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A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia

Journal of the Korean Pediatric Society 2000;43(1):117-122.
Published online January 15, 2000.
A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia
Kyung-Yil Lee1, Yon-Joo Lee1, Sang-Won Cha1, Dong Joon Lee1, Ji-Whan Han1, Kyung-Tae Whang1, Joong-Gon 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
용혈성 빈혈을 동반한 공통 가변성 면역부전 (Common Variable Immunodeficiency) 1례
이경일1, 이연주1, 차상원1, 이동준1, 한지환1, 황경태1, 김중곤2
1가톨릭대학교 의과대학 소아과학교실
2서울대학교 의과대학 소아과학교실
Kyung-Yil Lee, Email: leekyungyil@yahoo.com
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.
Key Words: Common variable immunodeficiency, Autoimmune hemolytic anemia, PHA, Anti-CD3, PMA, Ionomycin, PPD

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