Author:
de Latour Régis Peffault,Risitano Antonio,Kulasekararaj Austin,Dufour Carlo
Abstract
AbstractSevere aplastic anemia (SAA) is an autoimmune disorder due to the attack of autoreactive cytotoxic T lymphocytes to the hematopoietic component of the bone marrow. SAA might be associated with PNH, resulting from the clonal expansion of hematopoietic stem cells that have somatic mutations in the X-linked gene PIG-A. This review is summarizing the management of SAA from the diagnosis, where inherited disorders need to be excluded, to the overall treatment algorithm of this disease, including immunosuppression and bone marrow transplantation.
Publisher
Springer International Publishing