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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-old girl who developed an EDPE-like erythrocytosis after removal 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 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.
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