Successful long-term immunologic reconstitution by allogeneic hematopoietic stem cell transplantation cures patients with autosomal dominant hyper-IgE syndrome
E Goussetis, I Peristeri, V Kitra… - Journal of Allergy and …, 2010 - jacionline.org
To the Editor: The hyper-IgE syndromes (HIESs) are rare primary immune deficiencies
characterized by increased serum IgE levels, eczematous rash, and recurrent skin and lung
infections. Both autosomal recessive and autosomal dominant inheritance have been
described, but most HIES cases are sporadic. 1-3 Autosomal dominant HIES (AD-HIES), the
most common form, has various immunologic and nonimmunologic abnormalities arising as
consequences of impaired cytokine signal transduction and TH17 cell deficiency caused by …
characterized by increased serum IgE levels, eczematous rash, and recurrent skin and lung
infections. Both autosomal recessive and autosomal dominant inheritance have been
described, but most HIES cases are sporadic. 1-3 Autosomal dominant HIES (AD-HIES), the
most common form, has various immunologic and nonimmunologic abnormalities arising as
consequences of impaired cytokine signal transduction and TH17 cell deficiency caused by …
