慢性粒单核细胞白血病诊治进展VIP

.,CMML诊治进展,江苏省人民医院血液科洪鸣,.,.,.,Contents,.,Definition,.,WHOClassificationofMDS/MPN,.,.,Diagnosis,.,Clinicalmanifestation,MDS-typeFatigueanddyspneaduetoanemiasusceptibilitytoinfectionsrarelybleedingMPN-typesignificantweightlossdrenchingnighsweatsleftupperquadrantpainfromsignificantsplenomegaly,.,.,.,Monocytosiswithmorphologicallynormalmonocytes(PB),MonocyteswithnuclearandCytoplasmicabnormalities(PB),CMML-1(BM),CMML-2(BM),RepresentativeperipheralbloodandBMsmearsdistinctionbetweenpromonocytesandabnormalmonocytesmaybeproblematic,Promonocytestypicallyhavealight-graycytoplasmwithafewlilac-coloredgranulesandastipplednuclearchromatin.,Abnormalmonocyteshavedenserchromatin,nuclearconvolutionsandfoldsandamoregreyishcytoplasm.,.,.,Histopathology,.,.,Chromosomalabnormalities,NospecificcytogeneticalterationshavebeenidentifiedinpatientswithCMML.Someofthemorefrequentlyreportedrecurringabnormalitiesinclude:,.,Chromosomalabnormalities,.,Chromosomalabnormalities,110/414(27%)patientshadcytogeneticabnormalities,Multivariableanalysis,SurvivalandProgressiontoAML,Low-risk:normalor-YasasingleanomalyOSat5years:35%,Intermediate-risk:allotherabnormalitiesOSat5years:26%,high-risk:trisomy8orabnormalitiesofchromosome7orcomplexkaryotypeOSat5years:4%,SuchE,CerveraJ,CostaD,etal.Cytogeneticriskstratificationinchronicmyelomonocyticleukemia.Haematologica.2011;96(3):375-383.,.,MyelomonocyticClonalproliferation,Diseaseprogression,Somaticmutations,.,Spliceosomalmutations,Yoshida,etal.Frequentpathwaymutationsofsplicingmachineryinmyelodysplasia.Nature2011;478(7367):64-9.,Lessconspicuouslybutsignificantly,SRSF2mutationsweremorefrequentinCMMLcases,.,MeggendorferM,etal.SRSF2mutationsin275caseswithchronicmyelomonocyticleukemia(CMML).Blood.2012Oct11;120(15):3080-8.,.,3months,1109/L,.,CMML-1:blast(includingpromonocytes)5%inPBand10%inBMCMML-2:blastsfrom5%19%inPBand10%19%inBMorAuerrodsarepresentirrespectiveofblastcount,.,.,.,Diagnosticwork-up,.,.,Riskstratification,.,.,RiskstratificationMDAPS(M.D.AndersonPrognosticScore),.,Onepointforeachofthefollowingvariables,ALC:absolutelympcytecountIMC:immaturemyeloidcells,.,Riskmodel,.,NewMDSmodelappliedinCMMLwithleukocytosis(WBC12109/L),.,Score,.,Levelsofrisk,.,Therapeuticoptions,.,.,Cytotoxicchemotherapy,.,Hypomethylatingagen