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A Case of Wiskott-Aldrich Syndrome

Journal of the Korean Pediatric Society 1993;36(3):439-446.
Published online March 15, 1993.
A Case of Wiskott-Aldrich Syndrome
Dong Un Kim, Seung Hoon Han, Jin Han Kang, Joon Sung Lee
Department of Pediatrics, Catholic University Medical College, Seoul, Korea
Wiskott-Aldrich증후군 1례
김동언, 한승훈, 강진한, 이준성
가톨릭대학교 의과대학 소아과학교실
Abstract
Wiskott-Aldrich syndrome s an X-linked combined immunodeficency disorder characterzed 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 deecreased. The patient also shows skin test anergy and progressive T-lymphocytopenia, Bleedings and recurrent infectins are the main causes of death and the patients usually die befor age 10. Bone marrow transplantation is accepted to be the only radical therapy. We experienced a case compatible with Wiskott-Aldrichsyndrome in a 5 year old male child who accompanied above clinical manifestations and laboratory findings.
Key Words: Thrombocytopenia, Eczema, Recurrent infections


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