I-leukemia ayiyona isifo esisodwa, kodwa iningi. Ososayensi baqala ukuqonda ukuthi ngisho noma uhlobo olulodwa olulodwa lwe-leukemia lunezinhlayiyana ezihlukene ngezindlela ezibalulekile.
Izinhlobo ezine eziyinhloko ze-leukemia zincike ekutheni ziyi-leukemia enesifo esiphuthumayo noma engapheliyo, futhi lezi zigaba eziyinhloko zilandelayo:
- I-myeloid engapheli (i-myelogenous) i-leukemia (i-CML)
- I-lymphocytic (noma i-lymphoblastic) ye-leukemia (YONKE)
- I-lymphocytic leukemia (CLL) engapheli
Mayelana ne-AML
I-leukemia evamile ye-myelogenous ingumdlavuza we- bone lomnkantsha-ama -spiny insides amathambo, lapho amangqamuzana egazi enza khona-futhi futhi umdlavuza wegazi.
I-AML ibhekwa njenge-"acute" leukemia ngoba iqhubekela phambili ngokushesha. Ingxenye ye-myelogenous yegama ivela kumaseli-myeloid-iqoqo lamaseli ngokuvamile evame ukuba yizinhlobonhlobo zamaseli egazi avuthiwe, njengezibomvu zegazi elibomvu, amaseli egazi amhlophe namaplatelet.
I-AML inezixazululo eziningi: I-leukemia evamile ye-myelogenous iyaziwa nangokuthi i-lemonemic acute myeloid, i-myeloblastic leukemia, i-leukemia ejulile ye-granulocytic kanye ne-acute leyemia ye-nonlymphocytic.
I-AML ingathinta abantu bazo zonke ubudala. Umkhankaso we-World Health Organization ka-2012 we-GLOBOCAN uphakamise ukuthi abantu abangaba ngu-352,000 emhlabeni wonke babe ne-AML, futhi lesi sifo siba seminyaka eminingi kakhulu.
Izimpawu nezimpawu ze-AML zihlanganisa:
- Fever
- Ubuhlungu be-Bone
- I-Lethargy nokukhathala
- Ukuphelelwa umoya
- Isikhumba esiphezulu
- Ukutheleleka okuvamile
- Ukulimala kalula
- Ukusuka okungavamile, okufana nokuphuza okwephuza njalo nokuphuma ezinsini
Ama-subtypes
Ukuhlelwa kwe-AML okusekelwe ekubukeni ezincane zamangqamuzana omdlavuza, noma i-morphology, kwandiswa yizinto ezintsha ezitholakalayo mayelana nezinguquko zofuzo noma izinguquko ezithintekayo ezifweni ezihlukahlukene zalesi sifo.
Abacwaningi e-Wellcome Trust Sanger Institute nabahlanganyeli abasanda kufaka esiteshini solwazi, ukubika ngokushintshashintsha kwezakhi zofuzo okusiza ukujula ukuqonda kwe-AML-okuqhubekayo kushintshe umqondo we-AML kusuka kwesifo esisodwa kuya kokunye okungenani izinhlobo ezingu-11 ezihlukahlukene zofuzo ukuhlukumezeka , ngokungafani okungasiza ekuchazeni izikhathi zokuphila ezihlukahlukene phakathi kweziguli ezincane ezine-AML.
Abacwaningi bashicilela isifundo sabo kwi-genetics ye-AML kumagazini ka-June 2016 we-"New England Journal of Medicine," futhi ochwepheshe bakholelwa ukuthi lezi zithole zingathuthukisa izilingo zokwelashwa futhi zithinte indlela iziguli ezine-AML ezifunyanwa futhi ziphathwe ngayo esikhathini esizayo.
Isifundo se-NEJM
Abacwaningi bacwaninga iziguli ezingu-1,540 ezine-AML ababhalisile ekuvivinyweni kwemitholampilo. Bahlaziya izakhi zofuzo ezingaphezu kuka-100 ezaziwa ukuthi zibangelwa i-leukemia ngenhloso yokukhomba "izihloko zofuzo" ngemuva kokuthuthukiswa kwesifo.
Bathole ukuthi iziguli ezine-AML zingahlukana zibe amaqembu amakhulu ama-11, ngayinye enezinhlobonhlobo zezinguquko zofuzo kanye nezici nezici ezihlukile. Ngokusho kocwaningo, iziguli eziningi zinezinhlanganisela eziyingqayizivele zokuguqula izakhi zofuzo ezihambisa i-leukemia, ezingasiza ukuchaza ukuthi kungani i-AML ibonisa ukwehluka okunjalo kumanani okusinda.
Imiphumela
Ukwazi ukukhiqizwa kwesifo se-leukemia yesiguli kungathuthukisa ikhono lokubikezela ukuthi ngabe izindlela zokwelashwa zamanje zizophumelela yini. Ulwazi lwalolu hlobo lungasetshenziswa ukuklama ukuhlolwa okusha kwemitholampilo ukuthuthukisa ukwelashwa okungcono kakhulu kwe-AML subtype ngayinye; futhi ekugcineni, ukuhlolwa okubanzi kwezakhi zofuzo ze-AML ekuxilongweni kungaba isimiso esiningi.
Kuhlelo lukaHulumeni lwe-World Health Organization (WHO) lwe-2008, ososayensi sebevele baqala ukuhlukanisa ama-AML abantu abadala "emaqenjini ama-molecule" ahlukile, kuhlanganise nezinguquko ezithile zofuzo noma ukulimala kwama-chromosomes okuchazwe kanje: t (15; 17), t (8; 21) ), inv (16) -t (16; 16), t (6; 9), inv (3) -t (3; 3), izakhi zofuzo ze-MLL, kanye nesikhashana, ukuguqulwa kwe-CEBPA noma i-NPM1.
Kodwa-ke, njengoba kuboniswe ekutadisheni kwe-NEJM yakamuva, izigaba ze-WHO ze-WHO azisebenzi kahle inani elikhulu lama-AML amacala. Esicwaningweni, iziguli ezingu-736 ezine-AML, noma amaphesenti angu-48 zazo, zazingeke zihlukaniswe ngokusekelwe emaqenjini e-WHO, nakuba amaphesenti angama-96 alawo mkhuba enza kanjalo, okuthiwa izinguquko zomshayeli-izinguquko zofuzo ukulimala.
Ukutholakala kwamagciwane amaningi omusha we-leukemia, ukuguquguquka okuningi komshayeli ngesiguli, kanye namathrafikhi aguquguqukayo awenza abaphenyi babuyekeze kabusha ukuhlukaniswa kwe-AML kusukela ekuqaleni.
Ukuhlaziywa kwe-AML nokuHlelwa, ngokusekelwe ku-Genetic Mutations
Ngakho-ke, abacwaningi babuyela ebhodini lokudweba ukuze bazame ukuthuthukisa uhlelo olusha lokuhlukanisa i-AML esebenzisa ulwazi olusakhulayo.
Isigaba esamukelekayo kakhulu kanye nezinhlelo zokubikezela i-AML zisebenzisa isigaba se-WHO - kuhlanganise nezinto okuthiwa i-cytogenetic lesions-isibonelo t (15; 17) -nonke ne-NPM1, FLT3ITD, ne-CEBP, njengoba kubhalwe ngenhla.
Ngenxa yocwaningo olusha, abalobi bancoma ukuthi, ngesikhathi esifushane, i-TP53, i-SRSF2, i-ASXL1, i-DNMT3A, ne-IDH2 kufanele icatshangelwe ukuthi ifakwe emibhalweni yokubikezela ngoba ijwayelekile futhi ithonya elinamandla emiphumeleni yomtholampilo.
Ngokwezigaba ze-AML, ukuhlolwa kwe-"splicing-factor genees" i-RUNX1, i-ASXL1, ne-MLLPTD ekuxilongweni kuyokwazi ukubona iziguli "eqenjini le-chromatin-spliceosome." Leli yiqembu lesibili elikhulu kunazo zonke iziguli ze-AML esifundweni, futhi ngokungafani Amakilasi we-AML, ayikho isifo esisodwa sezakhi zofuzo esichaza leli qembu.
Ukusebenzisa le nqubo ehlongozwayo, iziguli ezingu-1,236 ezinezinguquko ezingu-1,540 zingafakwa esigabeni esisodwa, kanti iziguli ezingu-56 zihlangene nezigaba ezimbili noma ngaphezulu. Isiguli seziguli ezingu-166 ezinezinguquko zomshayeli zahlala zingenakulinganiswa.
Ingemuva yezinhlelo zokuHlelwa ezikhona
I-AML ayifakiwe njengezinye eziningi kansela. Umbono womuntu onama-AML uncike kunolunye ulwazi, njenge-subtype njengoba kunqunywe ukuhlolwa kwelebhu, kanye neminyaka yesiguli, neminye imiphumela yokuhlolwa kwelebhu.
I-AML subtypes ingahlobana nesimo sengqondo somuntu ngamunye kanye nokwelashwa okungcono kakhulu. Isibonelo, i-acute promyelocytic leukemia (APL) i-subtype ivame ukuphathwa ngokusebenzisa izidakamizwa ezihlukile kulabo asetshenziselwa ezinye izansi ze-AML.
Izinhlelo ezimbili eziyinhloko ezisetshenziselwe ukuhlukanisa i-AML zibe yi-subtypes yizigaba zeFrench-American-British (FAB) kanye nesigaba esisha se-World Health Organization (WHO).
Isigaba samaFrench-American-British (FAB) se-AML
Ngawo-1970, iqembu lamaFrench, eMelika, naseBrithani ochwepheshe be-leukemia bahlukanisa i-AML yaba yi-subtypes, i-M0 kuya kwe-M7, ngokususelwa kuhlobo lwesitembu lapho i-leukemia iqala khona nokuthi amangqamuzana avuthiwe kangakanani. Lokhu kwakusekelwe ngokuyinhloko ngokuthi amangqamuzana e-leukemia ayebheke kanjani ngaphansi kwe-microscope ngemuva kokugqoka izinqubo.
Igama le-FAB subtype
M0 Undifferentiated acute leyemia ye-myeloblastic
M1 I-leukemia elula ye-myeloblastic ene-maturation encane
I-M2 i-leukemia e-myeloblastic enamandla ne-maturation
I-M3 Acute promyelocytic leukemia (APL)
I-M4 i-myelomonocytic i-leukemia
I-M4 eos I-myelomonocytic i-leukemia elula ne-eosinophilia
I-M5 i-leukemia ye-monocytic elula
I-M6 i-leyemia ye-erythroid elula
I-M7 I-leukemia ye-megakaryoblastic elula
I-Subtypes M0 ngokusebenzisa i-M5 yonke iqala ngamafomu amancane amangqamuzana egazi elimhlophe. I-M6 AML iqala ngamafomu alula kakhulu amangqamuzana egazi obomvu, kuyilapho i-M7 AML iqala emafomini amasha angamafomu enza amaplatelets.
I-World Health Organization (WHO) isigaba se-AML
Uhlelo lwesigaba se-FAB luwusizo kanti lusasetshenziselwa ukuqoqa i-AML ibe yi-subtypes, kodwa ulwazi oluhambelana nokubikezela kanye nokubuka izinhlobo ezahlukene ze-AML, kanti enye yalezi zintuthuko kuboniswe ohlelweni luka-2008 lwe-World Health Organization (WHO).
Uhlelo lwe-WHO luhlukanisa ama-AML ngamaqembu amaningana:
I-AML enezinhlobo ezithile ezingavamile
- I-AML ene-translocation phakathi kwama-chromosomes 8 no-21
- I-AML ene-translocation noma i-inversion ku-chromosome 16
- I-AML ene-translocation phakathi kwama-chromosomes 9 no-11
- I-APL (M3) ene-translocation phakathi kwama-chromosomes 15 no-17
- I-AML ene-translocation phakathi kwama-chromosomes 6 no-9
- I-AML ene-translocation noma i-inversion ku-chromosome 3
- I-AML (megakaryoblastic) ne-translocation phakathi kwama-chromosomes 1 no-22
I-AML nezinguquko ezihlobene nemyelodysplasia
I-AML ihlobene ne-chemotherapy yangaphambilini noma imisebe
I-AML ayihlongozwanga ngenye indlela (i-AML engangeni kwelinye lamaqembu angenhla futhi ngenxa yalokho ihlukaniswe ngokufana nalokho okwenziwe ohlelweni lwe-FAB):
- I-AML enokuhlukanisa okuncane (M0)
- I-AML ngaphandle kokuvuthwa (M1)
- I-AML nokuvuthwa (M2)
- I-myelomonocytic leukemia elula (M4)
- I-leukemia ye-monocytic elula (M5)
- I-leukemia ye-erythroid enamandla (M6)
- I-leukemia ye-megakaryoblastic elula (M7)
- I-leukemia ye-basophilic elula
- I-panmyelosis enamandla ne-fibrosis
I-myeloid sarcoma (eyaziwa ngokuthi i-granulocytic sarcoma noma i-chloroma)
Ukwanda kweMyeloid okuhlobene ne-Down syndrome
I-leukemias e-undifferentiated ne-biphenotypic
Lezi yi-leukemias ezinezici ezimbili ze-lymphocytic kanye nemyeloyelo. Ngezinye izikhathi kuthiwa yi-ALL ngezimpawu ze-myeloid, i-AML enezimpawu ze-lymphoid, noma ama-leukemi ase-acute.
Ngaphezulu kwalezi zigaba ze-WHO zashintshwa kusukela ku-American Cancer Society.
Imithombo:
Papaemmanuil E, Gerstung M, et al. Ukuhlukaniswa kwe-genomic nokubikezela ku-leukemia ephawulekayo ye-myeloid. N Engl J Med . 2016; 374 (23): 2209-21.
I-Wellcome Trust Sanger Institute. I-myeloid leukemia enamandla kakhulu okungenani izifo ezingu-11 ezahlukeneyo. Kufinyelelwe ngo-June 2016.
I-American Cancer Society. Kanjani i-leukemia ephawulekayo ye-myeloid? Kufinyelelwe ngo-June 2016.