Hematopoietic stem cell transplantation and other management strategies for MHC class II deficiency.

Major histocompatibility complex (MHC) class II expression deficiency is a rare condition with autosomal recessive transmission. The defect of MHC class II leads to combined immunodeficiency with defective CD4(+) T-cell development and a lack of T helper cell-dependent antibody production by B cells. The clinical course of disease is characterized by the recurrence of bacterial, viral, fungal, and protozoan infections. The optimal symptomatic care that is available involves the prophylactic use of antibiotics and the administration of immunoglobulin with adequate nutritional support. Hematopoietic stem cell transplantation is the only known treatment available to cure MHC class II expression deficiency.