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Hydroxyurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease
Author(s) -
Reiss Ulrike M.,
Bensimhon Pamela,
Zimmerman Sherri A.,
Ware Russell E.
Publication year - 2007
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20925
Subject(s) - phlebotomy , medicine , hyperviscosity , hydroxycarbamide , mean corpuscular volume , hyperviscosity syndrome , hematocrit , heart disease , cardiology , blood viscosity , mean corpuscular hemoglobin , gastroenterology , surgery , disease , multiple myeloma
Secondary erythrocytosis in cyanotic congenital heart disease (CCHD) causes substantial morbidity because of complications of hyperviscosity, including stroke and chronic end organ damage. Phlebotomy provides temporary improvement but leads to iron deficiency and can actually increase blood viscosity. We describe the successful use of hydroxyurea (hydroxycarbamide) in four patients with uncorrected CCHD and symptomatic secondary erythrocytosis. In all patients, hydroxyurea improved symptoms of hyperviscosity. Substantial decreases in the red blood cell (RBC) count were observed, along with increases in the mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH), leading to only modest declines in the circulating hemoglobin concentration. Two patients experienced transient mild myelosuppression, which promptly resolved with dose reduction of hydroxyurea. Hydroyxurea provides a novel and useful therapeutic approach to reduce hyperviscosity from secondary erythrocytosis in patients with CCHD, while preserving oxygen carrying capacity and avoiding iron depletion by phlebotomy. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.