All issues > Volume 36(3); 1993

Case Report

J Korean Pediatr Soc. 1993;36(3):439-446. Published online March 15, 1993.

A Case of Wiskott-Aldrich Syndrome

Dong Un DU Kim¹, Seung Hoon SH Han¹, Jin Han JH Kang¹, Joon Sung JS Lee¹

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea

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.

Keywords :Thrombocytopenia, Eczema, Recurrent infections