All issues > Volume 43(1); 2000

Case Report

J Korean Pediatr Soc. 2000;43(1):117-122. Published online January 15, 2000.

A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia

Kyung-Yil KY Lee¹, Yon-Joo YJ Lee¹, Sang-Won SW Cha¹, Dong Joon DJ Lee¹, Ji-Whan JW Han¹, Kyung-Tae KT Whang¹, Joong-Gon JG Kim²

¹Department of Pediatrics, College of M edicine, The Catholic University of Korea, Seoul, Korea
²Department of Pediatrics, College of M edicine, Seoul National University, Seoul, Korea

Correspondence Kyung-Yil KY Lee ,Email: leekyungyil@yahoo.com

Abstract

Common variable immunodeficiency(CVID) is a heterogeneous collection of disorders with hypogammaglobulinemia with recurrent bacterial infections and high incidence of autoimmune disorders as its hallmark. We report a 7-year-old girl suffering from CVID with Coombs' test positive hemolytic anemia. She had been relatively well until 23-months old when she was admitted to Taejon St. Mary' s Hospital with pneumonia 5 years ago. Afterwards, she had suffered from recurrent otitis media, paranasal sinusitis, bronchitis and pneumonia, experiencing 13 admissions. She was diagnosed as autoimmune hemolytic anemia at 4-years old and had been treated with prednisolone. Laboratory finidings showed hypogammaglobulinemia(gamma-globulin in immunoelectrophoresis 0.04g/dL, IgG 170mg/dL, IgA 31mg/dL, IgM 27.5mg/dL) which was previously within normal limits checked at the age of 3- and 5-years old. Isohemmagglutinins(Anti-A,-B IgM and IgG) and anti-measles IgG, anti-mumps IgG, anti-rubella IgG and anti-HBs antibody along with PPD skin test were all negative. Peripheral lymphocyte subsets revealed as follows : pan T cells(CD3＋) 48.6%(normal values : 60-85%), pan B cells(CD19＋) 36.7%(8-20%), CD4＋ T cells 24.4%(28±8%), CD8＋ T cells 15.3%(5±10%), and CD4/CD8 ratio of 1.6(0.6-2.8). Proliferations of peripheral blood mononuclear cells induced by various T cell stimulants were all markedly depressed. Chronic paranasal sinusitis and lung parenchymal damages were revealed on computerized tomography and lung scan, and a monthly intravenous immunoglobulin therapy was started.

Keywords :Common variable immunodeficiency, Autoimmune hemolytic anemia; PHA, Anti-CD3, PMA, Ionomycin, PPD