ISSN:
1432-1076
Keywords:
Bare lymphocyte syndrome
;
HLA expression
;
Severe combined immunodeficiency syndrome
;
Neutrophil dysfunction
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A 4-year-old girl presented with recurrent infections. Immunoglobulin deficiency (serum and secretory IgA, serum IgG3) neutropenia and neutrophil dysfunction (defective spontaneous migration and chemotaxis) were found. T-lymphocyte counts were normal and they responded to phytohaemagglutinin but were not stimulated by Concanavalin A, pokeweed mitogen and microbial antigens in vitro. Delayed cutaneous hypersensitivity testing to purified protein derivative and candidin was negative. Despite bacille Calmette-Guérm vaccination and candidiasis, near normal β-2-microglobulin and human leucocyte antigen (HLA) class I concentrations were detected on mononuclear cells and phytohaemagglutinin-induced lymphoblasts. HLA class II antigens (HLA-DP, −DQ, −DR) were not expressed. These observations indicated a bare lymphocyte syndrome (BLS) type II. This is the first time neutrophil dysfunction has been noted in association with BLS.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01959526
