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@article{837034,
author = {Chapel, H. and Lucas, M. and Lee, M. and Bjorkander, J. and Webster, D. and Grimbacher, B. and Fieschi, C. and Thon, Vojtěch and Abedi, MR. and Hammarstrom, L.},
article_number = {2},
keywords = {DISEASES CLASSIFICATION COMMITTEE; ANTIBODY DEFICIENCY; IMMUNE DEFICIENCY; B CELL; PRIMARY HYPOGAMMAGLOBULINEMIA; IMMUNOLOGICAL FEATURES; INTERNATIONAL UNION; T CELL; MANIFESTATIONS; COMPLICATIONS},
language = {eng},
issn = {0006-4971},
journal = {Blood},
title = {Common variable immunodeficiency disorders: division into distinct clinical phenotypes},
volume = {2008/112},
year = {2008}
}
TY - JOUR
ID - 837034
AU - Chapel, H. - Lucas, M. - Lee, M. - Bjorkander, J. - Webster, D. - Grimbacher, B. - Fieschi, C. - Thon, Vojtěch - Abedi, MR. - Hammarstrom, L.
PY - 2008
TI - Common variable immunodeficiency disorders: division into distinct clinical phenotypes
JF - Blood
VL - 2008/112
IS - 2
SP - 277-286
EP - 277-286
SN - 00064971
KW - DISEASES CLASSIFICATION COMMITTEE
KW - ANTIBODY DEFICIENCY
KW - IMMUNE DEFICIENCY
KW - B CELL
KW - PRIMARY HYPOGAMMAGLOBULINEMIA
KW - IMMUNOLOGICAL FEATURES
KW - INTERNATIONAL UNION
KW - T CELL
KW - MANIFESTATIONS
KW - COMPLICATIONS
N2 - The European Common Variable Immunodeficiency Disorders registry was started in 1996 to define distinct clinical phenotypes and determine overlap within individual patients. A total of 7 centers contributed patient data, resulting in the largest cohort yet reported. Patients (334), validated for the diagnosis, were followed for an average of 25.6 years (9461 patientyears). Data were used to define 5 distinct clinical phenotypes: no complications, autoimmunity, polyclonal lymphocytic infiltration, enteropathy, and lymphoid ma-lignancy. A total of 83% of patients had only one of these phenotypes. Analysis of mortality showed a considerable reduction in the last 15 years and that different phenotypes were associated with different survival times. Types of complications and clinical phenotypes varied significantly between countries, indicating the need for large, international registries. Ages at onset of symptoms and diagnosis were shown to have a Gaussian distribution, but were not useful predictors of phenotype. The only clinical predictor was polyclonal lymphocytic infiltration, which was associated with a 5-fold increased risk of lymphoid malignancy. There was widespread variation in the levels of serum immunoglobulin isotypes as well as in the percentages and absolute numbers of B cells, confirming the heterogeneity of these conditions. Higher serum IgM and lower circulating CD8 proportions were found to be predictive markers for polyclonal lymphocytic infiltration and autoimmunity, respectively.
ER -
CHAPEL, H., M. LUCAS, M. LEE, J. BJORKANDER, D. WEBSTER, B. GRIMBACHER, C. FIESCHI, Vojtěch THON, MR. ABEDI and L. HAMMARSTROM. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. \textit{Blood}. 2008, 2008/112, No~2, p.~277-286. ISSN~0006-4971.
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