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ICOS Deficiency

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Humoral Primary Immunodeficiencies

Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Inducible costimulator (ICOS) deficiency is an autosomal recessive inherited primary immunodeficiency and was the first monogenetic defect described in common variable immunodeficiency. ICOS-deficient patients have high rates of immunedysregulatory complications, and bacterial, viral and opportunistic infections. Immunoglobulin substitution and antibiotic treatment of breakthrough infections are the mainstays of treatment in ICOS deficiency. In cases of conventional treatment failure, hematopoietic stem cell transplantation may be considered.

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Authors and Affiliations

  1. Faculty of Medicine, Department of Rheumatology and Clinical Immunology, Medical Center—University of Freiburg, Freiburg im Breisgau, Germany

    Ulrich Salzer

  2. Faculty of Medicine, Center for Chronic Immunodeficiency (CCI), Medical Center—University of Freiburg, Freiburg im Breisgau, Germany

    Ulrich Salzer

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  1. Ulrich Salzer
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Correspondence to Ulrich Salzer .

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Editors and Affiliations

  1. Careggi Hospital, Department of Clinical and Experimental Medicine, University of Florence, Firenze, Italy

    Mario Milco D'Elios

  2. University Medical Centre Freiburg, Department of Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency, Freiburg, Germany

    Marta Rizzi

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Salzer, U. (2019). ICOS Deficiency. In: D'Elios, M., Rizzi, M. (eds) Humoral Primary Immunodeficiencies. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-91785-6_6

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  • DOI: https://doi.org/10.1007/978-3-319-91785-6_6

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-91784-9

  • Online ISBN: 978-3-319-91785-6

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