再生障碍性贫血医学宣讲谁能拯救这个16岁的再生障碍性贫血少年？教学目的与要求概述WilliamsHematology6th流行病学：病因学Immunedestructionofhematopoiesis分型发病机制：临床表现2、非重型再障(NSAA)贫血慢性过程感染上呼吸道感染常见、其次牙龈炎、扁桃体炎，病原菌为革兰阴性杆菌和各类球菌出血以皮肤粘膜为主分型（国内）：21国外标准232425不典型再障诊断中应注意的问题实验室检查实验室检查31323334诊断与鉴别诊断鉴别诊断：全血细胞减少40PNH与AA自身抗体介导的全血细胞减少急性造血功能停滞急性白血病除外引起全血细胞减少的其它疾病再障分型诊断标准1、SAA-Ⅰ起病急，贫血、出血、感染严重，血象符合下列两条网织红细胞绝对值<15×109/L中性粒细胞绝对值<0.5×109/L血小板<20×109/L骨髓增生广泛重度减低，如SAA-Ⅰ的中性粒细胞<0.2×109/L称极重型再障2、NSAA未达到以上标准如NSAA病情恶化，达到以上标准称SAA-Ⅱ自身抗体介导的全血细胞减少可检测到自身抗体，网红不降低急性造血功能停滞骨髓尾部有巨大原红细胞，1月可恢复急性白血病(acuteleukemia)恶性组织细胞病(malignanthistocytosis)治疗：重型再障的治疗不主张应用的治疗方法再障的治疗选择英国血液学标准委员会2003年龄≤30~40y>40y有HLA相合同胞<20y≥20~40yIST(ATG+CsA)3月评价疗效BMTBMT/IST有效无效CsA维持至少2ndIST6月，渐减量3月评价疗效有效无效年龄≥40y年龄<40y3rdIST;雄激素;G-CSF无关供者BMT临床试验，支持治疗HLA精确配型预防预后summaryBeforemarrowtransplantationandimmunosuppressivetherapy,morethan25percentofthepatientsdiedwithin4monthsofdiagnosis;halfsuccumbedwithin1year.Marrowtransplantationishighlysuccessfulandcurativefor75to85percentofuntransfusedpatientsandfor55to60percentofthosewithmultipleprevioustransfusions.Unfortunately,asmanyas20to30percentoftransplantsurvivorssufferthedeleteriousconsequencesofseveregraft-versus-hostdisease.Immunosuppressivetherapyleadstoamarkedimprovementinabout50to70percentofthepatients;somearehematologicallynormal,butmanycontinuewithmoderateanemiaorthrombocytopenia.Relapseoccursinabout15percentofpatients,andindeedtheunderlyingstemcelldefectmayprogressover10yearstoparoxysmalnocturnalhemoglobinuria,amyelodysplasticsyndrome,oracutemyelogenousleukemiainasmanyas40percentofinitialresponderstoimmunosuppressivetherapy.