mds的诊断与治疗课件

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1、中国医学科学院血液学研究所血液病医院秦铁军电话：13332095356骨髓增生异常综合征Friday,June25,2021MDS的定义与最低诊断标准定义agroupofclonalhaematopoieticstemcelldiseasescharacterizedbycytopenia(s).dysplasiainoneormoreofthemajormyeloidcelllines.IneffectivehaematopoiesisIncreasedriskofdevelopmentofAMLMonday,August09,2010thresholdsforcytopeniasC

2、ytopeniasHaemoglobin<100g/LAbsoluteneutrophilcount(ANC)<1.8xl09/LPlatelets<100xl09/LDefinitivemorphologicand/orcytogeneticfindingssupportthediagnosisofMDSevenifvaluesabovethesethresholdsMonday,August09,2010流行病学(Epidemiology)Medianage70yearsincidenceof3-5/100000persons>20/100000amongthoseoverthea

3、geof70yearsmalepredominanceClinicalfeaturesSymptomsrelatedtocytopenia(s)Anemicandtransfusion-dependentNeutropeniaand/orthrombocytopenia--LessfrequentInfrequentlyorganomegalyMonday,August09,2010ThemorphologicalofMDSprincipallybasedonpercentofblastsintheBMandPBtypeanddegreeofthedysplasiapresenceof

4、ringsideroblasts500-celldifferentialofallnucleatedcellsintheBMsmear200-leukocytedifferentialinthePBnecessityofhighqualityslidepreparationsandthestainMonday,August09,2010骨髓和外周血涂片高质量的制片和染色新鲜标本(抗凝标本不应超过2小时)计数500个细胞粒红比≤1:1时计数500个非红系细胞，不包括淋巴细胞、浆细胞、肥大细胞原始细胞比例应为非红系比例环形铁粒幼细胞比例>15%时，即认为是红系发育异常Monday,Augu

5、st09,2010Morphologicmanifestationofdysplasia(1)DyserythropoiesisNuclear核出芽(nuclearbudding)核间桥(internuclearbridging)核破裂(Karyorrhexis)多核(Multinuclearity)核多分叶(nuclearhyperlobation)巨幼样变(megaloblasticchanges)Cytoplasmic环状铁粒幼红细胞(Ringsideroblast)空泡形成(Vacuolization)糖原染色阳性(Periodicacid-Schiffpositivity)M

6、onday,August09,2010Morphologicmanifestationofdysplasia(2)Dysgranulopoiesis细胞体积过小或过大(Smallorunusuallylargesize)核少分叶Nuclearhypolobation(pseudoPelger-Huet;pelgeroid)不规则的分叶过多(Irregularhypersegmentation)少颗粒(Decreasedgranules;agranularity)PseudoChediak-Higashigranules奥氏小体AuerrodsMonday,August09,2010Mo

7、rphologicmanifestationofdysplasia(3)Dysmegakaryocytopoiesis小巨核细胞(Micromegakaryocytes)核少分叶(Nuclearhypolobationinallsize)Mutinucleation(normalmegakaryocytesareuninucleatewithlobulatednuclei)Multiple,widely-separatednucleiMorer