Correction of the Hyper-IgM Syndrome after Liver and Bone Marrow Transplantation

Abstract

The hyper-IgM syndrome, a rare form of combined primary immunodeficiency, is characterized by neutropenia and defective B-cell isotype switching, which results in elevated or normal levels of serum IgM and low levels of serum IgG, IgA, and IgE.1 Clinically, patients with the syndrome have serious pyogenic infections caused by encapsulated bacteria, suggestive of the presence of humoral immunodeficiency. They are also susceptible to infections with intracellular pathogens such as Pneumocystis carinii, Cryptosporidium parvum, and leishmania, because of a possible defect in cellular immunity.2,3 The inheritance is usually X-linked, but autosomal recessive and autosomal dominant forms have also been documented. . . .