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1、急性髓系白血病WHO分型及治療魏輝中國(guó)醫(yī)學(xué)科學(xué)院血液病醫(yī)院(血液學(xué)研究所)WHO分型FAB與WHO分型newlydiagnosedpatientswithnewlydiagnosedpatientswith“AML,NOS.”BLOOD,2013121:2424-2431FAB與WHO分型NPM1–andNPM1andNPM1–/CEBPA–patientswithnewlydiagnosed“AML,NOS.”BLOOD,2013121:2424-2431duetothelackofprognosticsignificanceofmultilineagedy
2、splasiainpatientswithoutMDS-associatedcytogeneticfindingsandwithamutationofNPM1orbiallelicmutationofCEBPA87-89,thesemutationsnowsupersedethepresenceofmultilineagedysplasiaintheclassification.AMLwithmutatedCEBPAorNPM1?CEBPA要求雙突變,需要排除單突變?NPM1和CEBPA突變的AML診斷分型要優(yōu)先于伴有多系增生異常AML的分型。NPM1andM
3、ultilineagedysplasiaBlood.2010Dec23;116(26):6147-8CEBPAmutationAMLCEBPAmutationAMLJClinOncol28:2739-2747CEBPAmutation單突變單突變雙突變雙突變denovoAMLwithBCR-ABL1?一個(gè)新的建議分類(lèi)?可能從TKI治療中Ph+Ph+AMLAMLCML-MBC獲益AmJClinPathol2007;127:642-650Ph(+)AMLvsCML/BCL?Ph(+)AML常伴有免疫球蛋白及T細(xì)胞受體基因的隱性缺失。BrJHaematol.2013
4、May;161(4):541-50AnnHematol(2016)95:1211–1221AML‐MRC?由于NPM1突變及CEBPA雙突變常伴發(fā)del(9q),并且這種情況下的del(9q)沒(méi)有預(yù)后意義,因此,dl(9)del(9q)從定義MDS相關(guān)的細(xì)胞遺傳學(xué)異常中去除CEBPAandCEBPAandkaryotypeabnormalitiesRFSOSBlood.2013;122(9):1576-1582NPM1andkaryotypeabnormalitiesEFSOSBlood.2009;114:3024-3032AMLwithmutatedRUNX
5、1?一個(gè)新的建議分類(lèi)?主要見(jiàn)于細(xì)胞遺傳學(xué)中危組,尤其是正常核型,非復(fù)雜核型的+8。高危組中發(fā)生率低。?RUNX1突變與MDS相關(guān)的細(xì)胞遺傳學(xué)改變沒(méi)有相關(guān)性JClinOncol2011,29:1364-1372RUNX1andkaryotypeKtKaryotypeTtlTotalRUNX1-muttdtatedRUNX1-wildFavorable590(0.0)59(100.0)Intermediate32748(147)48(14.7)279(853)279(85.3)Unfavorable668(12.1)58(87.9)Normal23032(13.9
6、)198(86.1)Simple16919(11.2)150(88.8)Complex535(9.4)48(90.6)-7/7q-103(30.0)7(70.0)+8226(27.3)16(72.7)+21112(182)2(18.2)9(818)9(81.8)-5/5q-10(0.0)1(100.0)+1130(0.0)3(100.0)+1310(0.0)1(100.0)Blood.2009;114:5352-5361RUNX1mutationRUNX1mutation?發(fā)生率:5.6%,(6.3%inCNAML)?CR:RUNX1突變型和野生型的CR率分別
7、為60.4%and73.4%60.4%and73.4%(P=0.055)JClinOncol2011,29:1364-1372RUNX1mutationRUNX1mutationRFSOSJClinOncol2011,29:1364-1372AML,nototherwisespecified?急性紅白血?。ˋML,erythroid/myeloidtype從分類(lèi)中刪除?純紅血?。≒ureerythroidleukemia)仍然保留在AML,NOS亞型中。BMerythroidMyeloblast%inPriorRecurringMeetscriteriaFou
8、rtheditionUpdatedfo