Myelodysplastic syndrome, kapena MDS, imaphatikizapo zovuta zosiyanasiyana zomwe zimakhudza mafupa ogwira ntchito. Mafupa a mafupa amapanga maselo ofiira a maselo ofiira, maselo oyera ndi mapuloletti ophimbitsa thupi, ntchito yamtambo wosauka imatha kuwonetsa kuchepa kwa magazi, kuchepa kwa maselo, ndi mavuto ena.
Zomwe zimadetsa nkhaŵa ndi MDS ndi) ziwerengero zochepa ndi mavuto onse; ndi b) kuthekera kwa MDS kukhala khansara - mitsempha yambiri ya myeloid, kapena AML.
Mitundu yosiyanasiyana ya MDS imathandizidwa mosiyana. Sikuti mankhwala onse a MDS ndi ofunika kwa wodwala aliyense ndi MDS. Zosankha za mankhwala a MDS zimaphatikizapo chisamaliro chothandizira, chithandizo chochepa kwambiri, mankhwala apamwamba, ndi / kapena mayesero a chipatala.
Zotsatira za Chithandizo
Pokambirana za dongosolo lanu la mankhwala a MDS ndi dokotala, zomwe zimatchedwa odwala-zifukwa zingakhale zofunikira kwambiri. Zitsanzo za zinthu zokhudzana ndi odwala zikuphatikizapo zotsatirazi:
- Momwe mumagwirizira ntchito tsiku ndi tsiku musanayambe kudziwa za MDS
- Matenda ena omwe muli nawo
- Zaka zanu
- Ndalama za ndalama za mankhwala osiyanasiyana
- Ndi zoopsa ziti za mankhwala zomwe zimakuvomerezeka
Zizindikiro za mtundu wanu wa MDS ndizofunikira kwambiri. Zitsanzo za makhalidwe enieni ndi zotsatirazi ndizo zotsatirazi:
- Zolemba ndi zotsatira za kuyesa kwa majeremusi ya mafupa anu, zomwe zimakuthandizani kudziwa zomwe mungapeze, mwinamwake MDS yanu idzapita ku khansa ya m'magazi , ndipo zotsatira zake zikhoza kuyembekezedwa kuchokera kuchipatala china.
- Momwe MDS yanu ikukhudzizira kuchuluka kwa maselo abwino m'thupi lanu
- Matendawa ndi ovuta kwambiri poyerekezera ndi chiwerengero cha maselo omwe amatha kuphulika mumtambo wanu
Zolinga zanu pa zomwe mukufuna kuti mutuluke kuchipatala zimalowanso mu ndondomekoyi. Zitsanzo za zolinga zamankhwala zosiyana zikuphatikizapo izi:
- Kungomverera bwinoko
- Pewani kufunika kokhala ndi magazi ochulukirapo
- Limbikitsani kuchepa kwa magazi , neutropenia , ndi thrombocytopenia
- Pezani chikhululuko
- Chithandizo MDS yanu
Yang'anani ndi Dikirani
Kwa odwala omwe ali ndi chiopsezo chachikulu chotchedwa MDS monga momwe awonetsera ndi International Prognostic Scoring System, kapena IPSS, ndi ndalama zokhazikika zamagazi (CBC) , nthawizina njira yabwino kwambiri yothandizira ndi kuyang'ana ndi kuthandizira, ngati pakufunikira.
Pankhani iyi, muyenera kuyang'aniridwa kuti musinthe maukonde anu omwe angasonyeze kupititsa patsogolo kwa matendawa. CBCs nthawi zonse, komanso aspirate ya mafupa a mafupa ndi biopsy , ikhoza kukhala mbali yowunika.
Care Support
Kusamalira chithandizo kumatanthawuza mankhwala ochiza ndi kusamalira MDS; Mankhwalawa amatha kusintha kwambiri mkhalidwe wa munthu, koma amalephera kumenyana ndi maselo omwe amachititsa MDS.
Kuthamangitsidwa
Ngati magazi anu ayamba kugwa ndipo mumakhala ndi zizindikiro, mukhoza kupindula ndi kuikidwa magazi a maselo ofiira kapena mapiritsi. Kusankha kuikidwa magazi kumadalira matenda ena omwe muli nawo komanso momwe mumamvera.
Iron Overload ndi Therapy Chelation
Ngati mumayamba kuika magazi ambiri mwezi uliwonse, mukhoza kukhala pachiopsezo chokhala ndi vuto lotchedwa chitsulo chowopsa.
Mapulogalamu apamwamba a chitsulo mu magazi ofiira maselo ena amachititsa kuwonjezeka kwazitsulo zamagetsi m'thupi lanu. Mankhwalawa amatha kuwononga ziwalo zanu.
Madokotala amatha kuteteza ndi kuteteza chitsulo kuwonjezera pa kuikidwa magazi pogwiritsira ntchito mankhwala omwe amatchedwa iron chelators, omwe amatenga mankhwala opatsirana pakamwa, deferasirox (Exjade), kapena kulowetsedwa kotchedwa deferoxamine mesylate (Desferal). Gwiritsani ntchito malangizo a National Comprehensive Cancer Network, kapena NCCN, perekani zomwe dokotala angagwiritse ntchito posankha ngati mukufuna chithandizo cha iron chelation.
Zinthu Zakukula Anthu ena omwe ali ndi magazi a MDS angapindule ndi kulandira mankhwala omwe amatchedwa erythropoietin okakamiza othandizira kapena mapuloteni (ESAs).
Zitsanzo za ESA zimaphatikizapo epoetin alfa (Eprex, Written or Epogen) kapena nthawi yayitali darbepoetin alfa (Aranesp). Mankhwalawa amaperekedwa ngati jekeseni mu minofu yanu yambiri (jekeseni yapansi). Ngakhale mankhwalawa sathandiza kwa odwala onse a MDS, akhoza kuthandizira kuika magazi ena.
Dokotala wanu angakupatseni inu kuti muyambe kuyambitsa chinthu choyambitsa matenda , monga G-CSF (Neupogen), kapena GM-CSF (leukine) , ngati kuchuluka kwa maselo oyera a magazi kumakhala otsika chifukwa cha MDS yanu. Zinthu zowonongeka za Coloni zimathandiza kulimbikitsa thupi lanu kuti likhale ndi matenda ambiri omwe amamenyana ndi maselo oyera a m'magazi otchedwa neutrophils. Ngati chiwerengero chanu cha neutrophil chiri chochepa, muli pachiopsezo chachikulu chotenga kachilombo koopsa. Yang'anirani zizindikiro za matenda kapena malungo, ndipo muwone wothandizira zaumoyo mwamsanga ngati muli ndi nkhawa.
Thandizo Labwino
Mankhwala otsika kwambiri amatanthauza kugwiritsa ntchito mankhwala osokoneza bongo omwe amadziwika kuti biologic reaction modifiers. Mankhwalawa amaperekedwa makamaka kuchipatala, koma ena mwa iwo amafunikira chithandizo chothandizira kapena kuchipatala pambuyo pake, mwachitsanzo, kuti athetse matenda opatsirana.
Epigenetic Therapy
Gulu la mankhwala lotchedwa hypomethylating kapena demethylating mawotchi ndizo zida zatsopano zotsutsana ndi MDS.
Azacitidine (Vidaza) yavomerezedwa ndi FDA kuti igwiritsidwe ntchito pazigawo zonse za French-American-British (FAB) ndi magulu onse a PPS a MDS. Mankhwalawa amaperekedwa monga jekeseni wothandizira kwa masiku asanu ndi awiri motsatira, masiku 28 aliwonse osachepera 4-6. Kafukufuku wa azacitidine wasonyeza kuchuluka kwa maperesenti 60 peresenti, ndipo pafupifupi 23 peresenti amapindula pang'onopang'ono kapena kukhululukidwa kwathunthu kwa matenda awo. Azacitidine kaŵirikaŵiri amachititsa kuti magazi akuyambe kugwiritsidwa ntchito m'magazi omwe sangakhalepo mpaka atatha yoyamba kapena ziwiri.
Mtundu wina wa hypomethylating wothandizira omwe amagwiritsidwa ntchito pa mankhwala a MDS ndi decitabine (Dacogen). Chofanana kwambiri pakukonzekera kwa azacitidine, komanso FDA imavomerezedwa ku mitundu yonse ya MDS. Nthawi zambiri mankhwalawa ankagwiritsidwa ntchito ndi poizoni wochepa kwambiri, choncho amaonanso kuti ndi otsika kwambiri. Decitabine ingaperekedwe mwakuya kapena subcutaneously. Kafukufuku wina yemwe decitabine anapatsidwa intravenously kwa masiku asanu anasonyeza kuchuluka kwa chikhululukiro cha pafupifupi 40 peresenti. Malamulo ena amodzi akutsatiridwa akufufuzidwa.
Mankhwala Opatsirana ndi Immunosuppressive and Biological Response Modifiers
Mu MDS, maselo ofiira a m'magazi, maselo oyera a magazi ndi mapiritsi amaphedwa kapena kufa asanafe okhwima kuti athe kumasulidwa ku fupa lamagazi kupita m'magazi. Nthaŵi zina, ma lymphocytes (mtundu wa selo loyera la magazi) ali ndi udindo pa izi. Kwa odwalawo, zingakhale zogwiritsidwa ntchito kugwiritsa ntchito mankhwala omwe amachititsa chitetezo cha mthupi.
Non-chemotherapy, otsika kwambiri (biologic reaction modifiers) akuphatikizapo anti-thymocyte globulin (ATG), cyclosporine, thalidomide, lenalidomide, anti-tumor necrosis factor receptor fusion mapuloteni, ndi mavitamini D ofanana. Zonsezi zakhala zikuwonetsa zina m'mayesero oyambirira, koma ambiri akusowa mayesero ambiri a kuchipatala kuti amvetsetse bwino ntchito zosiyanasiyana za MDS.
Anthu omwe ali ndi mtundu wina wa MDS wotchedwa 5q-syndrome, umene umakhala ndi vuto la chibadwa mu chromosome 5, akhoza kukhala ndi yankho kwa mankhwala otchedwa lenalidomide (Revlimid). Kawirikawiri, lenalidomide imagwiritsidwa ntchito kwa odwala omwe ali otsika kapena ochepa omwe ali ndi kachilombo ka HIV kamene kamakhala ndi kachilombo kofiira magazi. Pa kafukufuku wa lenalidomide, odwala ambiri anali atapatsidwa magazi ochepa - pafupifupi 70 peresenti, makamaka - koma anapitiriza kukhala ndi mapepala otsika ndi neutrophil. Phindu lokhazikitsa matenda a MDS, kapena subtypes osati a 5q-syndrome ndi lenalidomide akuwerengabebe.
Thandizo Labwino
Chemotherapy
Odwala ena omwe ali ndi chiopsezo chachikulu cha MDS, kapena FAB mitundu RAEB ndi RAEB-T, akhoza kuchiritsidwa ndi mankhwala a chemotherapy. Mankhwalawa, omwe amagwiritsidwa ntchito pochiza mayelo a myelogenous leukemia (AML), amayesetsa kuwononga chiwerengero cha maselo osadziwika omwe amapita ku MDS.
Ngakhale chemotherapy ingakhale yopindulitsa kwa odwala ena a MDS, nkofunika kulingalira kuti okalamba omwe ali ndi matenda ena amakumana ndi zoopsa zina. Zopindulitsa za mankhwalawa ziyenera kupitirira mowopsa.
Kafukufuku akupitirirabe poyerekeza zotsatira za mankhwala amphamvu kwambiri pa mankhwala a azacitidine kapena decitabine.
Kusuntha kwa Cell Shem
Odwala omwe ali ndi chiopsezo chachikulu chotchedwa IPSS MDS amatha kuchiza matenda awo pogwiritsa ntchito alloceneic stem cell transplantation . Mwatsoka, chikhalidwe choopsa kwambiri cha njirayi chimachepetsa kugwiritsa ntchito kwake. Ndipotu, kupititsa maselo a allogeniki kumatha kukhala ndi chiwerengero cha imfa chokhudza 30%. Choncho, mankhwalawa amagwiritsidwa ntchito makamaka kwa odwala omwe ali ndi thanzi labwino.
Kafukufuku wamakono akufufuzira udindo wa mankhwala osagwiritsa ntchito myeloablative omwe amatchedwa "mini" omwe akudwala ndi MDS. Ngakhale kuti izi zimawoneka ngati zosagwira ntchito kusiyana ndi zomwe zimawoneka, kuchepa kwao kungawapatse mwayi kwa odwala omwe sangavomereze.
Chidule:
Chifukwa cha mitundu yosiyanasiyana ya MDS ndi mitundu yosiyana ya odwala, palibe mankhwala amodzimodzi-kukula. Choncho ndi kofunika kuti odwala MDS akambirane zomwe angasankhe ndi gulu lawo lachipatala, ndipo apeze mankhwala omwe angapindule nawo kwambiri ndi poizoni.
Mavuto a chipatala ndi mankhwala atsopano a MDS akuyenda, choncho khalani maso. Mwachitsanzo, ruxolitinib (Jakafi) ikufufuzidwa kuti athe kuchiritsidwa ndi odwala omwe ali ndi vuto lochepa kapena lapakatikati-1 la MDS.
Zotsatira:
Greenberg PL, Attar E, Bennett JM, et al. Myelodylastic syntndrome JNCCN. 2013; 11 (7): 838-874.
Kantarjian H, O'Brien S, Giles F, et al. Mlingo wa mlingo wochepa wa Decitabine (100 mg / m2 / Kosi) mu myelodysplastic syndrome (MDS). Kuyerekeza ndi ndondomeko zitatu za mlingo. Magazi. 2005; Kutuluka 2522.
Malcovati L, Hellström-Lindberg E, Bowen D, et al. Kuzindikira ndi chithandizo cha mankhwala oyambirira a myelodystlastic syndromes: akuluakulu ochokera ku European LeukemiaNet. Magazi . 2013; 122 (17): 2943-2964.
Nimer, S. "Myelodysplastic Syndromes" Magazi May 2008. 111: 4841- 4851.
Scott, B., Deeg, J. "Myelodysplastic Syndromes" Kukambitsirana Kwachidule kwa Mankhwala 2010. 61: 345-358.