J Korean Pediatr Soc.  1993 Mar;36(3):439-446.

A case of Wiskott-Aldrich syndrome

• Kim DU
• Han SH
• Kang JH
• Lee JS

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.

Keyword

Thrombocytopenia; Eczema; Recurrent infections

MeSH Terms

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
Immunoglobulin A
Immunoglobulin E
Immunoglobulin G
Immunoglobulin M