I-Myelodysplastic syndrome, okanye i-MDS, iquka iintlobo ezahlukahlukeneyo zeziphazamiso ezichaphazela ukusebenza komnatha wethambo. Umnxeba wethambo wenza amangqamuzana egazi obomvu, iiseli ezimhlophe kunye neeplatelet zokucima, umsebenzi ongenalutho lomnxeba ungakhokelela ekudiseni i-anemia, ukubalwa kweeseli kunye nezinye iingxaki.
Ukuxhalabisa kakhulu nge-MDS yiyo) ezi zibalo eziphantsi kunye nazo zonke iingxaki ezinxulumene nazo; kwaye b) amandla okuba i-MDS iguqulwe ngumhlaza - i-leukemia ye-myeloid, okanye i-AML.
Iindidi ezahlukeneyo ze-MDS ziphathwa ngokuhlukileyo. Azikho zonke ii-MDS zokwelapha ezifanelekileyo kuzo zonke izigulane ezine-MDS. Izinketho zonyango lwe-MDS zibandakanya ukunakekelwa okuxhasayo, unyango oluphantsi, unyango olunzulu, kunye / okanye iimvavanyo zeklinikhi.
Ingqwalasela yonyango
Xa uxoxa ngesicwangciso sakho sonyango se-MDS kunye nogqirha wakho, izinto ezibizwa ngokuba zizigulane zinokubaluleka kakhulu. Imizekelo yeziganeko ezinxulumene nesigulane ziquka ezi zilandelayo:
- Indlela ohamba ngayo nemisebenzi yemihla ngemihla phambi kokuxilongwa kwe-MDS
- Ezinye iimeko zonyango onayo
- Iminyaka yakho
- Iindleko zezemali zonyango ezahlukeneyo
- Ziziphi izingozi zonyango ezamkelekileyo kuwe
Impawu zendlela ethile ye-MDS nazo zibaluleke kakhulu. Imizekelo yemimiselo ethile kunye neziphumo zibandakanya oku kulandelayo:
- Amakhonkco kunye neziphumo zokuvavanya ufuzo lomongo wakho, oluza kunceda ukukhetha iinketho ezikhoyo kuwe, amathuba okuthi i-MDS yakho iya kuqhubela phambili kwi- leukemia , kwaye zeziphi iziphumo ezinokulindelwa kwiirhile ezithile.
- Indlela i-MDS yakho ifuthe ngayo inani leeseli eziphilileyo kwigazi lakho elijikelezayo
- Ngaba isifo sakho sisisigxina ngokubhekiselele kwinani lezitshixo ezinokuthi "ziqhume" ezinomtsalane kumncedi wakho
Iinjongo zakho malunga noko ufuna ukuphuma kwonyango kwakhona zibandakanya kwisicwangciso. Imizekelo yeenjongo ezichaseneyo zonyango ziquka ezi zilandelayo:
- Ukuze uzive ungcono
- Nciphise imfuno yakho yokuba uninzi lwegazi
- Ukuphucula i- anemia , i- neutropenia , kunye ne- thrombocytopenia
- Ukufumana ukuxolelwa
- Philisa i-MDS
Jonga kwaye ulinde
Kwizigulane ezine-MDS engciphekweni ephantsi njengoko zichongiwe yiNkqubo ye-International Prognostic Scoring System, okanye i-IPSS, kunye nezibalo ezipheleleyo zegazi (CBC) , ngamanye amaxesha indlela efanelekileyo yokwenza unyango ibonwa kunye nenkxaso, njengoko kuyimfuneko.
Kule meko, kuyakufuneka uhlolwe ukuba utshintshe kwimboleko yakho engabonisa ukuqhubela phambili kwesi sifo. I-CBC rhoqo, kunye ne- bone marrow aspirate kunye ne-biopsy , inokuba yinxalenye yokubeka iliso.
Unonophelo loNcedo
Ukunyamekelwa okuxhasayo kubhekisela kwiipilisi ezisetyenziswa ekuphatheni nasekulawuleni i-MDS; ezi zonyango zinokuphucula kakhulu imeko yomntu, kodwa ziyeka ukufutshane nokuhlaselwa iiseli ezidala i-MDS.
Ukutshintshwa
Ukuba igazi lakho liqala ukuwa kwaye ufumana izibonakaliso, unokufumana inzuzo yokumpontshelwa kwamaseli obomvu okanye iiplatelet. Isigqibo sokufakela igazi siya kuxhomekeka kwezinye iimeko zonyango onayo kunye nendlela ozizwa ngayo.
I-Iron Overload kunye neCheration Therapy
Ukuba uqala ukufuna ukumanyelwa igazi rhoqo ngenyanga, usenokuba ngumngcipheko wokuphuhlisa imeko ebizwa ngokuba yintsimbi ephezulu.
Amazinga aphezulu entsimbi kwi-red cell cell transfusion angabangela ukwanda kwintsimbi egcina umzimba wakho. Amanqanaba aphezulu anjalo angonakalisa izitho zakho.
Oogqirha banokuyiphatha nokuthintela isinyithi kwi-multiple transfusion ngokusebenzisa imithi ebizwa ngokuba yi-iron chelators, equka unyango lomlomo, i-deferasirox (Exjade), okanye i-infusion i-deferoxamine mesylate (i-Desferal). Isikhokelo soMsebenzi weNational Cancer Network, okanye iNCCN, unikezela umlinganiselo ugqirha wakho unokusebenzisa ukuba anqume ukuba ufuna i-chelation yonyango.
Izinto ezibalulekileyo zokukhula Abantu abathile abane-anemia ye-MDS banokuzuza ngokufumana imveliso yokukhula imithi ebizwa ngokuba yi-erythropoietin yokuvuselela ama-protein (ii-ESA).
Imizekelo yee-ESA iquka i-epoetin alfa (i-Eprex, iNgxelo okanye i-Epogen) okanye isenza i-darbepoetin alfa (Aranesp) ixesha elide. La mayeza anikezwa njengentsimbi kumathambo akho enamafutha (injection subcutaneous). Nangona la mayeza angenakunceda kuzo zonke izigulane ze-MDS, zinokukunceda ukuthintela ukumpontshelwa igazi kwezinye.
Ugqirha wakho unokucela ukuba akuqale kwi- colony stimulating factor , efana ne-G-CSF (Neupogen), okanye i- GM-CSF (leukine) , ukuba inani lakho le-cell white libe liphantsi ngenxa ye-MDS yakho. Izinto ezivuselela iColony zinceda ukukhulisa umzimba wakho ukuvelisa izifo ezininzi ukulwa namaseli omhlophe amhlophe abizwa nge-neutrophils. Ukuba inani lakho le-neutrophil liphantsi, usemngciphekweni ophezulu wokuhlawula isifo esinobungozi. Gcina iliso malunga nayiphi na impawu zentsholongwane okanye umkhuhlane, kwaye ubone umboneleli wezempilo ngokukhawuleza ukuba unokukhathazeka.
Unyango ophantsi
Unyango olusisiseko olunzulu lubhekisela ekusebenziseni i-chemotherapy okanye i-agents eyaziwa njenge-biologicalic modifiers. Ezi zonyango zibonelelwa ngokusisigxina, kodwa ezinye zazo zingadinga ukunakekelwa ngenkxaso okanye ukuhlala esibhedlele ngokukhawuleza, ngokwesibonelo, ukunyanga ukusuleleka kwintsholongwane.
Unyango lwe Epigenetic
Iqela lemithi ebizwa ngokuba yi-hypomethylating okanye i-demethylating agents yizona zixhobo ezitsha zokulwa ne-MDS.
I-Azacitidine (iVidaza) ivunyiwe yi-FDA yokusetyenziswa kuzo zonke izigaba zeFrench-American-British (FAB) kunye nazo zonke iintlobo zeengozi ze-IPS ze-MDS. La mayeza anikezelwa njengentsimbi engaphantsi kweentsuku ezi-7 ngokulandelanayo, nganye iintsuku ezingama-28 ubuncinane ubuncinane bama-4-6. Izifundo ze-azacitidine ziye zabonisa amaxabiso okuphendula ngamaphesenti angama-60, malunga neepesenti ezingama-23 ekufezekiseni ukuhluthwa ngokukodwa okanye okupheleleyo kwezifo zabo. I-Azacitidine idla ngokubangela ukulahla kokuqala kwi-cell cell counts ezingenakubuyela emva emva kokuqala okanye ezimbini.
Olunye uhlobo lwe-agent ye-hypomethylating esebenzisa unyango lwe-MDS yi-decitabine (iDacogen). Kuyafana kakhulu kwisakhiwo ku-azacitidine, kwakhona i-FDA ivunyelwe kuzo zonke iintlobo ze-MDS. I-regimen yonyango yayinxulumene neentlobo ezinobungozi obuncinane, ngoko ke ikwacatshungulwa nokuba yinyango ephantsi. I-Decitabine inokunikezwa kwangaphakathi okanye ngaphantsi. Olunye uphando apho i-decitabine yanikezelwa kwangaphakathi kwiintsuku ezingama-5 ibonise izinga elipheleleyo lokuxolelwa malunga nama-40 ekhulwini. Ezinye iirjimenti zeerandi ziphandwa.
Unyango lwe-Immunosuppressive ne-Biologic Response Modifiers
Kwi-MDS, iiseli ezibomvu zegazi, iiseli zegazi ezimhlophe kunye neeplatelets zibulawa okanye zife ngaphambi kokuba zikhule ngokwaneleyo ukuba zikhutshwe emnzini wethambo zibe ngegazi. Kwezinye iimeko, i-lymphocytes (uhlobo lwe-cell white white) lijongene nale nto. Kuzo zi gulane, kunokusebenza kakuhle ukusebenzisa unyango oluchaphazela i-immune system.
I-non-chemotherapy, i-agent-intensity-agents (i-biologic response modifiers) iquka i-thymocyte globulin (ATG), i-cyclosporine, i-thalidomide, i-lenalidomide, i-anti-tumor necrosis yeprotheni ye-fusion receptor, kunye ne-vitamin D. Zonke ezi zibonise ubuncinci kwezinye iilingo zokuqala, kodwa abaninzi banesidingo sezilingo ezininzi zeklinikhi ukuqonda ukusebenza kweentlobo ezahlukeneyo ze-MDS.
Abantu abanoluhlobo oluthile lwe-MDS olubizwa ngokuba yi-5q- syndrome, apho kukho i-defect yomzimba kwi-chromosome yesi-5, unokufumana impendulo kwisiza esibizwa nge-lenalidomide (Revlimid). Ngokuqhelekileyo, i-lenalidomide isetyenziswe kwizigulane eziphantsi okanye eziphantsi kwe-IPSS ingozi ye-MDS exhomekeke ekuxhomekeni kwegazi. Kwisifundo se-lenalidomide, ezininzi izigulane ziye zanciphisa iimfuno zokumpontshelwa igazi-malunga nama-70 ekhulwini, eqinisweni-kodwa zaqhubeka zifumana amanqaku aphantsi kunye ne-neutrophil. Iintlobo zokuphatha i-MDS ephezulu, okanye i-subtypes ngaphandle kwe-5q- syndrome kunye ne-lenalidomide isacatshungulwa.
Unyango ophezulu
Chemotherapy
Ezinye izigulane ezinomngcipheko ophezulu we-MDS, okanye iifayile ze-FAB ze-RAEB kunye ne-RAEB-T, zinokuphathwa nge-chemotherapy ephezulu. Olu hlobo lwe-chemotherapy, uhlobo olufanayo olusetyenziselwa kunyango lwe-leukemia ye-myelogenous acute (AML), lujolise ekutshatyalaliseni inani labantu abangaqhelekanga kwi-bone yomnatha okhokelela kwi-MDS.
Nangona i-chemotherapy ingaba luncedo kwezinye izigulane ze-MDS, kubalulekile ukuqwalasela ukuba izigulane ezithe zinezinye iimeko zonyango zijongene nemingcipheko eyongezelelweyo. Iintlobo ezinokuthi zonyango kufuneka zigqithise ingozi ebandakanyekayo.
Uphando luqhubekeka ukuthelekisa iziphumo ze-chemotherapy ezinzulu ngaphezu kwe-azacitidine okanye i-decitabine.
Utshintsho lweStem Cell
Izigulane ezinobungozi obuphezulu be-IPSS MDS zinokukwazi ukuphumeza unyango lwesifo sabo nge- allogeneic stem cell transplantation . Ngelishwa, ubunobungozi obuphezulu bale nkqubo bawunciphisa ukusebenzisa kwawo. Enyanisweni, i-allogeneic stem cell transplant ingaba nomlinganiselo wokufa ochaphazeleka unyango ofikelela kwi-30%. Ngako oko, olu unyango lulusetyenziswa kuphela kwizigulane ezincinci eziphilileyo.
Izifundo ezikhoyo ziphanda indima ye- non-syeloablative okuthiwa "mini" yokufakelwa kwezigulane ezidala kunye ne-MDS. Nangona ezi ntlobo zokutshintshwa ziye zacingelwa ukuba zingasebenzi ngaphantsi kunezinto eziqhelekileyo, ukunciphisa ukutyhafisa kwabo kunokubangela ukhetho kwizigulana ezingenakufaneleka.
Isishwankathelo:
Ngenxa yeentlobo ezahlukeneyo ze-MDS kunye neentlobo ezahlukeneyo zesigulane, akukho bukhulu-bukhulu-bukho konke-unyango. Ngoko ke kubalulekile ukuba izigulane ze-MDS zixubushe zonke iinketho kunye neqela labo loononophelo lwezempilo, kwaye zifumane unyango oluya kubabonelela ngezona zibonelelo ezintle kunye nenani elincinci lobunobuthi.
Uvavanyo lwezonyango oluneendlela ezintsha zokwelapha ze-MDS ziyaqhubeka, ngoko qhubeka ulungelelanise. Ngokomzekelo, i- ruxolitinib (i-Jakafi) iphandwa malunga nokunyangwa kwezigulane ezine-MDS eziphantsi okanye eziphakathi kwe-1.
Imithombo:
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Kantarjian H, O'Brien S, Giles F, et al. I-Decitabine ishedyuli ye-dose ephantsi (100 mg / m2 / i-Course) kwi-myelodysplastic syndrome (MDS). Uthelekiso lweeshedyuli ezi-3 ezahlukeneyo. Igazi. 2005; 106. I-Asbtract 2522.
Malcovati L, Hellström-Lindberg E, Bowen D, et al. Ukuxilongwa kunye nokunyangwa kwe-primary myelodysplastic syndromes kubantu abadala: iingcebiso ezivela kwi-European LeukemiaNet. Igazi . 2013; 122 (17): 2943-2964.
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