All issues > Volume 38(1); 1995

Case Report

J Korean Pediatr Soc. 1995;38(1):99-103. Published online January 15, 1995.

A Case of Congenital Agammaglobulinemia

Sang Yong SY Kim¹, Jong Hyun JH Kim¹, Jin Han JH Kang¹, Joon Sung JS Lee¹, Sang In SI Shim²

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea
²Department of Clinical Pathology, Catholic University Medical College, Seoul, Korea

Abstract

Congenital agammaglobulinemia was described first among all human immunodeficiencies, and characterized by defect of B lymphocyte maturation and severe panhypogammaglobulinemia. These patients frequently acquire infections with high-grade extracellular pyogenic pyogenic organisms since infancy. We experienced a case of congenital agammaglobulinemia in a 10 years old male. He had history of recurrent pneumonia and purulent otitis media since late infancy. Also he had recieved operations for pyogenic arthritis of hip joint & osteomyelitis at 4 years of age. He had been suffered from productive cough, intermittent fever and skin infections for 3 months before admission. His immune status revealed panhypogammaglobulinemia and deficiency in mature B lymphocyte, but cell-mediated immunity was intact. He was treated with antibiotics and intravenous immunoglobulin with good results.

Keywords :Congenital agammaglobulinemia, B lymphocyte maturation