2008
Common variable immunodeficiency disorders: division into distinct clinical phenotypes
CHAPEL, H., M. LUCAS, M. LEE, J. BJORKANDER, D. WEBSTER et. al.Základní údaje
Originální název
Common variable immunodeficiency disorders: division into distinct clinical phenotypes
Název česky
Běžná variabilní imunodeficience: rozdělení odlišných fenotypů
Autoři
CHAPEL, H., M. LUCAS, M. LEE, J. BJORKANDER, D. WEBSTER, B. GRIMBACHER, C. FIESCHI, Vojtěch THON, MR. ABEDI a L. HAMMARSTROM
Vydání
Blood, 2008, 0006-4971
Další údaje
Jazyk
angličtina
Typ výsledku
Článek v odborném periodiku
Obor
30102 Immunology
Stát vydavatele
Spojené státy
Utajení
není předmětem státního či obchodního tajemství
Impakt faktor
Impact factor: 10.432
Organizační jednotka
Lékařská fakulta
UT WoS
000257696300016
Klíčová slova česky
imunodeficience; běžná variabilní imunodeficience
Klíčová slova anglicky
DISEASES CLASSIFICATION COMMITTEE; ANTIBODY DEFICIENCY; IMMUNE DEFICIENCY; B CELL; PRIMARY HYPOGAMMAGLOBULINEMIA; IMMUNOLOGICAL FEATURES; INTERNATIONAL UNION; T CELL; MANIFESTATIONS; COMPLICATIONS
Štítky
Příznaky
Mezinárodní význam, Recenzováno
Změněno: 18. 6. 2009 12:11, prof. MUDr. Vojtěch Thon, Ph.D.
V originále
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.
Česky
Běžná variabilní imunodeficience: rozdělení odlišných fenotypů
Návaznosti
NR9035, projekt VaV |
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