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Immunological investigations in two brothers with ataxia telangiectasia Louis-Bar

✍ Scribed by H. Schulte-Wissermann; P. Gutjahr; P. Zebisch; M. Reitz; E. -M. Lemmel

Publisher
Springer
Year
1976
Tongue
English
Weight
634 KB
Volume
122
Category
Article
ISSN
0340-6997

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✦ Synopsis

Two of three brothers with the classical signs of ataxia telangiectasia were investigated for their immunological disorders at the ages of 13 and 16 years, respectively. The elder brother also suffers from autoimmune hemolytic anemia, a complication which has not yet been described in the course of ataxia telangiectasia. Immunological investigations made in both brothers showed a reduction in the number and function of T lymphocytes. The number of B lymphocytes was normal, among which there were cells staining for IgA, although serum IgA was absent. It seems possible that this phenomenon is caused by a disturbance in the process of maturation of lymphoid cells with a lack of differentiation into IgA-synthesizing plasma cells.

πŸ“œ SIMILAR VOLUMES

Late-onset ataxia telangiectasia in two
Late-onset ataxia telangiectasia in two brothers presenting with juvenile resting tremor
✍ J. A. P. Hiel; C. M. R. Weemaes; D. F. C. M. Smeets; C. J. W. van de Vlasakker; πŸ“‚ Article πŸ“… 1994 πŸ› John Wiley and Sons 🌐 English βš– 284 KB

## Abstract Two young adult brothers presented with a 5‐ to 6‐Hz resting tremor of the upper limbs. Although ataxia was not unequivocally present and ocular telangiectasia was minimal, typical rearrangements of chromosomes 7 and 14, and increased α‐fetoprotein levels indicated the presence of ataxi