Titlebook: Bone Marrow Failure; Gary M. Kupfer,Gregory H. Reaman,Franklin O. Smith Book 2018 Springer International Publishing AG, part of Springer N

纯朴

Diamond-Blackfan Anemia,rogress using zebrafish and mouse models of DBA has advanced our understanding of the role of nucleolar stress (p53-dependent) and p53-independent mechanisms in the development of the DBA phenotype, providing strategies for therapeutic intervention. DBA is also confirmed to be a cancer predispositio

genesis

Hematopoietic Cell Transplantation for the Treatment of Patients with Bone Marrow Failure Syndromestransplantation in severe aplastic anemia, Fanconi anemia, dyskeratosis congenita, Diamond-Blackfan anemia, Shwachman-Diamond syndrome, severe congenital neutropenia, and congenital amegakaryocytic thrombocytopenia. The forthcoming role of gene therapy is also discussed.

AGATE

云状

Acquired Aplastic Anemia, majority of cases, hence the term idiopathic aplastic anemia. The major pathophysiologic mechanism implicated in acquired AA is autoimmune dysregulation resulting in damage to hematopoietic stem and progenitor cells. Hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen matc

混合,搀杂

Pediatric Myelodysplastic Syndromes,topoiesis with high risk for leukemic transformation to acute myeloid leukemia. The clinical, laboratory, and histologic presentation of pediatric MDS shares significant overlap with inherited and acquired bone marrow failure disorders, which makes the diagnosis challenging. Definitive diagnosis of

Etymology

Fanconi Anemia: A Pathway of Hematopoiesis and Cancer Predisposition, treatment delivered as multidisciplinary care across several medical and surgical specialties, and lifetime, close monitoring. While bone marrow failure is ultimately treated with bone marrow transplant, continued monitoring for squamous cell carcinoma and other cancer types is crucial due to the m

果核

Ribosomopathies Through a Diamond Lens,typical ribosomopathy, Diamond-Blackfan anemia, to explore relationships between the structure of the ribosome, its biogenesis, and the molecular mechanisms that contribute to disease pathology. Other ribosomopathies are discussed as they relate to the genes affected and pathophysiological mechanism