Myelodysplastic syndrome  內容預覽： 

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Myelodysplastic syndrome  內容簡(jiǎn)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.

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