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KMID : 0367419930360030439
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Journal of Korean Pediatric Society
1993 Volume.36 No. 3 p.439 ~ p.446
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A Case of Wiskott-Aldrich Syndrome
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Abstract
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Wiskott-Aldrich syndrome is an X-linked combined immunodeficiency disorder characterized by severely decreased number of platelets which are small in size, eczema resembling atopic dermatitis and recurrent infection. The serum of the patient
contains
elevated concentrations of IgA and IgE, whereas the IgG level is usually normal and IgM level is decreased. The patients also shows skin test anergy and progressive T-lymphocytopenia. Bleedings and recurrent infections are the main causes of
death
and
the patients usually die before age 10. Bone marrow transplantation is accepted to be the only radical therapy.
We experienced a case compatible with Wiskott-Aldrich syndrome in a 5 year old male child who accompanied above clinical manifestations and laboratory findings.
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KEYWORD
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