J Korean Pediatr Soc.  2000 Jan;43(1):117-122.

A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia

• Lee KY
• Lee YJ
• Cha SW
• Han JW
• Whang KT
• Kim JG

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.

Keyword

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

MeSH Terms

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
Immunoglobulin A
Immunoglobulin G
Immunoglobulin M
Ionomycin
Prednisolone