ICOS Deficiency

  • Ulrich SalzerEmail author
Part of the Rare Diseases of the Immune System book series (RDIS)


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.


ICOS T cells Costimulation CVID B cells 


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Faculty of Medicine, Department of Rheumatology and Clinical ImmunologyMedical Center—University of FreiburgFreiburg im BreisgauGermany
  2. 2.Faculty of Medicine, Center for Chronic Immunodeficiency (CCI)Medical Center—University of FreiburgFreiburg im BreisgauGermany

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