title: Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome.
abstract: Evans' syndrome (ES) is characterized by autoimmune hemolytic anemia and thrombocytopenia and has been associated with immune deficiency and lymphoproliferation in some cases. Abnormalities of Fas-mediated apoptosis have been reported in various immune dysregulation disorders associated with autoimmunity and lymphoproliferation. We measured lymphocyte Fas expression and Fas-mediated T lymphocyte apoptosis in 7 children with ES, 7 with acute idiopathic thrombocytopenic purpura (ITP) and 9 with non-immune-mediated disorders. Patients with ES had higher Fas expression on peripheral blood T and B lymphocytes (P<0.001 and P=0.046, respectively) and increased Fas-mediated elimination of activated T lymphocytes compared with the control groups. While two ES patients had panhypogammaglobulinemia at testing, three more developed it later, reaching a frequency of 83%. Some children with ES have increased lymphocyte Fas expression and Fas-mediated T lymphocyte apoptosis and these may be early signs of common variable immunodeficiency disorder in ES.
authors.affiliation: Division of Hematology/Oncology, Children's Hospital of Michigan, Carman and Ann Adams Department of Pediatrics, Wayne State University, Detroit, MI, USA. ssavasan@med.wayne.edu
authors.forename: Süreyya
authors.initials: S
authors.lastname: Savaşan
authors.forename: Indira
authors.initials: I
authors.lastname: Warrier
authors.forename: Steven
authors.initials: S
authors.lastname: Buck
authors.forename: Joseph
authors.initials: J
authors.lastname: Kaplan
authors.forename: Yaddanapudi
authors.initials: Y
authors.lastname: Ravindranath
mesh_terms: D000293:Adolescent; D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D015703:Antigens, CD / Q000096:biosynthesis; D017209:Apoptosis / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000453:epidemiology / Q000378:metabolism; D005434:Flow Cytometry; D006684:HLA-DR Antigens; D006801:Humans; D008213:Lymphocyte Activation / Q000276:immunology; D009503:Neutropenia / Q000150:complications / Q000378:metabolism; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000276:immunology / Q000378:metabolism; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism*; D013921:Thrombocytopenia / Q000150:complications / Q000378:metabolism; D019014:fas Receptor / Q000378:metabolism*
chemical_list: D015703:Antigens, CD; D006684:HLA-DR Antigens; D019014:fas Receptor