Familial eosinophilia: a benign disorder?

Author:

Klion Amy D.1,Law Melissa A.1,Riemenschneider William1,McMaster Mary Lou1,Brown Margaret R.1,Horne McDonald1,Karp Barbara1,Robinson Michael1,Sachdev Vandana1,Tucker Eben1,Turner Maria1,Nutman Thomas B.1

Affiliation:

1. From the National Institute of Allergy and Infectious Diseases, National Heart, Lung, and Blood Institute, National Cancer Institute, National Eye Institute, National Institute of Neurologic Diseases and Stroke, and the Warren Grant Magnusson Clinical Center, National Institutes of Health, Bethesda, MD.

Abstract

Abstract Familial eosinophilia (FE) is an autosomal dominant disorder characterized by marked eosinophilia and progression to end organ damage in some, but not all, affected family members. To better define the pathogenesis of FE, 13 affected and 11 unaffected family members (NLs) underwent a detailed clinical evaluation at the National Institutes of Health (NIH). No clinical abnormalities were more frequent in the family members with FE compared with the NLs. There was, however, a decreased prevalence of asthma in family members with FE compared with unaffected family members. Eosinophil morphology as assessed by either light or transmission electron microscopy was normal in family members with and without FE. Although levels of eosinophil-derived neurotoxin (EDN) and major basic protein (MBP) were elevated in patients with FE compared with NL, levels of both granule proteins were lower than in nonfamilial hypereosinophilic syndrome (HES). Similarly, increased surface expression of the activation markers CD69, CD25, and HLA-DR was detected by flow cytometry on eosinophils from patients with FE compared with NL, albeit less than that seen in HES. These data suggest that, despite prolonged marked eosinophilia, FE can be distinguished from HES by a more benign clinical course that may be related to a relative lack of eosinophil activation.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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