All issues > Volume 37(11); 1994

Case Report

J Korean Pediatr Soc. 1994;37(11):1615-1619. Published online November 15, 1994.

A Case of Wiskott-Aldrich Syndrome

Byoung Geun BG Lee¹, Soo Hee SH Chang¹, Soo Young SY Cho¹, Pyoung Han PH Hwang¹, Jung Soo JS Kim¹

¹Department of Pediatrics, Chonbuk National University Medical School, Chonju, Korea

Abstract

Wiskott-Aldrich Syndrome is an X-linked disorder characterized by recurrent infection, thrombocytopenia and eczema. Various defects in cell-mediated immunity and deficient antibody response to carbohydrate antigens have been described. We experienced a case of Wiskott-Aldrich Syndrome of 28 months old male patient. He has been suffered from multiple petechiae with bleeding, recurrent pyogenic infections and generalized eczema since 3 months of age. Immunological abnormalities are as follows: 1) Serum IgM was gradually decreased, while IgA and IgE were increased. 2) Antibody response against polysaccharide antigen (PRP) was not observed after 3 times of PRP-T immunization. 3) CD4/CD8 ratio was reversed (0.6). 4) Proliferative response of mononuclear cells was significantly reduced, and CMI skin test also showed negative results. A brief review of literature was made.

Keywords :Wiskott-Aldrich Syndrome, Immunity