T Cell Abnormalities in Mixed Connective Tissue Disease Complicated with Klinefelter's Syndrome

Katsuhiko ISHIHARA; Masafumi YOSHIMURA; Hirohisa NAKAO; Yuzuru KANAKURA; Yoshio KANAYAMA; Yuji MATSUZAWA

doi:10.2169/internalmedicine.33.714

Katsuhiko ISHIHARA, Masafumi YOSHIMURA, Hirohisa NAKAO, Yuzuru KANAKURA, Yoshio KANAYAMA, Yuji MATSUZAWA

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Keywords: CD57⁺/CD8⁺ T lymphocytes, IL-2 production, dehydroepiandrosterone sulfate

JOURNAL FREE ACCESS

1994 Volume 33 Issue 11 Pages 714-717

DOI https://doi.org/10.2169/internalmedicine.33.714

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Abstract

We report a 28-year-old Japanese with Klinefelter's syndrome who developed mixed connective tissue disease (MCTD) and Sjögren syndrome. Previously being well, he presented with Raynaud's phenomenon, dry eye, fever and polyarthralgia. Clinical examinations revealed anti-nRNP autoantibody, leukopenia and lung fibrosis. Then he was found to have Klinefelter's syndrome. Flow cytometric analysis showed a relative increase of peripheral CD8⁺ T lymphocytes carrying either HLA-DR or CD57. Lymphocyte IL-2 production induced in vitro by concanavalin A was decreased. Such T cell abnormalities may be implicated in the development of autoimmune disease in Klinefelter's syndrome.
(Internal Medicine 33: 714-717, 1994)

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