1、LOGOLOGODefinition Aplastic anemia is a life-threatening syndrome characterized by failure of the bone marrow to produce peripheral blood cells and their progenitors.2再生障碍性贫血英文LOGOEtiology and pathogenesisThe major causes for aplastic anemia:1.Infections(e.g.Hepatitis,Epstein-Barr virus and Parvovir
2、us B19).2.Radiation X rays,rays 3.Chemicals:benzene 4.Drugs:Chloramphenicol(氯霉素)3再生障碍性贫血英文LOGOImmune-Mediated injury is the main pathogenesis.In addition,absent or defective hematopoietic stem cells and abnormal bone marrow microenviroment can also cause aplastic anemia.4再生障碍性贫血英文LOGOClinical manife
3、station and classification Clinical manifestation Anemia,Bleeding and Infections are common symptoms Classification1.Severe Aplastic anemia（SAA）2.Non-severe Aplastic anemia（NSAA）5再生障碍性贫血英文LOGOPhysical Examination 1.Petechiae and ecchymoses are typical.2.Pallor of the skin and mucous membranes is com
4、mon.6再生障碍性贫血英文LOGOLaboratory Studies1.Blood Smear:Pancytopenia(anemia,leukopenia,and thrombocytopenia)is a universal presenting finding.SAA blood smearNormal peripheral blood smear7再生障碍性贫血英文LOGO2.bone marrow biopsy:Biopsy is superior for determination of cellularity and shows mainly fat under the mi
5、croscope,with hematopoietic cells occupying 25%of the marrow space;in the most serious cases the biopsy is virtually 100%fat.It is required to establish the diagnosis.8再生障碍性贫血英文LOGOA.Normal bone marrow biopsy.This specimen shows an abundance of hematopoietic cells,including Myeloid and erythroid pre
6、cursors and Normal-appearing megakaryocytesB.Aplastic anemia biopsy.This specimen shows few detectable hematopoietic cells,and those that can be seen(one small nest)are largely lymphocytes.9再生障碍性贫血英文LOGO3.Bone marrow smear The bone marrow is usually readily aspirated but dilute on smear,and the fatt
7、y biopsy specimen may be grossly pale on withdrawal;In severe aplasia the smear of the aspirated specimen shows only red cells,residual lymphocytes,and stromal cells.10再生障碍性贫血英文LOGODD.Marrow smear in aplastic anemia.The marrow shows replacement of hematopoietic tissue by fat and only residual stroma
8、l and lymphoid cells.C.Normal bone marrow aspirate smear.The marrow is normally 3070%cellular,and there is a heterogeneous mix of myeloid,erythroid,and lymphoid cells.C11再生障碍性贫血英文LOGOReference standard of diagnosis for AA 1.Peripheral blood pancytopenia,reticulocyte percentage less than 0.01 and the
9、 ratio of Lymphocyte is increased.2.Generally,without the liver and spleen enlargement.3.At least one area of hypoplasia in bone marrow.Non-hematopoietic cells increase.4.Elimination of other diseases which may cause pancytipenia such as PNH,MDS,acute arrest of hemopoiesis,etc.5.Common therapy for a
10、nemia is ineffective.12再生障碍性贫血英文LOGOSAA laboratory criteria To define SAA there must be at least two of the following:(1)absolute neutrophil count less than 0.5 109/L,(2)platelet count less than 20 109/L,or(3)reticulocytes less than 20 109/L.Non-severe AA:Patients not fulfilling the criteria for sev
11、ere aplastic anaemia.13再生障碍性贫血英文LOGOTreatmentHematopoietic Stem Cell Transplantation This is the best therapy for the young patient with a fully histocompatible sibling donor.ImmunosuppressionSupportive CareOther Therapies14再生障碍性贫血英文LOGOA pathway for the diagnostic management of patients with aplastic anemia15再生障碍性贫血英文LOGO16再生障碍性贫血英文
