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.