I-anemia ephazamisayo enezikhukhula ezidlulayo, noma i-RAEB, ibhekisela enkingeni yamaseli awenza igazi. I-RAEB ingenye yezinhlobo ezingu-7 zezifo ezinjalo, noma ama- syndrome ( myelodysplastic syndromes ) (MDS), okuqashelwa yi-World Health Organization (WHO), okuhlukanisa phakathi kwezigaba ezimbili ze-RAEB: RAEB-1 no-RAEB-2.
Zombili amafomu ngokuvamile athatha ukulandelwa okunzima: izikhathi ezishicilelwe zokusinda (manje zikhona) zivela ezinyangeni ezingu-9-16.
I-RAEB nayo ihlotshaniswa nengozi ephakeme yokuqhubekela phambili emthonjeni we -myeloid leukemia -umdlavuza wezingqamuzana ezenza igazi emnothweni wethambo .
Ukuqonda i-RAEB, uhlobo lwe-MDS
I-Myelodysplastic syndrome, noma i-MDS, ibhekisela emndenini wezifo ezingavamile zegazi lapho umnkantso wesithambo ungakhiqizi okwanele amangqamuzana egazi abomvu, amaseli egazi amhlophe noma amaplatelet. I-RAEB yinhlobo ejwayelekile ye-MDS, futhi ngeshwa, kuyindlela ephakeme kakhulu engozini ye-MDS.
Njengezinye izinhlobo ze-MDS, i-RAEB ivame ukuthinta abantu abangaphezu kweminyaka engama-50, kodwa kungenzeka kubantu abancane, futhi futhi imbangela yakhe engaziwa okwamanje.
Uma umuntu enesimo se-MDS efana ne-RAEB, umnyofu wethambo ungakhipha eziningi ezingakhiwe phansi, noma ezingapheli, amangqamuzana ngokuvamile anezinhlobo ezingafani, ubukhulu noma ukubukeka, uma kuqhathaniswa nempilo enempilo. Lezi zingane ezisencane, izinguquko zamangqamuzana egazi kuthiwa ziqhuma amaseli -igama elisetshenziswe kaningi ekuxoxweni kwegazi.
Ngempela, namuhla ososayensi abaningi babheka i-MDS njengendlela yegazi nomdlavuza womnyo we-bone.
Izinhlelo zokuhlukanisa ezahlukene ziye zasetshenziselwa lezi zinkinga. Uhlelo lwesigaba se-WHO luzama ukuhlunga izinhlobo ze-MDS, ngokucophelela ukuchazwa kwesifo esinikeziwe. I-WHO manje ihlonipha izinhlobo ezingu-7 ze-MDS, futhi ndawonye RAEB-1 ne-RAEB-2 akhawunti cishe ngamaphesenti angu-35-40 kuwo wonke amacala we-MDS.
- I-cytopenia ekhonkisayo nge-unilineage dysplasia (i-RCUD)
- I- anemia ephazamisayo ne-sideroblasts enezinsimbi (RARS)
- I-cytopenia ekhonkisayo nge-multilineage dysplasia (i-RCMD)
- I-anemia ekhumbule enezikhukhula ezidlulele-1 (RAEB-1)
- I-anemia engenalutho ene-explosion excess-2 (RAEB-2)
- I-Myelodysplastic syndrome, engaxhunyiwe (MDS-U)
- I-Myelodysplastic syndrome ehlobene ne-isol del (5q)
Lawa magama angenhla avame ukubhekisela ekutheni i-blood kanye namaseli omnyo wesibonakala avela kanjani, uma ihlolwe ngaphansi kwe-microscope. Igama lokugcina ohlwini olungenhla, noma kunjalo, luchazwa ukuguqulwa okuthile, noma ushintsho lwe-chromosome, ekuphatheni kwezakhi zofuzo ze-cell-forming bone marrow cell.
Endabeni ye-RAEB (zombili izinhlobo), igama linamalungu amabili: i-anemia yokuphikisa; kanye nokuqhuma ngokweqile. I-anemia, ngokuvamile, ukungabi namalungu amangqamuzana egazi abomvu okwanele. I-anemia ephazamisayo isho ukuthi i-anemia ayikho ngenxa yezizathu ezivamile eziyaziwayo ze-anemia nokuthi ngokuvamile i-anemia iyalungiswa kuphela ngokumpompela igazi. Uma umuntu ene-anemia kanye nokuhlolwa kwe-refractory yembula inani elikhulu lamangqamuzana aphefumulayo angaphezu kokujwayelekile, i-anemia ekhanyisayo enezikhukhula ezingaphezulu.
Kungenzeka ukuthi umuntu ophethe i-RAEB abe nokubala okuphansi kwamanye amangqamuzana akhiwa ngethambo lomthambo.
Abantu abane-RAEB bangase babe ne-anemia engabonakali (amangqamuzana egazi abomvu aphansi), i-neutrophils engenalutho (i-neutrophils ephansi), i-thrombocytopenia ekhonkisayo (amapleletthi aphansi), noma inhlanganisela yezintathu.
I-RAEB iyindlela ephakeme kakhulu ye-MDS
Kulezi ziguli ezitholakale ezine-MDS, kubalulekile ukucacisa izinga lezingozi. Ezinye izinhlobo ze-MDS zinengozi encane, ezinye izingozi ezingaphakathi, kanti ezinye zibeka ingozi enkulu. Zombili i-RAEB ne-RCMD zibhekwa njengezinhlobo eziphezulu ze-MDS. Noma kunjalo, akuzona zonke iziguli ezine-RAEB ezinokufanisa okufanayo. Ezinye izici ziqala ukudlala, njengokudala, impilo yonke, izici zesifo, kanye nezakhi zofuzo ezibandakanya amaseli okwenza amathambo.
Ukuxilongwa
Lapho i-MDS icatshangwa, kufanele kwenziwe i-marrow biopsy ne-aspirate. Lokhu kuhilela ukuthola amasampula omnkantso wamathambo futhi ubathumele ebhokisithri ukuze kuhlaziywe futhi kuchazwe.
Ukuxilongwa kwenziwa ngokusekelwe ekusebenzeni kwamangqamuzana ngaphansi kwe-microscope, ukuthi bahlotshiswe kanjani ngamaqoqo ahlukene amatala kanye namamaki ahlanganisa ukusetshenziswa kwamagciwane njengamathegi, futhi, uma kwenzeka kune-subSpes ephakeme kakhulu ye-MDS, okuthiwa yi-cytometry yokugeleza . I-cytometry yokugeleza iyindlela evumela amaseli anezici ezithile ukuthi abonakale futhi ahlungwe kusukela kubantu abaningi beseli kwisampuli esinikeziwe.
Izinhlobo
Zombili amafomu (1 no-2) we-RAEB afakwe engozini yokuqhubekela phambili ku-acute myeloid leukemia (AML). Ukwengeza, isiguli esinomthelela ophezulu we-MDS njengo-RAEB singase siphumelele ekuhlulekeni komnyofu, ngaphandle kokuqhubeka kwe-AML, ngakho-ke isimo sisengcupheni yokuphila yedwa, ngaphandle kokuqhubeka ne-leukemia.
I-terminology ehlobene ne-RAEB
Ukuhlelwa kwe-RAEB kuncike ekuqondeni kwemigomo eminingana:
- I-bone marrow blast ibala : Kutholakala isampula somnyosa wakho, futhi inani lezinhlamvu ezingavamile, ezifayo ezingalingani zilinganiswa.
- Ukubala kwegazi okubhebhethekayo: Isampula egazini lakho elivela emthanjeni lidonsa besebenzisa inaliti, futhi inani lamangqamuzana angavamile, amangqamuzana amancane alinganiswa.
- Izindlebe ze-Auer : Lokhu kuyinto odokotela abayoyibheka lapho bebona ukuqhuma kwakho ngaphansi kwe-microscope. Ngisho noma kuthiwa yi-Auer "izinduku," empeleni ziza ngezimo nobukhulu obuhlukahlukene. Zincane-ezincane kune-nucleus, futhi zitholakala ngaphakathi kwe-cytoplasm. Ngokuvamile zinezinaliti ezinomumo ophethwe, kodwa zingabe zifana nombala, zenziwe ngedayimani, noma zinde futhi ziningi ngaphezulu.
Ngokususelwa ebukhosini noma ukungabikho kweziphakamiso ezingenhla, umuntu uzimisele ukuba ne-RAEB-1 noma i-RAEB-2 kanje:
Iziguli zitholwa ukuthi zinesifo se-RAEB-1 uma ngabe (1) umthambo we-bone umbala phakathi kwama-5 no-9 amaphesenti okungenani amangqamuzana angama-500 abalwa noma (2) ukubalwa kwe-peripheral blast phakathi kwama-2 no-4 amaphesenti okungenani amangqamuzana angu-200 abalwa, futhi (3) amakhodi akwa-Auer engekho. Ukuba khona kwesilinganiso 1 noma 2 plus 3 kubeka icala le-MDS njengo-RAEB-1.
Amathuba ka-RAEB-1 aphendukela ebuchosheni obukhulu besifo se-myeloid kulinganiselwa cishe ngamaphesenti angu-25.
Iziguli zitholwa nge-RAEB-2 uma ngabe (1) umthambo wesibhakela ubala phakathi kwamaphesenti angu-10 no-19 okungenani amangqamuzana angama-500 abalwa noma (2) ukubalwa kwesiphambano phakathi kwamaphesenti angu-5 no-19 okungenani amangqamuzana angama-200 abalwa, noma (3) izintambo ze-Auer zitholakala. Ukuba khona kweminye imikhawulo 1, 2 noma 3 kuhlukanisa icala le-MDS njengo-RAEB-2.
Kulinganiselwa ukuthi amathuba okuba yi-RAEB-2 aphenduke i-acute myeloid leukemia angase abe ngamaphesenti angama-33 kuya kwangu-50.
Kuyini i-RAEB-T?
Ungase uhlangane nkulumo ethi "i-anemia ekhanyelayo ngokuqhuma ngokweqile ekuguqulweni," noma i-RAEB-T. Leli gama liye lashiywa yi-WHO-classification of syndromes.
Iziguli eziningi ngaphambili ezazingaphansi kwalesi sigaba manje zihlukaniswe njengegazi elinomdlavuza we-myeloid. Kuhlelo lokuhlukanisa oluhlukile, isiFulentshi-American-British (i-FAB-classification), iziguli zanikezwe esigabeni se-RAEB-T uma ngabe (1) umthambo wamathambo ubala phakathi kwamaphesenti angu-20 no-30, (2) ukubalwa kwe-peripheral okungenani okungamaphesenti angu-5, noma (3), izintambo ze-Auer zitholakala, kungakhathaliseki ukuthi inani lokuqhuma.
Kukhona ukuphikisana okuqhubekayo mayelana nokubaluleka kokuhlukanisa i-RAEB-T njengenqubo ye-FAB, ngokwehlukana "ne-AML-20-30," njengenqubo ye-WHO. Izivivinyo eziningana zemitholampilo emikhulu eminyakeni yamuva zisebenzise igama elithi RAEB-T, naphezu kwezinguquko ohlelweni lwezinhlelo ze-WHO. Umgomo ophansi weziguli nabanikezeli bezempilo kubonakala sengathi kungabalulekile ukwazi ukuthi kukhona amagama asetshenziswayo, ukuze angaphuthelwa ithuba lokubhalisa esilingo somtholampilo.
I-RAEB iphathwa kanjani?
Ukwelashwa kwe-RAEB kuhluke ngezimo ezihlukile. Impilo yobudala bomuntu ngamunye ingase ibangele ezinqumweni ezinjalo zokwelashwa. Iziguli ezine-RAEB kufanele zithole izibuyekezo ekugonyeni kwazo, futhi abashayeli nge-RAEB bakhuthazwa ukuba bayeke ukubhema. Izimpawu ukuthi i-RAEB ingaqhubeka ihlanganisa ukutheleleka okuvamile, ukuphuma kwegazi okungavamile, ukulimaza, nesidingo sokumpompela igazi njalo.
Akuzona zonke iziguli ezine-MDS ezidinga ukwelashwa okusheshayo, kodwa iziguli ezinezibalo eziphansi zesifo (i-anemia, thrombocytopenia, ne- neutropenia enezifo ezijwayelekile), futhi lokhu kufaka iziguli eziningi ezinezinkinga eziphezulu noma eziphezulu kakhulu ezibandakanya i-MDS (kufaka phakathi i-RAEB-2, emelela phezulu ibanga le-MDS ne-prognosis eyimpofu kakhulu).
I-Practice Guidelines ye-National Comprehensive Cancer Network (NCCN) ihlanganisa impilo kanye nokusebenza jikelele komuntu ngamunye, uhlelo lwe-International Prognostic Scoring System (IPSS) kanye nezigaba zezinhlekelele ze-IPSS (IPSS-R) ze-MDS, nezinye izici zesifo ukuze kusize izinqumo zokuphatha. Akukho "usayizi owodwa ohambisana nayo yonke" indlela yokwelapha abantu abane-RAEB, noma kunjalo.
Ngokuvamile kunezigaba ezintathu zokwelashwa : ukunakekelwa okusekelayo, ukwelapha okunamandla kakhulu, kanye nemithi ephakeme kakhulu. Lezi zonyango zichazwe ngezansi:
- Ukunakekelwa okusekelayo kuhlanganisa ama-antibiotics okutheleleka kanye namaseli abomvu nama-platelet transfusion for counts low counts.
- Izindlela zokwelashwa eziphansi kakhulu zihlanganisa izici zokukhula kwegazi, ezinye izinhlaka ezifana ne-azacitidine ne-decitabine, ukwelashwa kwe-immunosuppressive, kanye ne-chemotherapy ephansi kakhulu. Lezi zokwelapha zingakhululwa ngesineke futhi zingathuthukisa izimpawu kanye nekhwalithi yokuphila, kodwa aziphilisi isimo.
- Izindlela zokwelapha eziphakeme kakhulu zihlanganisa ukuhlanganiswa okukhulu kwe-chemo ne-allogeneic transplant bone. Lezi zithimba zidinga ukunakekelwa esibhedlela futhi zenze ingozi yemiphumela emibi yokuphila, kodwa futhi zingakwazi ukuthuthukisa igazi libala ngokushesha kunokwelashwa okuncane kakhulu futhi lingashintsha indlela isimo esizoqhubeka ngayo. Kuphela abantu abathile kuphela abazokhethwa ukwelashwa okukhulu kakhulu.
Ukuvivinywa kwemitholampilo nakho kuyindlela yokuthola iziguli ezithile. Akukona nje isikhathi esidlule, eqinisweni, kwakukhona isilingo somtholampilo esibonisa izinzuzo nge-decitabine, uma kuqhathaniswa nokunakekelwa okungcono okusekelayo, kuziguli ezindala ezine-anemia ezinezikhukhula ezingaphezulu kwenguquko (RAEBt).
Izwi elivela
Uma uthola ukuthi une-RAEB-1, RAEB-2, noma unomunye uhlobo lwe-MDS ozobhekwa njengengozi enkulu, khuluma neqembu lakho lokunakekelwa kwezempilo mayelana nezinketho zakho.
Kulezi ziguli ezine-MDS engozini ephakeme, i-azacitidine (i-5-AZA, i-Vidaza) ne-decitabine (i-Dacogen) yimithi emibili evunywe yi-FDA ye-MDS ukuthi ithimba elibhekele ukunakekelwa kwakho lingacubungula. Lezi zidakamizwa kuthiwa yi-hypomethylating agents.
Amaqembu amaningana okubambisana abonise ukuthi, ngenxa ye-MDS, i-allogeneic HSCT (ukufakelwa komnyofu we-bone) noma ukwelapha nge-hypomethylating agents kumele kuqaliswe ngokushesha. I-HSCT yama-allogeneki (ukuguqulwa kwamathambo emnothweni ovela kumnikezeli) yiyona ndlela kuphela yokuphefumula eya ku-MDS, kodwa, ngeshwa, kuyindlela engokoqobo yeziguli ezimbalwa kakhulu, ngenxa yeqembu elidala elichaphazelekayo yi-MDS, ngokubambisana nempilo engapheli izimo nezinye izici eziqondene neziguli.
> Imithombo:
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> Holkova B, Supko JG, u-Ames MM, et al. Isigaba i-trial of vorinostat ne-alvocidib ezigulini ezine-relapsed, refractory or poor prognosis, i-leukemia enkulu, noma i-anemia ekhanyela ngokuqhuma ngokweqile-2. I-Clin Cancer Res. 2013; 19 (7): 1873-1883.
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> Pleyer L, Burgstaller S, Stauder R, et al. I-Azacitidine phambili-line ku-339 iziguli ezine-myelodysplastic syndromes kanye ne-lemonemic acute lemeemia: ukuqhathanisa izigaba zeFrance-American-British kanye ne-World Health Organization. J Hematol Oncol. 2016; 9: 39.