A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia.
- Author:
Kyung Yil LEE
1
;
Yon Joo LEE
;
Sang Won CHA
;
Ji Whan HAN
;
Kyung Tae WHANG
;
Joong Gon KIM
Author Information
1. Department of Pediatrics, College of Medicine, The Catholic University of Korea.
- Publication Type:Case Report
- Keywords:
Common variable immunodeficiency;
Autoimmune hemolytic anemia: PHA;
Anti-CD3;
PMA;
Ionomycin;
PPD
- MeSH:
Agammaglobulinemia;
Anemia, Hemolytic;
Anemia, Hemolytic, Autoimmune*;
B-Lymphocytes;
Bacterial Infections;
Bronchitis;
Child;
Child, Preschool;
Common Variable Immunodeficiency*;
Coombs Test;
Daejeon;
Female;
Humans;
Immunization, Passive;
Immunoelectrophoresis;
Immunoglobulin A;
Immunoglobulin G;
Immunoglobulin M;
Incidence;
Infant;
Ionomycin;
Lung;
Lymphocyte Subsets;
Otitis Media;
Pneumonia;
Prednisolone;
Sinusitis;
Skin Tests;
T-Lymphocytes
- From:Journal of the Korean Pediatric Society
2000;43(1):117-122
- CountryRepublic of Korea
- Language:Korean
-
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.
