HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Common Variable Immunodeficiency

Heterogeneous group of immunodeficiency syndromes characterized by hypogammaglobulinemia of most isotypes, variable B-cell defects, and the presence of recurrent bacterial infections.
Also Known As:
Common Variable Hypogammaglobulinemia; Common Variable Immune Deficiency; Hypogammaglobulinemia, Acquired; Immunoglobulin Deficiency, Late-Onset; Acquired Hypogammaglobulinemia; Acquired Hypogammaglobulinemias; Common Variable Hypogammaglobulinemias; Common Variable Immunodeficiencies; Deficiencies, Late-Onset Immunoglobulin; Deficiency, Late-Onset Immunoglobulin; Hypogammaglobulinemia, Common Variable; Hypogammaglobulinemias, Acquired; Hypogammaglobulinemias, Common Variable; Immunodeficiencies, Common Variable; Immunoglobulin Deficiencies, Late-Onset; Immunoglobulin Deficiency, Late Onset; Late-Onset Immunoglobulin Deficiencies; Late-Onset Immunoglobulin Deficiency; Variable Hypogammaglobulinemia, Common; Variable Hypogammaglobulinemias, Common; Immunodeficiency, Common Variable
Networked: 1342 relevant articles (25 outcomes, 72 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Infections
2. COVID-19
3. Severe Combined Immunodeficiency (Bare Lymphocyte Syndrome)
4. Bruton type agammaglobulinemia
5. Hyperplasia

Experts

1. Cunningham-Rundles, Charlotte: 35 articles (01/2022 - 06/2002)
2. Aghamohammadi, Asghar: 31 articles (02/2022 - 09/2003)
3. Rezaei, Nima: 26 articles (01/2020 - 01/2004)
4. Grimbacher, Bodo: 18 articles (01/2021 - 12/2005)
5. Quinti, Isabella: 16 articles (01/2022 - 04/2003)
6. Aukrust, Pål: 15 articles (10/2022 - 06/2003)
7. Spadaro, Giuseppe: 15 articles (01/2021 - 05/2007)
8. Plebani, Alessandro: 14 articles (09/2021 - 03/2003)
9. Radigan, Lin: 13 articles (10/2021 - 05/2005)
10. Fevang, Børre: 12 articles (10/2022 - 02/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Common Variable Immunodeficiency:
1. Immunoglobulins (Immunoglobulin)IBA
2. Intravenous Immunoglobulins (IVIG)FDA Link
3. Rituximab (Mabthera)FDA Link
4. Immunoglobulin G (IgG)IBA
5. AntibodiesIBA
6. AntigensIBA
7. VaccinesIBA
8. T-Cell Antigen Receptors (T-Cell Receptor)IBA
9. Cimetidine (Biomet)FDA LinkGeneric
10. Proteins (Proteins, Gene)FDA Link

Therapies and Procedures

1. Therapeutics
2. Splenectomy
3. Hematopoietic Stem Cell Transplantation
04/01/2015 - "Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency."
01/01/2021 - "Clinical features of a 3-month-old male infant who underwent hematopoietic stem cell transplantation because of an IKZF1 mutation-associated common variable immunodeficiency, AIHA, and pancytopenia are described. "
03/19/2007 - "These new insights will support the understanding of peripheral B cell regeneration after hematopoietic stem cell transplantation or B cell-directed antibody therapy, and the identification of defects in homeostatic or antigen-induced B cell proliferation in patients with common variable immunodeficiency or another antibody deficiency."
10/01/2018 - "These include (1) the role of follicular T cells in the differentiation of B cells and development of optimal antibody responses; (2) impaired nuclear factor κB subunit 1 signaling in the pathogenesis of common variable immunodeficiency, revealing an association between impaired B-cell maturation and development of inflammatory conditions; (3) autoimmune and inflammatory manifestations in patients with PIDDs in T- and B-cell deficiencies, as well as in neutrophil disorders; (4) newly described gene defects causing PIDDs, including exostosin-like 3 (EXTL3), TNF-α-induced protein 3 (TNFAIP3 [A20]), actin-related protein 2/3 complex-subunit 1B (ARPC1B), v-Rel avian reticuloendotheliosis viral oncogene homolog A (RELA), hypoxia upregulated 1 (HYOU1), BTB domain and CNC homolog 2 (BACH2), CD70, and CD55; (5) use of rapamycin and the phosphoinositide 3-kinase inhibitor leniolisib to reduce autoimmunity and regulate B-cell function in the activated phosphoinositide 3-kinase δ syndrome; (6) improved outcomes in hematopoietic stem cell transplantation for severe combined immunodeficiency (SCID) in the last decade, with an overall 2-year survival of 90% in part caused by early diagnosis through implementation of universal newborn screening; (7) demonstration of the efficacy of lentiviral vector-mediated gene therapy for patients with adenosine deaminase-deficient SCID; (8) the promise of gene editing for PIDDs using CRISPR/Cas9 and zinc finger nuclease technology for SCID and chronic granulomatous disease; and (9) the efficacy of thymus transplantation in Europe, although associated with an unexpected high incidence of autoimmunity. "
4. Transplantation
01/01/2018 - "[Successful cord blood transplantation in a patient with adult-onset common variable immunodeficiency]."
09/01/1998 - "Five patients had acquired immunodeficiency syndrome (AIDS); one had common variable immune deficiency; and 18 were receiving immunosuppressive therapy for heart, lung, or heart-lung (n =15) or bone marrow (n = 2) transplantation and vasculitis (n = 1). "
01/01/2023 - "Recipients transplanted for bronchiectasis in the context of a primary immune deficiency, such as common variable immunodeficiency, are at a high risk of severe infection in post-transplantation leading to poorer long-term outcomes than other transplant indications. "
10/01/2018 - "These include (1) the role of follicular T cells in the differentiation of B cells and development of optimal antibody responses; (2) impaired nuclear factor κB subunit 1 signaling in the pathogenesis of common variable immunodeficiency, revealing an association between impaired B-cell maturation and development of inflammatory conditions; (3) autoimmune and inflammatory manifestations in patients with PIDDs in T- and B-cell deficiencies, as well as in neutrophil disorders; (4) newly described gene defects causing PIDDs, including exostosin-like 3 (EXTL3), TNF-α-induced protein 3 (TNFAIP3 [A20]), actin-related protein 2/3 complex-subunit 1B (ARPC1B), v-Rel avian reticuloendotheliosis viral oncogene homolog A (RELA), hypoxia upregulated 1 (HYOU1), BTB domain and CNC homolog 2 (BACH2), CD70, and CD55; (5) use of rapamycin and the phosphoinositide 3-kinase inhibitor leniolisib to reduce autoimmunity and regulate B-cell function in the activated phosphoinositide 3-kinase δ syndrome; (6) improved outcomes in hematopoietic stem cell transplantation for severe combined immunodeficiency (SCID) in the last decade, with an overall 2-year survival of 90% in part caused by early diagnosis through implementation of universal newborn screening; (7) demonstration of the efficacy of lentiviral vector-mediated gene therapy for patients with adenosine deaminase-deficient SCID; (8) the promise of gene editing for PIDDs using CRISPR/Cas9 and zinc finger nuclease technology for SCID and chronic granulomatous disease; and (9) the efficacy of thymus transplantation in Europe, although associated with an unexpected high incidence of autoimmunity. "
5. Aftercare (After-Treatment)