Topic
Common variable immunodeficiency
About: Common variable immunodeficiency is a research topic. Over the lifetime, 2864 publications have been published within this topic receiving 76328 citations. The topic is also known as: CVID & acquired agammaglobulinemia.
Papers published on a yearly basis
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Papers
TL;DR: In this article, the authors describe the clinical and immunological status of 248 consecutively referred CVI patients of age range 3-79 years who have been followed for a period of 1-25 years.
1,552 citations
TL;DR: The clinical evaluation of 303 patients with the established diagnosis of CVID demonstrated a significant coincidence of granulomatous disease, autoimmune cytopenia, and splenomegaly, and an improved classification for CVID (EUROclass), separating patients with nearly absent B cells, and expansion of transitional or CD21(low) B cells.
847 citations
TL;DR: The European Common Variable Immunodeficiency Disorders registry was started in 1996 to define distinct clinical phenotypes and determine overlap within individual patients and analysis of mortality showed a considerable reduction in the last 15 years and that different phenotypes were associated with different survival times.
788 citations
TL;DR: The phenotype of human ICOS deficiency, which differs in key aspects from that of the ICOS−/− mouse, suggests a critical involvement of ICOS in T cell help for late B cell differentiation, class-switching and memory B cell generation.
Abstract: No genetic defect is known to cause common variable immunodeficiency (CVID), a heterogeneous human disorder leading to adult-onset panhypogammaglobulinemia. In a search for CVID candidate proteins, we found four of 32 patients to lack ICOS, the "inducible costimulator" on activated T cells, due to an inherited homozygous deletion in the ICOS gene. T cells from these individuals were normal with regard to subset distribution, activation, cytokine production and proliferation. In contrast, naive, switched and memory B cells were reduced. The phenotype of human ICOS deficiency, which differs in key aspects from that of the ICOS-/- mouse, suggests a critical involvement of ICOS in T cell help for late B cell differentiation, class-switching and memory B cell generation.
773 citations
TL;DR: Mortality was associated with lymphoma, any form of hepatitis, functional or structural lung impairment, and gastrointestinal disease with or without malabsorption, but not with bronchiectasis, autoimmunity, other cancers, granulomatous disease, or previous splenectomy.
770 citations
Related Topics (5)
Performance Metrics
| Year | Papers |
|---|---|
| 2025 | 41 |
| 2024 | 145 |
| 2023 | 142 |
| 2022 | 254 |
| 2021 | 162 |
| 2020 | 167 |