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wms65352003

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[交流] 骨髓增生异常综合征的治疗选择

???????????????(MDS)??????????????????????????????????????????????????????????????/???????????(dysplasia)????Ч???(ineffective hamatopoiesis)???????????????ǹ????????????з???????????????????????????????????????????????????(AML)??Σ??????[1]??????????FDA???????MDS?????????3????2004??5??19???????5-????????azacytidine??5AC????2005??12??27????????????????Lenalidomide??CC-5031,Revlimid)??2006??5??2???????????????decitabine??DAC????
Salvi??[2]??1999??1????2005??6??680??MDS??????????????鷢?????????????????37%??????13%??????????50%??Malcovati??[3]??1992????2002??400??MDS?????????????????70??????????????65??70???????RA/RARS????????????????????????????RA/RARS????????????????????????????????????70??????????????????????????????????????????????????????MDS?????????????????????????????????廯???????????????????????????????????????????????IPSSΣ????????????????????????????????????????????????????????????????ΣMDS??????????????÷???????????????Ρ?
?????MDS?????????????2004??2005????MDS??????????飬????????102??????????????????????????????????????????????94%??????28%??Ψ???????????EPO??G-/GM-CSF??????ù??A??CSA?????????????????B12??B6????????????????????IPSS??Σ/??Σ-1?????в?????????????????????????????????41%/50%???????/??λ??,???????34%/30%??25%/25%??????Σ-2/??Σ?????????30%/30%??63%/70%??7%/10%[4]???????????????????MDS???????????????????????????????????????????????????????????ж?????????????????????Σ?????MDS???????????????????????????????????????????????????????????Щ????????????Ω???????Ρ????????????????????????????????????????????????IPSS??Σ/??Σ-1 MDS?????????????????????????RBC?? 25??λ???5g???????????????? 1500 mg/L????????????????????EPO??G??GM??-CSF???????о??????RBC?????<2U?????EPO??<500U/L???????Ч????74%???????RBC???????2U?????EPO??>500U/L???????Ч???23%???????RBC???????2U?????EPO??>500U/L???????Ч????7%??
??????allo-HSCT??Ψ?????????MDS????Σ???????MDS??????????????????????????????????????[5]??Cutler??[6]???о????????IPSS??Σ/??Σ-1?????????????????????HSCT?????????????棬??IPSS??Σ-2/??Σ??????????????HSCT?????????????棬???????????????<55?????Σ????Σ???????????HLA??????????壬??????????????allo-SCT??????<40?????Σ????Σ????Σ??????????HLA??????????allo-SCT????Σ/??Σ-1?????????HSCT??????????????????????????????????????????????????IPSSΣ?????С?
2005??List??[7]??α????????????Lenalidomide??Revlimid, CC-5013)????MDS??????5q-???????????MDS??????Ч??????????????????????й???148?????????(RBC??2U/2??)??5q31.1-??MDS????(111??????5q31.1-,????37???????ж?????????)?μ?, ?????????????10mg/d(104??)????10mg/d??21d,?28d??????(44??)??????24??????IWG??Ч?????????Ч??????64%????????????,Hb??λ??????39g/L,??λ??Ч????4?????Σ????Σ-1?????????????????????66%,?????????52%,????5q31.1-?????????????????????????ж??????????????,????69%??49%??????????Ч?????????76%?????????????????????Ч(??????????????????50%),????55%?????????????CR???????????????????5q_????????????????????????????Σ????Σ-1 MDS????????????????????????10mg/d??????????????????
5-????????5-agacitidine, 5AC????????????decitadine, DAC?? ?DNA??????????????????DNA???????????????????????????silence??????????±???5AC????MDS CR???10%?????????PR?????1%???????????HI??36%?? ????Ч???48%[8]??DAC II?????????????Ч???49????CR???24??[9]?????????????????DAC??????????????Ч??DAC?????飨89???????????HI?????17????????CR???9??????13???????????????????飨81??????????0????7????[10]??????????о?[11]?У?????DAC??????п????????????????????31%????????????Ч????Σ??/??Σ???????????п???????????????????<75???????HSCT???????????5AC(75mg/m-2.d-1, ??????????7??, ?4????????????4?????)??DAC??15mg/m-2, ???????????3h??????8h??Σ?????3??, ?6????????????4???????????
????????????[12??13]????????????????绷??ù??A??3mg/Kg/d)??ATG(40 mg/kg.d??4d)????С???????????С????????????2mg/d,?????????????ù???3mg~14 mg/ m-2.d-1??????2С??????????7d??10d??????????????????VP-16??50mg/d??????2С???????????????2d~7d??????4??????????濵??2mg/m-2•d-1 ??5d??????????????????????100mg/d??????????400mg/d????????????????????MDS????Щ?????д???????????Ρ?
???????о???MDS????????????????涨???????????????MDS???????????IPSS??????????????????????MDS????????н?????Ч???????Ч?????????2001??????????MDS???????????Ч???[14]???????????????????????????????????DAC???????????MDS??????CR??????
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