{"title":"Postpheochromocytoma erythropoietin-dependent erythrocytosis. A postischemic mechanism?","authors":"M Bosio, G Barosi","doi":"","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>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.</p><p><strong>Patients and methods: </strong>As occurs in EDPE, this post-pheochromocytoma erythrocytosis was associated with a high serum erythropoietin (s-Epo) level that maintained physiological regulation.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>We hypothesize that the intense and prolonged pheochromocytoma-induced renal ischemia before surgery could be the cause of this unique case of erythrocytosis.</p>","PeriodicalId":22558,"journal":{"name":"The American journal of pediatric hematology/oncology","volume":"16 4","pages":"365-7"},"PeriodicalIF":0.0000,"publicationDate":"1994-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"The American journal of pediatric hematology/oncology","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
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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