A case of Wiskott-Aldrich syndrome.
- Author:
Dong Un KIM
;
Seung Hoon HAN
;
Jin Han KANG
;
Joon Sung LEE
- Publication Type:Original Article
- Keywords:
Thrombocytopenia;
Eczema;
Recurrent infections
- MeSH:
Bone Marrow Transplantation;
Cause of Death;
Child;
Child, Preschool;
Dermatitis, Atopic;
Eczema;
Humans;
Immunoglobulin A;
Immunoglobulin E;
Immunoglobulin G;
Immunoglobulin M;
Male;
Skin Tests;
Thrombocytopenia;
Wiskott-Aldrich Syndrome*;
X-Linked Combined Immunodeficiency Diseases
- From:Journal of the Korean Pediatric Society
1993;36(3):439-446
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
