Pediatric common variable immunodeficiency: Immunologic and phenotypic associations with switched memory B cells

被引:42
作者
Yong, Pierre L. [1 ,2 ,3 ,4 ,6 ]
Orange, Jordan S. [5 ]
Sullivan, Kathleen E. [5 ]
机构
[1] Univ Penn, Sch Med, Robert Wood Johnson Clin Scholars Program, Philadelphia, PA 19104 USA
[2] Hosp Univ Penn, Div Pulm Allergy & Crit Care, Philadelphia, PA 19104 USA
[3] Univ Penn, Leonard Davis Inst Hlth Econ, Philadelphia, PA 19104 USA
[4] Univ Penn, Ctr Publ Hlth Initiat, Philadelphia, PA 19104 USA
[5] Childrens Hosp Philadelphia, Div Allergy & Immunol, Philadelphia, PA 19104 USA
[6] Philadelphia Vet Affairs Med Ctr, Dept Med, Philadelphia, PA USA
关键词
common variable immunodeficiency; memory B cells; autoimmune disease; recurrent infections; IgG; ANTIBODY-DEFICIENCY SYNDROME; MUTATIONS; DISEASE; TACI; HYPOGAMMAGLOBULINEMIA; COMPLICATIONS; SUBGROUPS; VARIANTS; CHILDREN; FEATURES;
D O I
10.1111/j.1399-3038.2010.01004.x
中图分类号
R392 [医学免疫学];
学科分类号
100102 ;
摘要
Recent studies suggest that patients with common variable immunodeficiency (CVID) and low numbers of switched memory B cells have lower IgG levels and higher rates of autoimmune disease, splenomegaly, and granulomatous disease; however, no prior literature has focused exclusively on pediatric cases. We examined the relationship between switched memory B cells and clinical and immunologic manifestations of CVID in a pediatric population. Forty-five patients were evaluated. Patients were categorized as Group I (< 5 switched memory B cells/ml, n = 24) or Group II (>= 5 switched memory B cells/mL, n = 21). CD3+ T-cell counts and CD19+ B-cell levels were lower among Group I patients. Only those in Group I had meningitis, sepsis, bronchiectasis, granulomatous lung disease, autoimmune cytopenias, or hematologic malignancies. Segregation of pediatric patients into high risk (Group I) and average risk (Group II) may assist in targeting surveillance appropriately.
引用
收藏
页码:852 / 858
页数:7
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