RAC2 loss-of-function mutation in 2 siblings with characteristics of common variable immunodeficiency.

Alkhairy, Omar K; Rezaei, Nima; Graham, Robert R; Abolhassani, Hassan; Borte, Stephan; Hultenby, Kjell; Wu, Chenglin; Aghamohammadi, Asghar et al. · J Allergy Clin Immunol · 2015

case_report · Level V

Where this comes from

Abstract

We report the first patients with a homozygous loss of function mutation in the <i>RAC2</i> gene, presenting with clinical features of common variable immunodeficiency. In addition, the patients suffered from glomerulonephritis, coagulopathy, multiple hormone deficiencies potentially on the autoimmune basis and abnormalities of neutrophil granules.

Medical subject headings