内科学再生障碍性贫血(英文).ppt

HLA-identical transplantation 75~90% long term cure GVHD: acute ? 18% chronic ? 26% Indication: 1. SAA or VSAA 2. Age 30~40 years old HLA: histocompatibility leukocyte antigen * HLA-identical transplantation Conditioning regimen Cyclophosphamide ATG Prednisolone Post-transplantation medication Cyclosporin for 9~12 months MTX ? short term use * STRATEGY OF AA PATHOGENIC THERAPY SAA SCTs: For Younger patients with Suitable doner,Enough money and Families consent IST+HS: For those unsuitable to SCTs ATG/ALG+CSA+Androgen ? HGFs CSA ? HD-IVIg+Androgen ? HGFs * Algorithms for Treatment of Acquired Aplastic Anemia -SAA * STRATEGY OF AA PATHOGENIC THERAPY NSAA Androgen Chinese Herb Medicine Angrogen+CSA Androgen+CSA+HGFs * Algorithms for Treatment of Acquired Aplastic Anemia -NSAA * Thank You！ * * * * Aplastic anemia * CONTENTS INTRODUCTION ETIOLOGY /PATHOGENESIS MANIFESTATIONS LABORATORY FINDINGS DIAGNOSIS DIFFERENTIAL TREATMENT * Aplastic anemia(AA) Definition AA , an unusual hematologic disease, is the paradigm of the human bone marrow failure syndromes. -marrow hypoplasia -peripheral pancytopenia Incidence: 7.4 per million population East West Biphasic peak at 20-25 and 60 y * Causes: Inherited (20%) Fanconi anemia Dyskeratosis congenita Cartilage hair hypoplasia Pearson syndrome Amegakaryocytic thrombocytopenia Shwachman-Diamond syndrome Dubowitz syndrome Diamond-Blackfan syndrome Familial aplastic anemia * * Causes: Acquired (80%) Infections: hepatitis, EBV, HIV, parvovirus Radiation and chemicals such as benzene Drugs: chloramphenicol, phenylbutazone ,gold PNH (paroxysmal nocturnal hemoglobinuria) Transfusional graft versus host disease Liver transplantation Pregnancy * Pathogenesis Depletion of hemopoietic stem cell Suppression of proliferation and maturation Mechanism -Immune related -Cyto