I-anemia ye-refractory ene-excess blasts, okanye i-RAEB, ibhekisela kwingxaki yeeseli ezenza igazi. I-RAEB yenye yeentlobo ezisixhenxe zeengxaki ezinjalo, okanye i- myelodysplastic syndromes (MDS), ebonwa yiSigaba soMhlaba wezeMpilo (WHO), ekwahlula phakathi kweendidi ezimbini ze-RAEB: RAEB-1 kunye ne-RAEB-2.
Ezi zombini iifom zithwala ubunzima bokuthi: izihlandlo zokusinda eziqhelekileyo ezishicilelweyo (ngoku zixesha) zivela kwiinyanga ezi-9-16.
I-RAEB nayo idibene nomngcipheko okhuseleyo wokuqhubela phambili kwi -leukemia engummangaliso- umhlaza weeseli ezenziwe ngegazi lomnatha wethambo .
Ukuqonda i-RAEB, uhlobo lwe-MDS
I-Myelodysplastic syndrome, okanye i-MDS, ibhekisela kwintsapho yezifo ezingabonakaliyo egazini apho umongo wethambo ungavelisi okwaneleyo iiseli zegazi ezibomvu, iiseli zegazi ezimhlophe okanye iiplatelet. I-RAEB yinto eqhelekileyo ye-MDS, kwaye ngelishwa, ifom ephezulu yengozi ye-MDS.
Njengazinye iifom ze-MDS, i-RAEB ixhaphazela abantu abangaphezu kweminyaka engama-50, kodwa inokuthi ivele kubantu abatsha, ngokunjalo, kunye nesizathu esingaziwayo ngoku.
Xa umntu enefom ye-MDS efana ne-RAEB, umongo wethambo unokuvelisa uninzi olungaphantsi, okanye oludala, iiseli ezidla ngokubunzima, ubukhulu okanye ukubonakala, xa kuthelekiswa nempilo. Ezi zakuqala, iintsana, iinguqu zegazi zibizwa ngokuba yi- cell cells -igama elisetyenziswa ngokuqhelekileyo kwingxoxo ye-leukemia.
Enyanisweni, namhlanje abaninzi abanzululwazi bajonga i-MDS njengendlela yegazi kunye nomhlaza wesifo somnyo.
Iinkqubo ezahlukeneyo zokuhlalutya ziye zasetyenziselwa ezi ngxaki. Inkqubo yesigaba se-WHO izama ukuhlenga iindidi ze-MDS, ingqalelo kwi-prognosis ye-disorder. I-WHO ngoku yaqonda iindidi ezi-7 ze-MDS, kunye kunye ne-RAEB-1 kunye ne-RAEB-2 akhawunti malunga neepesenti ezingama-35-40 zazo zonke iimeko ze-MDS.
- I-cytopenia ye-Refractory kunye ne-unilineage dysplasia (i-RCUD)
- I- anemia ye- Refractory enee-sideroblasts eziphambeneyo (RARS)
- I-cytopenia ye-Refractory ene-multilineage dysplasia (i-RCMD)
- I-anemia ye-Refractory ene-blast-excess-1 (RAEB-1)
- I-anemia ye-refractory ene-blast-ex 2-RAEB-2)
- I-Myelodysplastic syndrome, i-unclassified (MDS-U)
- I-Myelodysplastic syndrome edibene ne-del del (5q)
La magama angentla ahlala ebhekisela kwindlela igazi kunye neeseli zethambo lomzimba ezibonakala ngayo, xa zihlolwe phantsi kwe-microscope. Igama lokugqibela kule nkcukacha engenhla, nangona kunjalo, ichazwa nginguqu oluthile, okanye utshintsho lwe-chromosome, kwizinto eziphathekayo zofuzo zeeseli zamathambo esele.
Kwimeko ye-RAEB (zombini iindidi), igama linamacala amabini: i-anemia yokukhanyela; kunye nokugqithiswa kwemfuneko. I-anemia, ngokubanzi, ukungabikho kwintsholongwane yegazi ebomvu okwaneleyo. I-anemia ye-refractory ithetha ukuba i-anemia ayikho ngenxa yezizathu eziqhelekileyo ezibangelwa i-anemia kwaye ukuba i-anemia ngokubanzi iyalungiswa kuphela nge-transfusion. Xa umntu ene-anemia kunye neemvavanyo eziphazamisayo zibonisa inani elikhulu lezisele ezingaphantsi kokuqhelekileyo kunokuba ziqhelekileyo, yi-anemia yokuphazamiseka ngokugqithiseleyo.
Kungenzeka ukuba umntu onama-RAEB abe namaxabiso aphantsi kwezinye iiseli ezenziwe ngumbongo wethambo.
Abantu abane-RAEB bangenayo i-anemia (i-red blood cells), i-neutrophils engaphantsi (i-neutrophils ephantsi), i-thractytopenia engabonakaliyo (iplatelet ephantsi), okanye idibanisa ezintathu.
I-RAEB yindlela ephezulu ye-MDS
Kwizigulane ezixilongwa nge-MDS, kubalulekile ukuchonga izinga leengozi. Ezinye iifom ze-MDS ziphantsi komngcipheko, ezinye zibeka umngcipheko phakathi, kunye nabanye abasengozini. Zombini i-RAEB kunye ne-RCMD zibhekwa njengeengcipheko eziphezulu zengozi ye-MDS. Sekunjalo, akubona zonke izigulane ezine-RAEB ezifanayo. Ezinye izinto ziza kudlala, ezifana nobudala, impilo yonke, izifo zesifo, kunye ne-genetics yama-cell-forming cells.
Ukuxilongwa
Xa i-MDS ikhunjulwa, umongo we-bone and aspirate kufuneka uyenze. Oku kubandakanya ukufumana iisampulu zentsipho yethambo kwaye zizithumele ebhokisatri ukuhlalutya nokutolika.
Ukuxilongwa kwenzelwa ngokusekelwe kwindlela iiseli ezibonakala ngayo ngaphantsi kwe-microscope, ukuba zihlambuluke njani ngeesethi ezahlukeneyo zedayi kunye namakishi aquka ukusetyenziswa kwamagciwane ashegi, kwaye, kwimeko ye-subtypes ephezulu kakhulu ye-MDS, into ebizwa ngokuba yi-cytometry flow flow . I-cytometry ehambayo yinkqubo evumela ukuba iiseli ezineempawu ezithile zichongwe kwaye zihlulwe ukusuka kubemi abaninzi beseli kwisampuli esinikeziwe.
Iintlobo
Zombini iifom (1 no-2) ze-RAEB zidibene nomngcipheko wokuqhubela phambili kwi-acute leememiaid (AML). Ukongezelela, isigulane esinomngcipheko we-MDS onjenge-RAEB sinokutshatyalaliswa kokungaphumeleli komnatha we-bone, ngaphandle kokuqhubela phambili kwi-AML, kwaye ngoko imeko ihlala isongela yedwa, ingenayo i-leukemia.
I-Terminology ehlobene ne-RAEB
Ukwahlulelwa kwe-RAEB kuxhomekeke ekuqondeni kwamagama ambalwa:
- Ukubalwa komnyola we-Bone : I-sampuli yamathambo akho atholakala, kunye nenani elingaqhelekanga, elingaqhelekanga.
- Ukubala kwegazi okuphambene nomzimba: Isampuli yegazi lakho ukusuka kwisithambiso isetyenziswe ngokusebenzisa inaliti, kwaye inani lezinto ezingavamile, iiseli ezifayo zingalinganiswa.
- Iindonga ze-Auer : Yiyo into oogqirha abaya kuyijonga xa bebona ukuqhuma kwakho phantsi kwe-microscope. Nangona kuthiwa yi-Auer "iindonga," ngokwenene bafika ngeendlela ezahlukeneyo kunye nobukhulu. Zincinci-ezincinci kuneyona nucleus, kwaye ziyafumaneka ngaphakathi kwe-cytoplasm. Ngokuqhelekileyo zinemizobo enesilingi kunye neziphelo eziqingqiweyo, kodwa zinokuba zi-comma-shaped, zenziwe ngedayimani, okanye zide kwaye zininzi zixande.
Ngokusekelwe kubukho okanye ukungabikho kwezi ziphumo ezingentla, umntu uzimisele ukuba ne-RAEB-1 okanye i-RAEB-2 ngale ndlela:
Izigulane zifunyaniswa ne-RAEB-1 ukuba zikhona (1) i-bone marrow blast ibalwa phakathi kwe-5 ne-9 ekhulwini yee-ateresenti ezingama-500 ezibalwe okanye (2) ukubalwa kwe-peripheral blast phakathi kwe-2 ne-4 ekhulwini yeeseli ezingama-200 ezibalwa, kunye (3) neentsimbi ze-Auer ezingekho. Ubukho be-1 okanye ye-2 kunye ne-3 kunye no-3 lubeka icala kwi-MDS kwimeko ye-RAEB-1.
Amathuba e-RAEB-1 ajika kwi-leukemia engummangaliso eqikelelwa malunga nama-25 ekhulwini.
Izigulane zifunyaniswa ne-RAEB-2 ukuba zinokuthi (1) i-bone marrow blast ibalo phakathi kwe-10 ne-19 yeepesenti ezingama-500 ezingama-cell count okanye (2) ukubalwa kwe-peripheral blast phakathi kwama-5 no-19 ekhulwini ngamanqanaba angama-200 abalwa, okanye (3) Iintambo ze-Auer ziyafunyanwa. Ubukho beendlela zoku-1, 2 okanye 3 zibeka iimeko ze-MDS njenge-RAEB-2.
Kuqikelelwa ukuba amathuba a-RAEB-2 ajika kwi-leukemia enesifo esingaqhelekanga anokufikelela kuma-33 ukuya kuma-50 ekhulwini.
I-RAEB-T?
Unokuhlangabezana nalo binzana elithi "i-anemia yokukhanyela ngokugqithiseleyo kwenguqu," okanye i-RAEB-T. Leli gama liye lashiywa ngokutsha kwi-WHO-classification syndromes.
Uninzi lwezigulane zangaphambilini ezikweli candelo zihlulwe njengoluhlu lwe-leukemia olunzulu. Kwinkqubo eyahlukileyo yohlulo, i-French-American-British (i-FAB-classification), izigulane zinikezelwe kwisigaba se-RAEB-T ukuba ngaba (1) i-bone marrow blast ibalwa phakathi kwe-20 ne-30 ekhulwini, (2) kubalwa ubuncinane beepesenti ezingama-5, okanye (3), iintonga ze-Auer ziyafunyanwa, kungakhathaliseki ukubalwa kwenani.
Kuqhubeka ukuphikisana malunga nexabiso lokubeka i-RAEB-T njengenkqubo ye-FAB, ngokwahlukileyo kwi-"AML-20-30," njengendlela ye-WHO. Uninzi lwezilingo zamaklinikhi amakhulu kwiminyaka yakutshanje lusetyenzise igama elithi RAEB-T, nangona kukho utshintsho kwinkqubo yodidi lwe-WHO. Umgca ophezulu kwizigulane kunye nabanikezeli benonophelo lwempilo kubonakala ngathi kubalulekile ukwazi ukuba kukho isigama esichazayo, ukuze ungaphumeleli ithuba lokubhalisa kwilingo leklinikhi.
I-RAEB iphathwa njani?
Uphuhliso lwe-RAEB luhluke kwiimeko ezahlukileyo. Ubudala kunye nempilo yonke yomntu inokubangela ukuba kwenziwe izigqibo zonyango. Iziguli ezine-RAEB kufuneka zifumane ukuhlaziywa kwezigonyo zabo, kwaye ababhemayo ne-RAEB bakhuthazwa ukuba bayeke ukutshaya. Iimpawu zokuthi i-RAEB ingaqhubela phambili iquka ukusulelwa rhoqo, ukuphaphaza ngokweqhelekileyo, ukunyunyuzwa, kunye nesidingo sokumpontshelwa igazi rhoqo.
Akunabo bonke abagulane abane-MDS abafuna unyango olukhawulezileyo, kodwa izigulane ezinomlinganiselo ophantsi wezilwanyana (i-anemia, thrombocytopenia, i- neutropenia kunye nezifo eziphindaphindiweyo), kwaye oku kubandakanya izigulane ezininzi ezinezikhupha eziphezulu okanye eziphezulu kakhulu (kuquka i-RAEB-2, emele ibanga le-MDS kunye nesigxina esiphambeneyo).
Ukusebenzisa izikhokelo ze-National Comprehensive Cancer Network (NCCN) zibandakanya impilo kunye nesebenza ngokubanzi, inkqubo ye-International Prognostic Scoring System (IPSS) kunye ne-IPSS (IPSS-R) iindidi zeengozi ze-MDS kunye nezinye iziganeko zesifo ukukunceda ukukhokela izigqibo zolawulo. Akukho "ubungakanani obufanayo buhambisana nayo yonke" indlela yokonyango kubantu abane-RAEB, nangona kunjalo.
Kukho iindidi zentathu zonyango : ukunakekelwa okuxhasayo, unyango oluphantsi, kunye neendlela eziphambili zokwelapha. Ezi zonyango zichazwe ngezantsi:
- Ukunyamekela okubandakanya i-antibiotiki yezifo kunye neeseli ezibomvu kunye ne-platelet transfusion kunye nezibalo eziphantsi.
- Izindlela zokwelapha ezincinci ziquka izinto zokukhula kweeseli zegazi, ezinye i-agents ezifana ne-azacitidine kunye ne-decitabine, unyango lwe-immunosuppressive, kunye ne-chemotherapy. Ezi zonyango ziyakwazi ukuhanjiswa kwisigulane kwaye ziyakwazi ukuphucula iimpawu kunye nomgangatho wobomi, kodwa abayiphilisi imeko.
- Iimpawu zonyango eziphezulu zibandakanya intsebenziswano enkulu ye-chemo kunye ne-allogeneic bone Ezi zonyango zidinga ukulaliswa esibhedlele kunye nokubeka umngcipheko wemiphumo yecala lokusongela ubomi, kodwa nazo ziyakwazi ukuphucula igazi zibala ngokukhawuleza kunokuba unyango olungaphantsi kwaye lunokutshintsha indlela leyo imeko iya kuqhubeka ngayo. Kuphela abantu abathile abaviwa ngabagulisa kakhulu.
Izilingo zezonyango zikhethwa ngabanye izigulane. Kungekude kakhulu, enyanisweni, kwakukho uvavanyo lweklinikhi olubonisa inzuzo nge-decitabine, xa kuthelekiswa nokunyamekelwa okusemandleni, kwizigulane ezindala ezine-anemia ezineziphumo ezingaphezulu kweenguqu (RAEBt).
ILizwi
Ukuba unikwe i-RAEB-1, i-RAEB-2, okanye unomnye uhlobo lwe-MDS oluya kuthathwa njengengozi enkulu, xela iqela lakho lezempilo malunga neenketho zakho.
Izigulane ezine-MDS ephezulu, i-azacitidine (i-5-AZA, iVidaza) kunye ne-decitabine (iDacogen) ngamachiza amabini avunywe yi-FDA ye-MDS ukuba iqela elijongene nokunyamekela lingaqwalasela. Ezi zi yobisi zibizwa ngokuba yi-hypomethylating agents.
Amaqela amanyathelo okubambisana abonise ukuba, kwi-MDS ephezulu, i-HSCT (i-bone marrow transplant) okanye unyango kunye ne-hypomethylating agents kufuneka liqaliswe ngokukhawuleza. I-HSCT ye-Allogeneic (ithambo lomnkantsha wokutshintshwa kumniki-mali) yindlela kuphela yokunakekelwa kwe-MDS, kodwa, ngelanga, yindlela yokwenene yezigulane ezimbalwa kakhulu, ngenxa yecala elidala elichaphazelekayo yi-MDS, ngokubambisana nempilo engapheliyo imiqathango kunye nezinye iziganeko ezithile zesigulane.
> Imithombo:
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> IGerming U, Strupp C, Kuendgen A, et al. I-anemia yokuchithwa ngokugqithiseleyo (RAEB): ukuhlaziywa kwe-reclassification ngokwemiqathango ye-WHO. Br J Haematol. 2006; 132 (2): 162-7.
> Holkova B, Supko JG, uAmes MM, et al. Isigaba esilingo se-vorinostat kunye ne-alvocidib kwizigulane eziphinda ziphinde ziphinde zitshatyalaliswe, zikhanyele okanye zihlasele i-leukemia enkulu, okanye i-anemia ye-refractory ene-blast-excess-2. Can Cancer Res. 2013; 19 (7): 1873-1883.
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> Pleyer L, Burgstaller S, Stauder R, et al. Umgca we-Azacitidine ngaphambili kwii-339 izigulane ezine-myelodysplastic syndromes kunye ne-leukemia enesifo esilumkileyo: ukuthelekiswa kwezigaba zeFrench-American-British kunye neMpilo Yehlabathi. J Hematol Oncol. 2016; 9: 39.