HUMAN IMMUNODEFICIENCY RESULTING FROM A MATURATIONAL ARREST OF GERMINAL CENTER B-CELLS

被引:14
作者
BRAUN, J [1 ]
GALBRAITH, L [1 ]
VALLESAYOUB, Y [1 ]
SAXON, A [1 ]
机构
[1] UNIV CALIF LOS ANGELES,SCH MED,DEPT MED,DIV CLIN IMMUNOL & ALLERGY,LOS ANGELES,CA 90024
关键词
IMMUNODEFICIENCY; GERMINAL CENTER; VH FAMILY; LYMPHOCYTE-B DEVELOPMENT;
D O I
10.1016/0165-2478(91)90152-Z
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Common variable immunodeficiency (CVI) is an acquired human disorder involving a striking and heterogeneous maturational defect of B lymphocytes. In this study, we used a recently developed VH gene utilization assay to analze the abundance of developmentally restricted and unrestricted V genes in blood B cells from nine CVI patients. Unrestricted clones (bearing rearranged VH5, VH4, or VH6 genes) were present in normal abundance in this group of CVI patients. However, clones bearing VH3L, a subgroup of the VH3 family normally abundant in blood B cells but absent in B cells at the germinal center stage, were deficient in seven of nine CVI patients. Based on these findings and a reconsideration of previously reported B cell features in CVI, we propose that the disorder represents in most cases a maturational arrest of B cells at the germinal center stage.
引用
收藏
页码:205 / 208
页数:4
相关论文
共 8 条
[1]   LOCALIZATION OF INTERLEUKIN-6 MESSENGER-RNA IN HUMAN TONSILS BY INSITU HYBRIDIZATION [J].
BOSSELOIR, A ;
HOOGHEPETERS, EL ;
HEINEN, E ;
CORMANN, N ;
KINETDENOEL, C ;
VANHAELST, L ;
SIMAR, L .
EUROPEAN JOURNAL OF IMMUNOLOGY, 1989, 19 (12) :2379-2381
[2]  
GADOL N, 1988, BLOOD, V71, P1048
[3]   IDIOPATHIC LATE-ONSET IMMUNOGLOBULIN DEFICIENCY - CLINICAL OBSERVATIONS IN 50 PATIENTS [J].
HERMANS, PE ;
DIAZBUXO, JA ;
STOBO, JD .
AMERICAN JOURNAL OF MEDICINE, 1976, 61 (02) :221-237
[4]  
HONG R, 1981, CLIN IMMUNOL ALLERGY, V1, P509
[5]   FAILURE OF B-CELLS IN COMMON VARIABLE IMMUNODEFICIENCY TO TRANSIT FROM PROLIFERATION TO DIFFERENTIATION IS ASSOCIATED WITH ALTERED B-CELL SURFACE-MOLECULE DISPLAY [J].
SAXON, A ;
GIORGI, JV ;
SHERR, EH ;
KAGAN, JM .
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 1989, 84 (01) :44-55
[6]   ISOLATED HUMAN FOLLICULAR DENDRITIC CELLS DISPLAY A UNIQUE ANTIGENIC PHENOTYPE [J].
SCHRIEVER, F ;
FREEDMAN, AS ;
FREEMAN, G ;
MESSNER, E ;
LEE, G ;
DALEY, J ;
NADLER, LM .
JOURNAL OF EXPERIMENTAL MEDICINE, 1989, 169 (06) :2043-2058
[7]  
VALLESAYOUB Y, 1990, BLOOD, V76, P17
[8]  
VELARDI A, 1986, J IMMUNOL, V137, P2808