Kudzokorora kweStem Cell Transplantation yeM Primary Myelofibrosis
Kusarudza zvakanakisisa kurapwa kwemasimboti myelofibrosis kunogona kunge kwakaoma. Kazhinji hapachina chisarudzo chakajeka. Kune mishonga yakasiyana-siyana yekutanga myelofibrosis, asi chete hematopoietic stem cell transplantation (iyo inonziwo sefupa rinokwidziridzwa) inorapa.
Sei Munhu Wese Asingaiti Kuwedzerwa?
Chirwere chinonzwika zvinoshamisa, saka unganetseka kuti nei munhu wose ane myelofibrosis wekutanga asingagamuchiri kuiswa.
Izvo zvose pamusoro pehuwandu hwehutano uye hunobatsira.
Kurapa mumusimboti myelofibrosis kunotungamirirwa nehuwandu hunotarisana nezviratidzo nezviratidzo zvauri kuona. Kana iwe usina zviratidzo kana kuti une zviratidzo zvine chirwere chisinganyanyi kuipa, zvinoreva kupona mushure mokunge uongororwa ndewe makore 15.4. Muchiitiko ichi, izvo zvinogona kuitika (acute kana chronic graft versus host disease uye rufu) inobatanidza nekugadzirisa kunze kwekubatsira kwehutano. Zvidzidzo zvakaratidza kuti kupona kwemakore mashanu kune vanhu vane ngozi shoma kana pakati-kati-1 chirwere chepakati chakanga chakadzika kudai vakanga vawana hematopoietic stem cell transplant. Zvisinei, pakati pepakati-2 nepamusoro-pwere chirwere, kupona kwemakore mashanu kwakasimbiswa kune vanhu vakagamuchira kuwedzerwa kufanana nevaya vasina.
Kana iwe unenge uri pakati-2 kana kuti uri pangozi myelofibrosis, hematopoietic stem cell transplant (HSCT) ndiyo inoshandiswa kurapwa. Nokuti kukwegura kunowedzera mukana wezvinetso zvinokonzerwa nekufambisa (zvakanyanya kune yechipiri kusvika kumatanho ekubatana nechirwere chirwere ), HSCT yakagara yakachengeterwa vanhu vane myelofibrosis inokosha pasi pemakore makumi matanhatu.
Kubva panguva yepakati pevanhu vane myelofibrosis inonyanya kuongororwa ndeye 67, izvi zvinorema kuwandisa nhamba yevanhu vane myelofibrosis yekutanga avo vanogona kutsvaga kurapwa uku. Uyezve, mupi anonyanya kupiwa weHSCT akafananidzwa nehama. Imwe hama yakakwana (amai vakafanana nababa semupiriridzi) ane rimwe mukana wechina wekufananidzwa, zvichiwedzera kuwandisa nhamba yevanhu vanogona kupinda murapi iyi.
Post-Transplant Prognosis
Parizvino, 40 kusvika ku60 muzana yevanhu vane myelofibrosis inonyanya kukosha iyo inopfurikidza inorarama makore matatu kusvika mana. Kune humwe humwe uchapupu hunoratidza kuti kuitika kweplenectomy (kuporesa kubviswa kwepleen) isati yashandurwa kunobatanidza nekuvandudzwa kwehuwandu hwemashoko kunyanya kune varume, asi chikonzero chekusazonyatsonzwisiswa. Uyezve, vanhu vanokura myelofibrosis mushure me polycythemia vera kana kuti hutachiona hunokosha hunoratidzika kuva nehuwandu hwekuchengetedza huri nani mushure mekutengesa kune vanhu vane myelofibrosis.
Ndiani Asingakwanisi?
Vamwe vanhu vane chirwere chakanyanya kukanganisa havakwanisi kuHSCT. Izvi zvinonyanya kugadziriswa nemukana wekurarama shure kweHSCT.
Zvinhu zvakanyanyisa zvakakura spleen (inopfuura masendimita masere pasi pejinga cage) uye zvinopfuura 20 kuwedzerwa ropa musati wapfurikidza (kunyanya kana iwe mune zvose) zvakasanganiswa nekusara kwemakore mashanu mushure mekugadzirwa.
Zvakadini Kana Usina Kukodzera?
Saka chii chinokurudzirwa kana uine mungofibrosis inonyanya kuisa pangozi asi iwe hauna kukodzera HSCT kana iwe usina mupiro? Kana iwe uine zviratidzo zvakadai sekuwedzera kunorwadza kwepleen, kuneta, kurwadziwa kwepfupa, usiku kutunga, nezvimwewo, ruxolitinib inogona kunge yakagadzirirwa kurapa.
Ruxolitinib yakaratidzwa kuderedza zviratidzo, kuderedza uswa hwepleen, uye kunatsiridza chirwere chemaperembudzi kune vanhu vane myelofibrosis. Imwe nzira inoshandiswa kuHSCT inogona kunge yakanyoresa kuchipatara chirongwa. Izvi zvingakupa kuwanika kwemishonga iri kuongororwa seyakwanisa kurapwa kwekutanga myelofibosis kare.
Hazvisi nyore kudzidza kuti hauna kukodzera kurapwa, asi rangarira kuongorora zvose zvaunogona.
Ramangwana rekushandura sekurapa
Nekunatsiridza mune marapirwo akakomberedzwa, sezvakaita regimen inoshandiswa kugadzira marora marara musati yashandurirwa uye mishonga inoshandiswa kudzivirira kubatanidzwa nehutachiona zvirwere, kuwedzera kunoitwa kune vanhu vanopfuura makore makumi matanhatu uye pamwe nevamwe vanopa mari (hama kwete zvakakodzera kufanana kana kufanana nevatsigiri vasingabatanidzi).
Dzimwe nzvimbo dzichapinza vanhu vane myelofibrosis kusvika kune makore 75.
Tariro, nekuvandudzika kunoramba kuripo, HSCT ichawanikwa kune vamwe vanhu vane myelofibrosis. Nemhaka yokuti myelofibrosis inokosha isiri mamiriro ezvinhu, zvinotora makore tisati tine kunzwisisa kwakakwana kwebasa revamwe vanopa mari muHSCT nekuda kwechirwere ichi.
Sources:
Ballen K. Kutarisa mubvunzo wekushandura mu myelofibrosis. Blood Cancer Journal. 2012; 2: e59.
Tefferi A. Mutungamiri wekutanga myelofibrosis. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Yakasvika musi waJune 29, 2016.)