Kuratidzira anemia nekumhanya kwakanyanya, kana RAEB, inoreva chirwere chemasero ekugadzira ropa. RAEB ndiyo imwe yemarudzi manomwe ematambudziko akadaro, kana myelodysplastic syndromes (MDS), inozivikanwa nehutano hweWorld Health Organization (WHO), inoparadzanisa pakati pemapoka maviri e RAEB: RAEB-1 ne RAEB-2.
Zvose zviri zviviri zvinowanzoenderera mberi zvinorema zvinotyisa: chikamu chakabudiswa chekupona nguva (ikozvino dated) chinobva pamwedzi 9-16.
RAEB inobatanidzwawo nehuwandu hwekuwedzera kune hutachiwana hwemyeloid leukemia -kenza yemasero ekugadzira ropa emufupa .
Kunzwisisa RAEB, Rudzi rweMDC
Myelodysplastic syndrome, kana MDS, inoreva mhuri yezvirwere zvisingaoneki zveropa umo marora emapfupa haabereki zvakakwana masero matsvuku eropa, masero eropa kana maplatelet. RAEB inoratidzirwa rudzi rwe MDS, uye zvinosuruvarisa, iyo inowanikwa kune ngozi ye MDS.
Kungofanana nedzimwe mhando dzeMDC, RAEB inowanzotambudza vanhu vanopfuura makore makumi mashanu, asi zvinogona kuitika kune vanhu vaduku, pamwe chete, uye chinokonzera ikozvino hachizivikanwe.
Apo munhu ane fomu ye MDS yakafanana ne RAEB, marora emapfupa angaita kuti vakawanda vasati vashandurwa, kana kuti vasati vasvike, masero anowanzova nemaitiro asinganzwisisiki, maitiro kana kuonekwa, achienzaniswa nehutano. Aya mavambo, vana vechidiki, mazana emasero eropa anoshevedzwa kuputika masero -izwi rinoshandiswa kakawanda mukukurukurirana kweukama.
Zvechokwadi, nhasi masayendisiti akawanda anoona MDS sechimiro cheropa uye nekenza yemuropa.
Kusiyana kwezvikwata zvehurongwa zvakashandiswa nekuda kwezvinetso izvi. IWIV classification system inoedza kuronga mhando dzeMDC, nekucherechedza kubvuma kwechirwere chakapiwa. WHO iye zvino anoziva marudzi 7 eMDC, uye pamwe RAEB-1 uye RAEB-2 nhoroondo ye 35-40 muzana yematambudziko ose eMDC.
- Refractory cytopenia neunilineage dysplasia (RCUD)
- Kuratidzira anemia neine ringed sideroblasts (RARS)
- Refractory cytopenia ne multilineage dysplasia (RCMD)
- Kugadziriswa anemia neupfu hwakanyanya-1 (RAEB-1)
- Kugadziriswa anemia neupfu hwakanyanya-2 (RAEB-2)
- Myelodysplastic syndrome, unclassified (MDS-U)
- Myelodysplastic syndrome inosanganiswa neyega (5q)
Izvo pamusoro pemazita zvinowanzotaura nezvekuti ropa neropa remasero rinowoneka sei, kana richitaridzwa pasi pe microscope. Zita rekupedzisira mune runyora rwuri pamusoro, zvisinei, rinotsanangurwa neimwe shanduko, kana kuchinja kwechromosome, mumararamiro emagetsi eropa-kuumba mapfupa emasese emasero.
Mune mhaka ye RAEB (zvose zviripo), zita rine zvikamu zviviri: kukanganisa anemia; uye kuwedzera kwakanyanya. Anemia, kazhinji, kusakwana kwemasero matsvuku eropa. Kuratidzira anemia kunoreva kuti chirwere chekushaya ropa chinenge chisina kukonzerwa nechimwe chezvinhu zvinowanzozivikanwa zvinokonzerwa nekushaya ropa uye kuti anemia inowanzogadziriswa neropa. Kana munhu aine chirwere chekushaya ropa uye kuongororwa kunoratidza huwandu hwakawanda hwekushanda kwemajeri kusina kuitika, iyo inopesana neanemia nehuwandu hunoputika.
Zvinokwanisika kuti munhu ane RAEB ave nezvikamu zvakaderera pane mamwe masero akaumbwa nemusepa wepafu, zvakare.
Vanhu vane RAEB vanogona kunge vane mafungiro anemia (masero matsvuku eropa), refractory neutropenia (pasi neutrophils), refractory thrombocytopenia (pasiplate shoma), kana kusanganiswa kwevatatu.
RAEB Ndiyo yeHigh-Risk Form ye MDS
Kune varwere vanowanikwa vane MDS, zvakakosha kuziva chiyero chematambudziko. Dzimwe mhando dzeMDC dzakaderera-panjodzi, vamwe vari pakati-pangozi, uye vamwe vane ngozi yakawanda. Zvose RAEB neRCMD dzinofungidzirwa zvakanyanya kune ngozi dzeMDC. Kunyange zvakadaro, havasi varwere vose vane RAEB vane kufanana kwakafanana. Zvimwe zvinhu zvinoitika, zvakadai sekukwegura, hutano hwehutano, hutano hwechirwere, uye genetics yeiyo inobatanidza masero ekugadzira mapfupa.
Kuziva
Apo MDS inofungidzirwa, mapfupa emapfupa anopisa uye aspirate anofanira kuitwa. Izvi zvinosanganisira kuwana zviyero zvepfupa remapfupa nekuvatumira ku laboratory kuti vaongorore uye kududzira.
Kuongorora kunoitwa kunobva pakuita kunoita masero pasi pe microscope, kuti vanosvibiswa sei nemasere akasiyana-siyana emadheyesheni uye zviratidzo zvinosanganisira kushandiswa kwezvirwere semagetsi, uye, pane imwe yakanyanya mazana eDMS, chimwe chinhu chinonzi flow cytometry . Kuyerera cytometry inzira inobvumira masero ane humwe hunhu hunozivikanwa uye hunorongwa kubva kune vakawanda vanoita masero mune imwe samuenzaniso inopiwa.
Aina
Zvose zviri zviviri (1 ne2) ye RAEB inobatanidzwa nehutano hwekufambira mberi kune myeloid leukemia (AML) yakaoma. Uyezve, murwere ane ngozi yakanyanya MDS yakadai seR RAEB inogona kukundwa nekushaya simba kwemapfupa, pasina kufambira mberi kwa AML, uye naizvozvo mamiriro acho ega anowanzoisa upenyu pangozi, pasina kufambira mberi kweukama.
RAEB-Related Terminology
RAEB kusarudzwa kunobva pakunzwisisa kwemashoko akawanda:
- Bhoora marrow blast kuverenga : Muenzaniso wemapfupa ako anowanikwa, uye nhamba yevasina kujairika, yakaremara masero masero inoyera.
- Purogiramu ropa rinopera kuverengwa: Muenzaniso weropa rako kubva mune mutsva unoswededzwa uchishandisa nheyo, uye nhamba yevasina kujairika, isina kuchena masero masero anoyerwa.
- Auer rds : Ichi ndicho chiremba vanachiremba vachatarisa pavanoona kuputika kwako pasi pe microscope. Kunyange zvazvo vakanzi Auer "tsvimbo," ivo chaizvoizvo vanouya nenzira dzakasiyana-siyana uye hukuru. Iyo iduku-duku kudarika nucleus, uye inowanikwa mukati me cytoplasm. Kazhinji ivo vane mashizha akaumbwa nemagumo akareruka, asi anogona kuva akafanana nemasikisi, akaumbwa nemadhimoni, kana akareba uye akawanda akareba.
Kubva pahupo kana kusasipo kwezviwanikwa zvataurwa pamusoro apa, munhu akatsunga kuva RAEB-1 kana RAEB-2 sezvinotevera:
Varwere vanoongororwa vane RAEB-1 kana vane (1) mapfupa emapfupa anoputika ari pakati pe5 ne9 muzana yemasero mazana mashanu anoverengerwa kana (2) mhirizhonga inoputika pakati pe2 ne4 muzana yemasero mazana maviri akaverengwa, uye (3) asipo paAraer tambo. Kuvapo kwechirevo chekutanga 1 kana 2 plus 3 chinoronga mhosva ye MDS se RAEB-1.
Mikana ye RAEB-1 inoshanduka muhutachiona hwemyeloid yeukemia inofungidzirwa pane anenge 25 muzana.
Varwere vanoongororwa vane RAEB-2 kana vaine (1) mapfupa emapfupa ari kuwedzera pakati pe10 ne19 muzana yemasero mazana mashanu akaverengwa kana (2) kubuda kwepakati kuverengwa pakati pe5 ne19 muzana yemasero mazana maviri akaverengwa, kana (3) Auer rods inoonekwa. Kuvapo kwevamwe nzira, 1 kana 2 kana 3 kunotarisa dambudziko re MDS se RAEB-2.
Inofungidzirwa kuti mikana ye RAEB-2 inoshanduka muhutu hwemyeloid yeukemia inogona kunge yakareba kusvika 33 kusvika ku50 muzana.
Chii RAEB-T?
Iwe unogona kusangana nemutsara unoti "kukanganisa chirwere chekushaya ropa nemafuta akawandisa mukushandurwa," kana RAEB-T. Izwi iri rave rakasiyiwa rakasara mune ikozvino WHO-classification ye myelodysplastic syndromes.
Vakawanda varwere vakanga vambova vechikwata zvino vava kutaridzika sevanenge vaine acute myeloid leukemia. Munharaunda yakasiyana-siyana, French-American-British (iyo FAB-classification), varwere vakanga vagoverwa kuRIREB-T kana vaive (1) mapfupa emapfupa anoverengwa pakati pe20 ne30, (2) a peripheral blast inowanikwa zvinenge 5 muzana, kana (3), Auer tsvimbo dzinoonekwa, pasinei nehuwandu huwandu.
Izvo zvinoramba zvichiine kupesana pamusoro pekukosha kwekuisa mahara RAEB-T semunyika yeFAB system, zvakasiyana ne "AML-20-30," sezviri mune WHO. Mamiriyoni makuru emakiriniki emitambo mumakore achangopfuura akashandisa izwi rokuti RAEB-T, pasinei nekushandurwa kweH WHO classification system. Chinokosha chevatachiona nevatapi vehutano vanoita sekuti zvinogona kukosha kuziva kuti kune izwi rinoputirana, kuitira kuti urege kurasikirwa nemukana wekunyoresa kuchipatara chirongwa.
RAEB Inotarisirwa Sei?
Mushonga we RAEB wakasiyana nemamiriro ezvinhu akasiyana. Zera uye hutano hwehutano hwemunhu hunoita kuti zviite zvakadai sarudzo. Varwere vane RAEB vanofanira kugamuchira zvinyorwa pamishonga avo, uye vanosvuta ne RAEB vanokurudzirwa kuti varege kusvuta. Zviratidzo izvo RAEB inogona kufambira mberi inosanganisira zvirwere zvinowanzoitika, kubuda kwemazuva ose, kuputika, uye kudikanwa kwokuwedzerwa ropa kakawanda.
Havasi vese varwere vane MDS vanoda kukurumidza kurapwa, asi varwere vane zviratidzo zvakaderera zvakanyanya (anemia, thrombocytopenia, neutropenia nehutachiona husingaperi) vanoita, uye izvi zvinosanganisira varwere vakawanda vane mireho yakakwirira kana yakawanda kwazvo (kusanganisira RAEB-2, iyo inomiririra yakakwirira bhidhiyo reMDC neShoko rakakosha zvikuru).
Kushandisa Mirayiridzo yeNational Comprehensive Cancer Network (NCCN) inosanganisira hutano hwehutano hwemunhu pamwe chete, International Prognostic Scoring System (IPSS) uye yakarongeka zvirongwa zve IPSS (IPSS-R) MDS, nezvimwe zvirwere zvehutano kubatsira kutungamira zvisarudzo zvekuita. Hapana "chiyero chimwe chete chinokodzera zvose" nzira yekurapa kune vanhu vane RAEB, zvisinei.
Iko kune maitiro matatu emishonga yekurapa : kuchengetedza kunotsigira, kushandiswa kwemaitiro mashoma, uye kukwirira kwemaitiro ekurapa. Aya marapirwo anotsanangurwa pasi apa:
- Kutarisira kunotsigira kunosanganisira mishonga inorwisa mabhakitiriya kune utachiona uye tsvuku dzemuviri uye platelet transfusions nokuda kwechiratidzo chinoderera.
- Kuderera kwemaitiro ekurapa kunosanganisira ropa masero ekukura, mamwe mazamu akadai sokuzacitidine uye decitabine, immunosuppressive therapy, uye kushandiswa kwema-chemotherapy. Iyi mishonga inogona kuendeswa kune imwe kunze uye inogona kuvandudza zviratidzo uye hutano hwehupenyu, asi hairi kurapa mamiriro acho.
- Kuwedzerwa kwemaitiro ekurapa kunosanganisira zvakasimba kusanganiswa chemo uye allogeneic bone marrow transplant. Izvi zvinoda kurapwa uye kuisa njodzi yehupenyu hunopisa mitezo, asi inogonawo kukwanisa kuwedzerwa ropa inokurumidza kukurumidza kupfuura chirwere chiduku chisingaiti uye inogona kuchinja nzira iyo inowanzoenderera mberi. Vamwe vanhu chete ndivo vanobvumirwa nepamusoro-soro remishonga.
Mishonga yemakiriniki isarudzo kune vamwe varwere. Kwete kwenguva yakareba kare, chaizvoizvo, paiva nechirwere chechipatara chinoratidza kubatsirana ne decitabine, kana ichienzaniswa nekutarisira kwakanyatsotsigira, kune vakwegura varwere vane chirwere chekushaya ropa nehuwandu hwekuputika mukushandurwa (RAEBt).
Shoko Rinobva
Kana wakamboonekwa kuti une RAEB-1, RAEB-2, kana iwe une rumwe rudzi rweMDC runganzi rwuri pangozi, taura neboka rako rehutano nezvehutano hwako.
Kune varwere vane njodzi dzakanyanya MDS, azacitidine (5-AZA, Vidaza) uye decitabine (Dacogen) mapiritsi maviri anobvumidzwa neDDA ye MDS kuti boka rinotarisira kutarisira kwako ringafunga. Iyi mishonga inonzi inonzi hypomethylating agents.
Masangano akawanda anobvumirana akaratidza kuti, kune huwandu hwehutachiwana MDS, allogeneic HSCT (bone marrow transplant) kana kuti kurapa ne hypomethylating agents vanofanira kutanga pakarepo. Allogeneic HSCT (bone marrow transplant kubva kune mumwe mubatsiri) ndiyo chete inogona kugadzirisa nzira ye MDS, asi, zvinosuruvarisa, iyo nzira chaiye yevarwere vashomanana zvikuru, zvichienderana nezera revanhu vakwegura rakagadzirirwa ne MDS, pamwe nehuwandu hwehutano husingaperi mamiriro ezvinhu nezvimwe zvinorwara-zvimwe zvinhu.
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