Dokotala wodwala magazi m'thupi mwawo, kapena RAEB, amatanthauza matenda a maselo opanga magazi. RAEB ndi imodzi mwa mitundu isanu ndi iwiri ya matendawa, kapena myelodysplastic syndromes (MDS), yomwe imadziwika ndi gulu la World Health Organization (WHO), lomwe limasiyanitsa pakati pa magulu awiri a RAEB: RAEB-1 ndi RAEB-2.
Mitundu yonseyi imakhala ndi zovuta zowonongeka: nthawi zochepa zomwe zimafalitsidwa (tsopano zatsimikiziridwa) zimachokera pa miyezi 9-16.
RAEB imayanjananso ndi chiopsezo chowonjezeka cha kupita patsogolo ku myeloid ya matenda a m'magazi -khansa ya maselo opanga magazi a mafupa .
Kumvetsetsa RAEB, mtundu wa MDS
Myelodysplastic syndrome, kapena MDS, imatanthawuza ku banja la matenda omwe sali ochepa m'magazi omwe mafupa sakubereka maselo ofiira a magazi oyenera, maselo oyera kapena mapulogalamu. RAEB ndi mtundu wamba wa MDS, ndipo mwatsoka, ndiwo mawonekedwe apamwamba a MDS.
Mofanana ndi mitundu ina ya MDS, RAEB imakhudza anthu omwe ali ndi zaka zopitirira 50, komabe zingatheke kwa achinyamata, komanso chifukwa chake sichidziwika.
Pamene munthu ali ndi mawonekedwe a MDS monga RAEB, mafupa angapangitse ambiri omwe sakhala amphamvu kwambiri, kapena maselo, omwe nthawi zambiri amakhala ndi mawonekedwe osamvetseka, kukula kapena maonekedwe, poyerekeza ndi athanzi. Maseŵerawa, oyambirira, maselo a magazi amatchedwa maselo a maselo -mawu omwe amagwiritsidwa ntchito nthawi zambiri pokambirana za khansa ya m'magazi.
Inde, lero asayansi ambiri amaona MDS ngati mtundu wa magazi ndi khansa ya m'mafupa.
Machitidwe osiyanasiyana osiyanitsira akhala akugwiritsidwa ntchito pa matendawa. Ndondomeko ya bungwe la WHO ikuyesetsa kuthetsa mitundu ya MDS, mosamala za chidziwitso cha matenda operekedwa. WHO tsopano akuzindikira mitundu 7 ya MDS, ndipo pamodzi RAEB-1 ndi RAEB-2 nkhani pafupifupi 35-40 peresenti ya milandu yonse ya MDS.
- Refractory cytopenia ndi unilineage dysplasia (RCUD)
- Nthenda yotaya magazi yowonongeka ndi mitsempha yozungulira (RARS)
- Chotsitsimutsa cytopenia ndi multilineage dysplasia (RCMD)
- Dokotala wodwala matenda ochepetsa magazi m'thupi ndi mafupa owonjezera-1 (RAEB-1)
- RAEM-2 (RAEB-2)
- Myelodysplastic syndrome, unclassified (MDS-U)
- Myelodysplastic syndrome yogwirizana ndi isol del (5q)
Maina apamwambawa nthawi zambiri amatanthawuza momwe magazi ndi mafupa a mafupa amawonekera, akamayang'aniridwa ndi microscope. Dzina lomaliza mu mndandanda wa pamwambawu, komabe, limatanthauzidwa ndi kusintha kwina, kapena kusintha kwa chromosome, mu ma genealo a maselo opanga magazi a mafupa.
Pankhani ya RAEB (mitundu yonse), dzinali liri ndi magawo awiri: kukana magazi m'thupi; ndi kuphulika kochuluka. Matenda a magazi, ambiri, ndi kusowa maselo ofiira a magazi okwanira. Kutaya magazi m'thupi kumatanthawuza kuti kuchepa kwa magazi si chifukwa chodziwika bwino chomwe chimayambitsa matenda obwera chifukwa cha kuchepa kwa magazi komanso kuti kuchepa kwa magazi kumangokonzedwanso ndi kuikidwa magazi. Pamene munthu ali ndi matenda ochepetsa magazi m'thupi ndi mayeso amasonyeza kuti chiwerengero chachikulu cha maselo omwe amamwalira sichizolowezi, ndizosawonongera magazi m'thupi mwambiri.
N'zotheka kuti munthu yemwe ali ndi RAEB akhale ndi chiwerengero chochepa m'maselo ena opangidwa ndi fupa la mafupa.
Anthu omwe ali ndi RAEB akhoza kukhala ndi magazi ochepetsa magazi a m'magazi, maselo otsika kwambiri a m'magazi (otsika kwambiri a neutrophils), opractory thrombocytopenia (mapiritsi otsika), kapena atatu.
RAEB ndi mawonekedwe apamwamba a MDS
Kwa odwala omwe ali ndi MDS, ndikofunika kudziwa mlingo wa chiopsezo. Mitundu ina ya MDS ili pangozi yochepa, ena amaopseza pakati, ndi ena omwe ali pangozi yaikulu. RAEB ndi RCMD zimaonedwa kuti ndizoopsa za MDS. Komabe, si odwala onse omwe ali ndi RAEB omwe amavomerezanso. Zinthu zina zimagwira ntchito, monga msinkhu, thanzi labwino, zizindikiro za matenda, ndi ma genetic a maselo opanga mafupa.
Kudziwa
Ngati MDS ikukayikira, fupa la fupa ndi aspirate liyenera kuchitidwa. Izi zimaphatikizapo kupeza zitsanzo za mafupa ndi kuwatumiza ku labotori kuti afufuze ndi kutanthauzira.
Matendawa amapangidwa malinga ndi momwe maselo amawonekera pansi pa microscope, momwe amawonongera ndi mitundu yosiyanasiyana ya utoto ndi zizindikiro zomwe zimaphatikizapo kugwiritsira ntchito ma antibodies ngati tags, ndipo, poyang'aniridwa ndi magawo apamwamba a MDS, wotchedwa cytometry yothamanga . Mpweya wa cytometry ndi njira yomwe imalola maselo omwe ali ndi makhalidwe ena kuti adziwe ndikusankhidwa kuchokera kwa anthu ochulukirapo m'maselo omwe apatsidwa.
Mitundu
RAEB (1 ndi 2) onsewa ali ndi chiopsezo chopita patsogolo ku myeloid leukemia (AML). Kuonjezerapo, wodwala amene ali ndi chiopsezo chachikulu chotchedwa MDS monga RAEB akhoza kugonjetsedwa ndi mafupa a mitsempha, popanda kupita kwa AML, choncho matendawa nthawi zambiri amakhala pangozi, popanda kupatsirana kwa khansa ya m'magazi.
Nkhani ya RAEB yokhudzana ndi mauthenga
Kafukufuku wa RAEB amadalira kumvetsetsa mawu angapo:
- Mafupa a mafupa amawerengera : Chitsanzo cha mafupa anu chimapezeka, ndipo chiwerengero cha zachilendo, maselo osakanikirana amadziwika.
- Kuwonongeka kwa magazi kumayambira: Chitsanzo cha magazi anu kuchokera mu mitsempha chimakokedwa pogwiritsa ntchito singano, ndipo chiwerengero cha zachilendo, maselo ophulika omwe amabadwa amayamba kuyeza.
- Ndondomeko zamagulu : Izi ndizo zomwe madokotala adzayang'ana pamene adzawona kuphulika kwanu pansi pa microscope. Ngakhale iwo amatchedwa "Auer" ndodo, "zimabwera mosiyanasiyana ndi maonekedwe osiyanasiyana. Iwo ali ang'onoang'ono kwambiri kuposa chinthu, ndipo amapezeka mkati mwa cytoplasm. Kawirikawiri amakhala ofanana ndi zosalala, koma amatha kukhala ofanana ndi a diamondi, kapena aatali kwambiri.
Malingana ndi kupezeka kapena kupezeka kwa zomwe tafufuzazi, munthu akutsimikiza kukhala RAEB-1 kapena RAEB-2 motere:
Odwala amapezeka ndi RAEB-1 ngati ali ndi (1) mafupa a mafupa amatha pakati pa 5 ndi 9 peresenti ya maselo 500 owerengeka kapena (2) kuphulika kwapakati kumawerengeka pakati pa 2 ndi 4 peresenti ya maselo 200 owerengedwa, ndi (3) opanda ma Auer ndodo. Kukhalapo kwa mtundu umodzi kapena 2 kuphatikizapo 3 kumayambitsa vuto la MDS monga RAEB-1.
Mpata wa RAEB-1 ukutembenukira ku myeloid khansa ya m'magazi imayesedwa pafupifupi 25 peresenti.
Odwala amapezeka ndi RAEB-2 ngati (1) mafupa a mafupa amatha pakati pa 10 ndi 19 peresenti ya maselo 500 owerengedwa kapena (2) kuphulika kwapakati kumawerengeka pakati pa 5 ndi 19 peresenti ya maselo 200 owerengedwa, kapena (3) Ndodo zalaer zimawoneka. Kukhalapo kwa zifukwa zina 1, 2 kapena 3 zimayambitsa vuto la MDS monga RAEB-2.
Akuti mwayi wa RAEB-2 ukutembenukira ku myeloid khansa ya m'magazi ingakhale yaikulu mpaka 33 mpaka 50 peresenti.
Kodi RAEB-T ndi chiyani?
Mungathe kukumana ndi mawu akuti "kuperewera kwa magazi m'thupi mwa kusintha kwakukulu," kapena RAEB-T. Mawu awa athawikidwa kale mu mndandanda wa WHO wa myelodysplastic syndromes.
Odwala ambiri omwe kale anali a mderali tsopano akuwerengedwa kukhala ndi mitsempha ya m'mitsempha yambiri. M'madongosolo osiyanasiyana, a French-American-British (FAB-classification), odwala anali atapatsidwa gawo la RAEB-T ngati (1) mafupa a mafupa amatha pakati pa 20 ndi 30 peresenti, (2) Kuphulika kwa pakhosi kumakhala pafupifupi 5 peresenti, kapena (3), ndodo za Auer zimawoneka, mosasamala kanthu za kuphulika chiwerengero.
Paliponse kutsutsana pankhani ya kuika RAEB-T monga momwe zilili mu FAB, mosiyana ndi "AML-20-30," monga momwe zilili mu WHO. Zaka zingapo zachipatala m'zaka zaposachedwapa zakhala zikugwiritsira ntchito RAEB-T, ngakhale kusintha kwa dongosolo la WHO. Chofunikira kwa odwala ndi ogwira ntchito zaumoyo zikuwoneka kuti ndikofunika kuti adziwe kuti pali mawu ophatikizana, kuti asaphonye mwayi wolembera mayeso.
Kodi RAEB Yatchulidwa Motani?
Chithandizo cha RAEB chimasiyana ndi zosiyana. Ukalamba ndi thanzi lathunthu la munthu lingakhalepo muzisankho zoterezi. Odwala omwe ali ndi RAEB ayenera kulandira zosinthira pa ma katemera awo, ndipo osuta fodya ndi RAEB akulimbikitsidwa kuti asiye kusuta. Zizindikiro zomwe RAEB ikukula zikuphatikizapo matenda opatsirana, kutuluka mwazidzidzidzi, kuvulaza, ndi kufunika kwa kuikidwa magazi nthawi zambiri.
Osati odwala onse omwe ali ndi MDS amafunika chithandizo chamankhwala, koma odwala omwe ali ndi vuto la kuchepa kwa magazi (matenda a kuchepa kwa magazi, thrombocytopenia, neutropenia ndi matenda opatsiranawo) amachita, ndipo izi zimaphatikizapo odwala omwe ali ndi matenda akuluakulu kapena aatali kwambiri (kuphatikizapo RAEB-2, omwe amaimira apamwamba kwambiri kalasi ya MDS ndi matenda ovuta kwambiri).
Gwiritsani ntchito Malangizowo a National Comprehensive Cancer Network (NCCN) kuphatikizapo thanzi labwino la munthu ndi ntchito yake, International Prognostic Scoring System (IPSS) ndi machitidwe omwe apangidwa ndi IPSS (IPSS-R) MDS, ndi zizindikiro zina za matenda kuti athandize kutsogolera zochita. Palibe "kukula kofanana kumagwirizanitsa" njira zonse zothandizira anthu omwe ali ndi RAEB, komabe.
Kawirikawiri pali mitundu itatu ya chithandizo : chithandizo chochirikizira, chithandizo chamankhwala chochepa kwambiri, ndi njira zothandizira kwambiri. Mankhwalawa akufotokozedwa pansipa:
- Thandizo lothandizira limaphatikizapo maantibayotiki a matenda ndi maselo ofiira ndi ma pulogalamu a m'munsi chifukwa cha zizindikiro zochepa.
- Mankhwala ofooketsa kwambiri amaphatikizapo kukula kwa maselo a magazi, othandizira monga azacitidine ndi decitabine, mankhwala osamalitsa thupi, komanso mankhwala otsika kwambiri. Mankhwalawa angaperekedwe pafupipafupi ndipo akhoza kusintha zizindikiro ndi khalidwe la moyo, koma samachiza.
- Mankhwala othandiza kwambiri amaphatikizapo kuphatikiza kwakukulu kokhala ndi chemo ndi feteleza ya mafupa. Mankhwalawa amafuna kulandira chithandizo kuchipatala ndikuika pangozi zoopsa zomwe zimawopsyeza moyo, komabe amatha kupititsa patsogolo magazi mofulumira kusiyana ndi mankhwala ochepa kwambiri ndipo angasinthe momwe chikhalidwecho chikapitilira. Ndi anthu ena okha amene amafunikila kuchipatala.
Zilonda zamakono ndizo mwayi kwa odwala ena. Osati kale kwambiri, panthawiyi, panali mayesero a zachipatala omwe amasonyeza zopindulitsa ndi decitabine, poyerekeza ndi chisamaliro chothandizira kwambiri, kwa okalamba omwe ali ndi matenda ochepetsa magazi m'thupi omwe ali ndi kupweteka kochuluka mu kusintha (RAEBt).
Mawu Ochokera
Ngati mwapezeka kuti muli ndi RAEB-1, RAEB-2, kapena muli ndi mtundu wina wa MDS umene ungawonedwe kuti uli pangozi yaikulu, lankhulani ndi gulu lanu la thanzi labwino za zomwe mungasankhe.
Kwa odwala omwe ali ndi chiopsezo chachikulu chotchedwa MDS, azacitidine (5-AZA, Vidaza) ndi decitabine (Dacogen) ndi mankhwala awiri omwe amavomerezedwa ndi FDA kwa MDS kuti gulu lomwe likuyang'anira likhoza kulingalira. Mankhwalawa ndi otchedwa hypomethylating agents.
Magulu angapo ogwirizanitsa awonetsa kuti, chifukwa cha masoka akuluakulu a MDS, kupatsirana kwa mafupa a mafupa a HSCT) kapena mankhwala opatsirana ndi hypomethylating ayenera kuyambitsidwa mwamsanga. HSCT (kugwiritsira ntchito mafupa a mafupa kuchokera kwa wopereka) ndiyo njira yokhayo yothetsera mavuto a MDS, koma, mwatsoka, ndi njira yabwino yeniyeni ya odwala kwambiri, chifukwa cha okalamba omwe akukhudzidwa ndi MDS, zikhalidwe ndi zifukwa zina zokhudzana ndi wodwala.
> Zotsatira:
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> Holkova B, Supko JG, Ames MM, et al. Gawo lomwe ndikuyesa vorinostat ndi alvocidib kwa odwala omwe abwereranso, amatsutsa kapena amavutika kwambiri ndi khansa ya m'magazi, kapena kupatsirana magazi m'thupi mwachangu. Clin Cancer Res. 2013; 19 (7): 1873-1883.
> Jiang Y, Dunbar A, Gondek LP, et al. Aberrant DNA methylation ndi njira yaikulu mu MDS kupita patsogolo kwa AML. Magazi . 2009; 113 (6): 1315-1325.
> Pleyer L, Burgstaller S, Stauder R, et al. Azacitidine kutsogolo kwa odwala 339 omwe ali ndi myelodysplastic syndromes ndi myeloid yamagazi a m'magazi: kuyerekezera zigawo za French-American-British ndi World Health Organization. J Hematol Oncol. 2016; 9: 39.