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Myelodysplastic syndrome overview

2013-11-08 11:34 閱讀:1455 來源:愛愛醫(yī) 作者:7****i 責(zé)任編輯:78322li
[導(dǎo)讀] 【專家課件】 Myelodysplasticsyndrome 內(nèi)容預(yù)覽: (點擊下圖可對專家課件進(jìn)行全文預(yù)覽) 【專家課件】 Myelodysplasticsyndrome 內(nèi)容簡介: Myelodysplasticsyndromeoverview RazelleKurzrock SeminarsinHaematology,Vol39,No3,Suppl2(July)2002,pp18-25 M

Myelodysplastic syndrome  內(nèi)容預(yù)覽: 

(點擊下圖可對專家課件進(jìn)行全文預(yù)覽)

Myelodysplastic syndrome  內(nèi)容簡介:

Myelodysplasticsyndromeoverview
RazelleKurzrock
SeminarsinHaematology,Vol39,No3,Suppl2(July)2002,pp18-25

Myelodysplasticsyndrome(MDS)
Itisatermforaheterogeneouscollectionofhaemopoieticstemcelldisordersaffectingolder**s.
Thereisunderlyingineffectivenessofhaemopoiesisthatresultsindysplasiaofbonemarrowprecursorsandpe**heralcytopenias.

ModerateanaemiaisthemostcommonclinicalprobleminMDSpatients,butcompletemyeloidbonemarrowfailurealsooccursleadingtodeathfrombleedingorinfection.
ApproximatelyhalfofthepatientstransformtoAML.

Prognosisdependsontheindividual’sriskfactors,withmediansurvivalrangingfrom5.7yearsinlower-riskgroupto1.2yearsorlessinthosewithhigher-riskMDS.
MDSisextremelydifficulttotreat.Mostcasesareresistanttocurrenttherapies,andthemostpotentanti-MDStreatments(transplantationanddoseintensivechemotherapy)areoftentootoxicforthemajorityofpatients.

MDSbackground
Pathobiology
ThecardinalfeaturesofMDSare
Increasedmarrowproliferation
Failureofstemcellstodifferentiate
Andincreasedmarrowapoptosis.
Thediseaseisofclonalorigin
Chromosomalabnormalitiesaredetectablein30-70%ofpatients.Theno.ofchromosomalabn.maycorrelatewiththeriskofprogressiontoAML.

FABclassification
In1982TheFABgroupclassifiedMDSaccordingtoMorphologyandthe%ofmyeloblastsintheBMandPB.
Theseincluded
Refractoryanaemia(RA)
Refractoryanaemiawithringedsideroblasts(RARS)
Refractoryanaemiawithexcessblastinmarrow(RAEB)
CMML
Refractoryanaemiawithexcessblastintransformation
(RAEB-t)

WHOclassification
TheWHOproposedchangesincludingreclassificationofRAEB-ttoAMLandaddingasubgroupcalledrefractorycytopeniaswithdysplasia(RCD)

InternationalPrognosticScoringSystem(IPSS)
ThemostpracticalandvalidatedMDSclassificationsystemcurrentlyavailabletocliniciansistheIPSSwhichpredictsbothsurvivalandriskoftransformationtoAMLbasedon:
Marrowblast%
Cytogenetics
Andnumberofcytopenias.

Myelodysplastic syndrome  ***下載 

 


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