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Chapter 15
Marrow failure syndromes
Bone marrow failure refers to the inability of hematopoiesis to meet physiologic demands for production of healthy blood cells. Pancytopenia may result from marrow failure, or cytopenias involving a single myeloid lineage may dominate; usually lymphopoiesis is relatively preserved.
The causes of marrow failure are diverse and may either be extrinsic to the marrow, as in the disordered immune response that characterizes aplastic anemia, or intrinsic, as in the hematopoietic progenitor or stem cell defects that underlie the myelodysplastic syndromes. Bone marrow failure syndromes can be acquired or, more rarely, congenital.
The range of molecular mechanisms responsible for congenital marrow
Congenital bone marrow failure syndromes
Fanconi anemia Epidemiology Pathobiology Clinical features and diagnosis of FA Treatment Diamond-Blackfan anemia Clinical features Pathobiology Treatment Congenital dyserythropoietic anemias General clinical features CDA type I CDA type II Other CDA types Dyskeratosis congenita Clinical features Pathobiology Treatment Congenital amegakaryocytic thrombocytopenia Clinical features Pathobiology Treatment
Acquired bone marrow failure conditions
Aplastic anemia Definition Epidemiology, etiology, and pathogenesis Clinical presentation Differential diagnosis Therapy Supportive care, transfusions, and hematopoietic growth factors Bone marrow transplantation Immunosuppressive therapy Immunosuppression versus bone marrow transplantation Late clonal complications Paroxysmal nocturnal hemoglobinuria Definition Pathophysiology Hemolysis Thrombosis Bone marrow failure and evolution of PNH Laboratory findings and diagnosis Clinical manifestations Treatment Prognosis Myelodysplastic syndromes Introduction and classification Epidemiology and etiology Diagnosis Prognosis Cytogenetics and molecular biology Cell biology Hypocellular MDS Idiopathic cytopenias of undetermined significance Treatment of MDS Supportive care: transfusions and iron chelation Growth factors Hypomethylating agents Histone deacetylase inhibitors Immunomodulatory drugs Immunotherapy Allogeneic HSCT   CiteULike  Connotea  Delicious  Digg  Facebook  Reddit  Technorati  Twitter What's this?
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