Bone marrow transplantation does not correct the hyper IgE syndrome

Abstract

Summary:Congenital immunodeficiency in hyper IgE syndrome is characterised by a markedly raised IgE level, recurrent staphylococcal skin infection and pneumatoceles. Standard treatments include anti-staphylococcal antibiotics. We report a severely affected patient in whom successful bone marrow transplantation was followed by reappearance of the immunodeficiency. We conclude that bone marrow transplantation does not cure the immunological features of the hyper IgE syndrome. Bone Marrow Transplantation (2000) 25, 1303-1305.

Insights into the Role of STAT3 in Human Lymphocyte Differentiation as Revealed by the Hyper-IgE Syndrome

“…Instead, the magnitude of the IgE increase in AD-HIES suggests a feed-forward loop involving cooperation between multiple cell types, such as those of the immune system and the skin. Indeed, a role for nonhematopoietic cells in the dysregulated production of IgE is consistent with the finding that bone marrow transplantation failed to correct the hyper-IgE phenotype of classical AD-HIES (86).…”

Cytokine and Chemokine Dysregulation in Hyper-IgE Syndrome

“…As an autosomal dominant disorder, a mutation on a single chromosome would have to be sufficient to impair production of multiple genes involved in the inflammatory process. The involvement of chemokines, which are produced by a range of nonhematopoietic cells, may explain why bone marrow transplantation has been ineffective in this syndrome (32).…”

NF-κB and STAT3 signaling hubs for lung innate immunity

“…Whether Th17 cells specifically are important for lung host defense, or whether HIES patients have defects in IL-17 or IL-22 in their infected lungs is unclear. Mice deficient in STAT3 in all T cells or all CD4+ cells do not have a phenotype resembling HIES (Takeda et al 1998;Harris et al 2007), and bonemarrow transplantation in an HIES patient has failed to prevent immunodeficiency and infections (Gennery et al 2000), results that, when taken together, suggest that STAT3 has roles beyond the development of Th17-like cells, which are essential to preventing lung infections and the resultant lung disease characteristic of HIES patients.…”