Postpheochromocytoma erythropoietin-dependent erythrocytosis. A postischemic mechanism?
OBJECTIVE
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.


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 increase of s-Epo, and a 6-week course of theophylline caused a decrease of both 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.
Department of Pediatrics, Magenta Hospital, Italy.
M
M
Bosio
G
G
Barosi
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications*; D004921:Erythropoietin / Q000502:physiology*; D005260:Female; D006801:Humans; D010673:Pheochromocytoma / Q000150:complications*; D011086:Polycythemia / Q000209:etiology / Q000503:physiopathology*
D004921:Erythropoietin