J Korean Pediatr Soc.
1994 Nov;37(11):1615-1619.
A Case of Wiskott-Aldrich Syndrome
Abstract
- Wiskott-Aldrich Syndrome is an X-linked disorder characterized by recurrent infection, thrombocypenia and eczema. Various defects in cell-mediated immunity and deficient antibody reponse 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 PRPT 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.
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