Autoimmune and Lymphoproliferative Complications of Common Variable Immunodeficiency

  • Paul J. MaglioneEmail author
Immune Deficiency and Dysregulation (DP Huston and C Kuo, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Immune Deficiency and Dysregulation


Common variable immunodeficiency (CVID) is frequently complicated by the development of autoimmune and lymphoproliferative diseases. With widespread use of immunoglobulin replacement therapy, autoimmune and lymphoproliferative complications have replaced infection as the major cause of morbidity and mortality in CVID patients. Certain CVID complications, such as bronchiectasis, are likely to be the result of immunodeficiency and are associated with infection susceptibility. However, other complications may result from immune dysregulation rather than immunocompromise. CVID patients develop autoimmunity, lymphoproliferation, and granulomas in association with distinct immunological abnormalities. Mutations in transmembrane activator and CAML interactor, reduction of isotype-switched memory B cells, expansion of CD21 low B cells, heightened interferon signature expression, and retained B cell function are all associated with both autoimmunity and lymphoproliferation in CVID. Further research aimed to better understand that the pathological mechanisms of these shared forms of immune dysregulation may inspire therapies beneficial for multiple CVID complications.


Common variable immunodeficiency Autoimmunity Cytopenia Granulomatous disease Lymphoid hyperplasia 


Compliance with Ethical Standards

Conflict of Interest

Dr. Maglione declares no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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© Springer Science+Business Media New York 2016

Authors and Affiliations

  1. 1.Division of Clinical Immunology, Department of MedicineIcahn School of Medicine at Mount SinaiNew YorkUSA

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