Article
Human leucocyte antigen-identical haematopoietic stem cell transplantation in major histocompatiblity complex class II immunodeficiency: reduced survival correlates with an increased incidence of acute graft-versus-host disease and pre-existing viral infections.
British journal of haematology - 1 Sept 2006
Renella Raffaele, Picard Capucine, Neven Bénédicte, Ouachée-Chardin Marie, Casanova Jean-Laurent, Le Deist Françoise, Cavazzana-Calvo Marina, Blanche Stéphane, Fischer Alain
Abstract excerpt
Major histocompatibility complex class II deficiency, a rare autosomal recessive primary immunodeficiency, is caused by the defective expression of human leucocyte antigen (HLA) class II molecules due to mutated trans-acting elements of any one of four regulatory genes (CIITA, RFXANK, RFX5, RFXAP). The impaired CD4 T-cell differentiation and antigen presentation in the periphery results in a severe defect of...
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