A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia
Journal of the Korean Pediatric Society
;
: 117-122, 2000.
Article
in Korean
| WPRIM
| ID: wpr-217858
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Otitis Media
/
Pneumonia
/
Sinusitis
/
Bacterial Infections
/
Bronchitis
/
Immunoglobulin A
/
Immunoglobulin G
/
Immunoglobulin M
/
Coombs Test
/
Prednisolone
Type of study:
Incidence study
/
Prognostic study
Limits:
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
Language:
Korean
Journal:
Journal of the Korean Pediatric Society
Year:
2000
Type:
Article
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