aplastic anemia概要1.ppt

* * Aplastic anemia Hematological Department, the 1st Affiliated Hospital of Harbin Medical University Shuchuan Liu Email: lsc1972@163.com Phone: Defintion Aplastic anemia (AA) is defined as pancytopenia with hypocellularity of the bone marrow; there are no leukemic, cancerous or other abnormal cells in the peripheral blood or bone marrow. Etiology Idiopathic: autoimmune inherited: Radiation Chemotjerapy Toxins (benzene, toluene, insecticides) Pharmacologic agents (chloramphenicol phenytoin, quinacrine and tolbutamide). Pathogenesis Autoimmune suppression of hematopoiesis by T cell-mediated celluar mechanism. Clinical features Anemia: weakness, failure Neutropenia:bacterial infection Thrombocytopenia: mucoasal and skin bleeding. Other abnormalities: hepatosplenomegaly, lymphadenopathy or bone tenderness should not be present. Laboratory finding Blood: Pancytopenia Reticulocyte: reticulocyte decreased Neutrophils and platelets are reduced in number Bone marrow and bone marrow biopsy Hypocellular Hematopoietic progenitors decreased No abnormal cells are seen Laboratory finding Peripheral blood Bone marrow Laboratory finding bone marrow biopsy Normal Aplastic anemia Differential diagnosis Aplastic anemia must be differential from other cause of pancytopenia including: Myelodysplasia Acute leukemia Myelofibrosis Hairy cell leukemia Megaloblastic anemia Hepersplenism Systemic lupus erythematosus Infection Tuberculosis and AIDS Treatment Supportive care: red blood cell transfusion and platelet transfusion are given as necessary and antibiotics are used to treat infections. Mild aplastic anemia Severe aplastic anemia: age50 years who have HLA-matched siblings is allogeneic bone marrow transplantation. age﹥50 not eligible for bone marrow transplantation. age﹥50 or those without HLA-matched siblings is immunosuppression with antithymoc