Myelodysplastic syndrome, kapa MDS, e kenyeletsa mathata a sa tšoaneng a fapaneng a amang mesifa ea masapo. Motsoako oa masapo o etsa lisele tse khubelu tsa mali, lisele tse tšoeu le li-platelets tsa ho koala, mosebetsi o mofutsana o joalo o ka lebisa ho phokolo ea mali, tekanyo e tlase ea lisele le mathata a mang.
Ho ameha haholo ka MDS ke) lintlha tsena tse tlaase le mathata ohle a amanang le ona; le b) monyetla oa hore MDS e fetohe kankere - kankere e matla ea myeloid, kapa AML.
Mefuta e sa tšoaneng ea MDS e tšoaroa haholo. Hase mefuta eohle ea phekolo ea MDS e loketseng mokuli e mong le e mong ea nang le MDS. Khetho bakeng sa phekolo ea MDS e kenyelletsa tlhokomelo e tšehetsang, phekolo e matla haholo, phekolo e matla haholo, le / kapa liteko tsa kliniki.
Litlhahlobo tsa Kalafo
Ha u bua ka morero oa hau oa phekolo ea MDS le ngaka ea hau, lintho tseo ho thoeng ke tsa bakuli li ka ba tsa bohlokoa haholo. Mehlala ea lintho tse amanang le mamello li kenyelletsa tse latelang:
- U ne u sebetsana joang le mesebetsi ea letsatsi le letsatsi pele MDS e hlahlojoa
- Meriana e meng ea bongaka eo u nang le eona
- Lilemo tsa hau
- Litšenyehelo tsa lichelete tsa phekolo e sa tšoaneng
- Ke likotsi life tsa phekolo tse amohelehang ho uena
Litšobotsi tsa mofuta oa hau oa MDS le tsona ke tsa bohlokoa haholo. Mehlala ea litšoaneleho tse khethehileng le liphuputso li kenyeletsa tse latelang:
- Lipontšo le liphello tsa ho hlahlojoa ha liphatsa tsa mafu tsa lefutso la hao, tse thusang ho fumana hore na u ka khetha hokae, monyetla oa hore MDS ea hau e tsoele pele ho ea ho kankere ea mali , 'me ke liphello life tseo u ka li lebellang ho tsoa mekhoeng e itseng ea phekolo.
- Kamoo MDS ea hau e amang palo ea lisele tse phetseng hantle kateng mali a hao a potolohileng
- Sefuba sa hao se matla hakae ho latela palo ea lisele tsa "ho phatloha" tse seng kae tsa 'mele oa hau
Lipakane tsa hau bakeng sa seo u batlang ho tsoa kalafo li boetse li kenyelletsa moralo. Mehlala ea lipakane tse sa tšoaneng tsa phekolo e kenyeletsa tse latelang:
- Feela ho ikutloa u le betere
- Fokotsa tlhokahalo ea hau ea ho ba le tšelo ea mali e ngata haholo
- Ntlafatsa khaello ea mali , neutropenia , le thrombocytopenia
- Finyella tšoarelo
- Phekola MDS ea hau
Sheba 'me U eme
Bakeng sa bakuli ba nang le ts'oaetso e tlase ea MDS e khethiloeng ke International Prognostic Scoring System, kapa IPSS, le lipalo tse tsitsitseng tsa mali (CBC) , ka linako tse ling mokhoa o nepahetseng oa ho phekola ke ho shebella le tšehetso, ha ho hlokahala.
Tabeng ena, o tla lokela ho hlahlojoa bakeng sa liphetoho marong a hau a ka bonang tsoelo-pele ea lefu lena. Li-CBC tse tloaelehileng, hammoho le aspirate ea mofu oa masapo le li-biopsy , e ka ba karolo ea ho shebella.
Tlhokomelo e Tšehetsang
Tlhokomelo e tšehetsang e bua ka phekolo e sebelisoang ho phekola le ho laola MDS; lits'ebeletso tsena li ka ntlafatsa haholo boemo ba motho, empa li khaotsa ho hlasela lisele tse bakang MDS.
Litšisinyeho
Haeba mali a hao a qala ho oela 'me u bona matšoao, u ka rua molemo ka ho tšeloa lisele tse khubelu tsa mali kapa liplatelete. Qeto ea ho tšeloa mali e tla itšetleha ka maemo a mang a bophelo bo botle le hore na u ikutloa joang.
The Iron Overload le Chelation Therapy
Haeba u qala ho hloka tšelo ea mali hangata khoeli le khoeli, u ka 'na ua ba kotsing ea ho hlaolela boemo bo boima bo nang le tšepe.
Mefuta e phahameng ea tšepe e tšeloang mali ea lefutso ea mali e ka baka ho eketseha ha mabenkele a tšepe 'meleng oa hau. Mefuta e phahameng ea tšepe e ka senya litho tsa hau.
Lingaka li ka tšoara le ho thibela tšepe e lekaneng ho tloha tšebetsong e mengata ea tšelo ea mali ho sebelisoa meriana e bitsoang iron chelators, e akarelletsang phekolo ea molomo, deferasirox (Exjade), kapa infusion e bitsoang deferoxamine mesylate (Desferal). Tataiso ea Koetliso ea Kankere ea Naha e Khethehileng, kapa NCCN, e fana ka litekanyetso tseo ngaka ea hao e ka li sebelisang ho etsa qeto ea hore na u hloka phekolo ea tšepe ea chelation.
Lintho tsa ho Hōla Batho ba bang ba nang le mali a mali a MDS ba ka rua molemo ka ho fumana lintlha tse eketsehileng tsa meriana e bitsoang erythropoietin e susumetsang baemeli kapa liprotheine (ESAs).
Mehlala ea li-ESA li kenyelletsa epoetin alfa (Eprex, Procrit kapa Epogen) kapa nako e telele e sebetsanang le darbepoetin alfa (Aranesp). Lithethefatsi tsena li fanoa e le ente e entsoeng ka mafura a hau a mafura. Le hoja lithethefatsi tsena li sa thuse bakuli bohle ba MDS, li ka 'na tsa thusa ho thibela ho tšeloa mali ho ba bang.
Ngaka ea hao e ka 'na ea u fa tšebetso ea ho qala ka ntho e hlasimollang kolone , e kang G-CSF (Neupogen), kapa GM-CSF (leukine) , haeba palo ea hao ea lisele tsa mali e tšoana ka lebaka la MDS ea hau. Lintho tse susumetsang Colone li thusa ho matlafatsa 'mele oa hao ho hlahisa mafu a mangata a loantšang lisele tse tšoeu tsa mali tse bitsoang neutrophils. Haeba litekanyetso tsa hau tsa neutrophile li le tlaase, u kotsing e kholo ea ho ba le tšoaetso e kotsi. E-ba leihlo bakeng sa matšoao leha e le afe a tšoaetso kapa feberu, 'me u bone mofani oa tlhokomelo ea bophelo hang ha ho khoneha haeba u amehile.
Phekolo e fokolang haholo
Phekolo e matla haholo e bua ka tšebeliso ea lik'hemotherapy e tlaase haholo kapa mahlahana a tsejoang e le li-modifiers tse arabelang likarabo. Litlhare tsena li fuoa tlhokomelo ea mokuli, empa tse ling tsa tsona li ka 'na tsa hloka tlhokomelo ea ts'ehetso kapa ka linako tse ling sepetlele, ka mohlala, ho phekola tšoaetso e hlahang.
Therapy Epigenetic
Sehlopha sa meriana e bitsoang hypomethylating kapa demethylating agent ke lihlomo tse ncha ka ho fetisisa khahlanong le MDS.
Azacitidine (Vidaza) e amohetsoe ke FDA ho sebelisoa likarolo tsohle tsa French-American-British (FAB) le likarolo tsohle tsa kotsi tsa IPS tsa MDS. Hangata meriana ena e fanoa e le ente e ka tlaase ho matsatsi a 7 ka tatellano, matsatsi a mang le a mang a 28 bakeng sa bonyane maqhubu a 4-6. Liphuputso tsa azacitidine li bontšitse litekanyetso tsa likarabo tsa karolo ea 60 lekholong, 'me hoo e ka bang karolo ea 23 lekholong e finyella ho finyelloa ka mokhoa o sa lokelang kapa o feletseng oa lefu la bona. Hangata Azacitidine e etsa hore lekhetlo la pele le hlahe ka seleng ea mali le se ke la hlola le hlaphoheloa ho fihlela kamora 'ngoe kapa lipotso tse peli.
Mofuta o mong oa moemeli oa hypomethylating o sebelisetsoang ho phekola MDS ke decitabine (Dacogen). Ho tšoana haholo le ho etsoa hozacitidine, hape ke FDA e amohelehang bakeng sa mefuta eohle ea MDS. Mokhoa oa phekolo o ne o atisa ho amahanngoa le mefuta e fokolang ea mofuta o joalo, 'me kahoo e nkoa e le theknoloji e tlaase ea matla. Decitabine e ka fuoa intravenously kapa subcutaneously. Phuputso e 'ngoe eo ho eona decitabine e ileng ea fuoa intravenously ka matsatsi a 5 e bontšitse tekanyo e feletseng ea ho fokotsa likarolo tse ka bang 40 lekholong. Mefuta e meng ea li-dosing e ntse e phenyekolloa.
Therapy The Immunosuppressive Therapy le Libibele tse arabelang likarabo
Ka MDS, lisele tse khubelu tsa mali, lisele tse tšoeu tsa mali le li-platelet li bolaoa kapa li shoa pele li se li hōlile ka ho lekaneng hore li ka lokolloa marong oa masapo ho ea maling. Maemong a mang, lymphocytes (mofuta oa sele e tšoeu ea mali) li ikarabella bakeng sa sena. Bakeng sa bakuli, ho ka 'na ha e-ba molemo ho sebelisa phekolo e amehang tsamaisong ea' mele ea ho itšireletsa mafung.
Li-chemotherapy, tse nang le matla a tlaase (li-biologic response modifiers) li kenyelletsa anti-thymocyte globulin (ATG), cyclosporine, thalidomide, lenalidomide, anti-tumor necrosis factor receptor fusion protheine, le vithamine D tse tšoanang. Tsena kaofela li bontšitse bonyane litekong tsa pele, empa ba bangata ba hloka liteko tse ling tsa kliniki ho utloisisa katleho mefuteng e fapaneng ea MDS.
Batho ba nang le mofuta o itseng oa MDS o bitsoang 5q-syndrome, moo ho nang le bothata ba lefutso ka chromosome 5, e ka 'na ea e-ba le karabo ho lithethefatsi e bitsoang lenalidomide (Revlimid). Ka tloaelo, lenalidomide e sebelisoa ho bakuli ba tlaase kapa ba tlaase-tlaase IPSS kotsi ea MDS e nang le bothehi ba tšelo ea mali ea lefutso. Liphuputsong tsa lenalidomide, bakuli ba bangata ba fokotsitse litlhoko tsa ho tšeloa mali - hoo e ka bang karolo ea 70 lekholong, ha e le hantle - empa ba tsoela pele ho ba le lipalo tse tlaase le li-neutrophil. Melemo ea ho phekola MDS, kapa subtypes e kgolo ho feta 5q-syndrome le lenalidomide e ntse e ntse e ithutoa.
Phekolo e phahameng haholo
Chemotherapy
Bakuli ba bang ba nang le kotsi e kholo ea MDS, kapa mofuta oa FAB RAEB le RAEB-T, ba ka phekoloa ka chemotherapy e matla. Khemotherapy ena, mofuta o tšoanang o sebelisetsoang ho phekola kankere ea myelogenous leukemia (AML) e matla, e ikemiselitse ho senya palo ea lisele tse sa tloaelehang masong a masapo a isang MDS.
Le hoja chemotherapy e ka ba molemo ho bakuli ba bang ba MDS, ke habohlokoa ho nahana hore bakuli ba nang le maemo a mang a bophelo bo botle ba tobana le likotsi tse eketsehileng. Melemo ea phekolo eo e ka ba molemo ho feta kotsi e amehang.
Lipatlisiso li tsoela pele ho bapisa liphello tsa chemotherapy e matla ho feta tsa azacitidine kapa decitabine.
Ho fetisetsa Cell Cell Stem
Bakuli ba nang le kotsi e kholo ea IPSS MDS ba ka khona ho fumana phekolo ea lefu la bona ka allogeneic stem cell transplantation . Ka bomalimabe, tlhaho e kotsing ea mokhoa ona e fokotsa tšebeliso ea eona. Ha e le hantle, transplant ea allogeneic stem cell cell e ka ba le tekanyo ea lefu le amanang le lefu ea ho fihlela ho 30%. Ka hona, phekolo ena e tloaelehile feela ho bakuli ba banyenyane ba phelang hantle.
Liphuputso tsa morao-rao li ntse li etsa lipatlisiso ka karolo ea li-non-myeloablative tse bitsoang "mini" tse fetisetsang ho batho ba baholo ba nang le MDS. Le hoja mefuta ena ea ho kenya limela e 'nile ea nkoa e le e tlase ho feta mekhahlelo e tloaelehileng, ho fokotseha ha bona ho ka etsa hore ba be le khetho bakeng sa bakuli ba neng ba ke ke ba lumelloa.
Kakaretso:
Ka lebaka la mefuta e sa tšoaneng ea MDS le mefuta e sa tšoaneng ea mokuli, ha ho na phekolo e lekanang le boholo ba motho. Ka hona ke habohlokoa hore bakuli ba MDS ba buisane ka litsela tsohle ka sehlopha sa tlhokomelo ea bophelo, 'me u fumane phekolo e tla ba fa melemo e molemo ka ho fetisisa ea chefo.
Liteko tsa meriana ea meriana le mekhoa e mecha ea phekolo ea MDS e ntse e tsoela pele, kahoo lula u lutse. Ka mohlala, ruxolitinib (Jakafi) e ntse e phenyekolloa bakeng sa phekolo ea bakuli ba nang le kotsi e tlase kapa e bohareng ba 1 MDS.
Lisebelisoa:
Greenberg PL, Attar E, Bennett JM, et al. Myelodysplastic Syndromes: Litlhahiso tsa Meriana ea Boipheliso Litlhapelong ho Oncology. JNCCN. 2013; 11 (7): 838-874.
Kantarjian H, O'Brien S, Giles F, le al. Decitabine kemiso e tlaase ea tekanyo ea tekano (100 mg / m2 / Koetliso) myelodysplastic syndrome (MDS). Ho bapisa mananeo a mararo a fapaneng. Mali. 2005; Sekhetho sa 2522.
Malcovati L, Hellström-Lindberg E, Bowen D, et al. Ho lemoha le ho phekola li-syndromes tsa mantlha tse ka sehloohong ho batho ba baholo: litlhahiso tse tsoang ho European LeukemiaNet. Mali . 2013; 122 (17): 2943-2964.
Nimer, S. "Myelodysplastic Syndromes" Mali May 2008. 111: 4841- 4851.
Scott, B., Deeg, J. "Myelodysplastic Syndromes" Tlhahlobo ea Selemo le Selemo ea Meriana 2010. 61: 345-358.