TL Lee, GCF Chan, SY Ha, YL Lau

Abstract

A total of 99 cases of primary immunodeficiency diseases (PID) (61 males, 38 females) were registered at the Paediatric Immunology Clinic, Queen Mary Hospital, University of Hong Kong between July, 1988 and December, 1998. The following frequencies were found: predominantly humoral defects (n=34); predominantly T cell defects (n=15); combined immunodeficiency (n=5); phagocytic disorders (n=35); others (n=10). Chronic neutropenia was the most frequent disorder (n= 19), followed by X-linked agammaglobulinemia (n= 12), chronic granulomatous disease (n=12), selective deficiency of IgG subclass (n=10), common variable immunodeficiency (n=7), Wiskott-Aldrich syndrome (n=6) and severe combined immunodeficiency (n=5). Infection was the major complication leading to mortality (n=6). Lymphoma developed in 3 patients. Bone marrow transplantation was done in 12 patients with over 90% success rate. The clinical approach and general principles of management of PIDs will also be discussed.