Table of Contents
Journal of Allergy
Volume 2009 (2009), Article ID 804910, 5 pages
http://dx.doi.org/10.1155/2009/804910
Case Report

Cardiac Manifestations from Non-FIP1L1-PDGFR 𝛼 -Associated Hypereosinophilic Syndrome in a 13-Year-Old African American Boy

1Department of Medicine, Aurora Health Care, Milwaukee, WI 53213, USA
2Department of Pediatrics, Children's Hospital of Wisconsin, Milwaukee, WI, USA
3Department of Pediatrics, Allergy, Asthma, and Clinical Immunology, Children's Hospital of Wisconsin, Milwaukee, WI, USA
4Department of Pediatrics, Cardiology, Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI, USA

Received 12 March 2009; Revised 2 September 2009; Accepted 8 October 2009

Academic Editor: Garry Walsh

Copyright © 2009 Cindy M. Salm et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Hypereosinophilic syndrome (HES) is a rare disorder typically seen in males, aged 20 to 50, with a predisposition for Caucasians. It is marked by overproduction of eosinophils (>1,500/ 𝜇 L) and multiorgan system damage due to eosinophilic infiltration and mediator release. There are multiple variants of HES. Cardiac complications are more common in myeloproliferative HES associated with the FIP1L1-PDGFR 𝛼 mutation. Sequelae range from acute necrosis and thrombus formation to fibrosis of the endomyocardium. We describe a young boy who presented with chest pain and dyspnea. A diagnosis of HES was made after all other etiologies of eosinophilia were excluded. Although he was found to be negative for the FIP1L1-PDGFR 𝛼 mutation, his cardiac complications included pericardial effusion and restrictive cardiomyopathy, without myocardial necrosis. Multi-organ involvement resulted in pericarditis, pleuritis, nephritis, and dermatitis. In this paper, we review his case and discuss the known subtypes of HES, the classic cardiac complications, and available treatment strategies.