I-leukemia ayiyodwa isifo, kodwa ininzi. Iinzululwazi ziqala ukuqonda indlela nokuba olunye uhlobo oluthile lwe-leukemia lunamacandelwana ahlukeneyo ngeendlela ezibalulekileyo.
Iintlobo ezine eziphambili ze-leukemia zisekelwe ekubeni ziyi-acute or chronic, and myeloid okanye i-lucphocytic leukemi, kwaye ezi zigaba eziphambili zilandelayo:
- I-myeloid engapheliyo (okanye i-myelogenous) i-leukemia (CML)
- I-lymphocytic (okanye i-lymphoblastic) ye-leukemia (YONKE)
- I-lymphocytic leukemia (CLL) engapheliyo
Malunga ne-AML
I-leukemia enesifo esilumkileyo ngumhlaza wesifo somnyo-i-spongy insides yamathambo, apho iiseli zegazi zenziwa-kwaye ngumhlaza wegazi.
I-AML ithathwa njenge-"acute" leukemia kuba iyaqhubeka ngokukhawuleza. Inxalenye yesigama segama esivela kumaseli angama-myeloid-iqela leeseli ezivame ukukhula zibe ziintlobo ezahlukeneyo zeeseli zegazi ezivuthiweyo, njengeeseli ezibomvu zegazi, iiseli zegazi ezimhlophe kunye neeplatelet.
I-AML inezidibaniso ezininzi: I-leukemia eqhelekileyo ye-leememia iyaziwa nangokuthi i-leukemia ephawulekayo, i-leukemia ephawulekayo, i-leukemia ye-granulocytic kunye ne-acute lememiacytic leukemia.
I-AML inokuchaphazela abantu bazo yonke iminyaka. Iprojekthi ye-World Health Organisation ka-GLOBOCAN ka-2012 yaphakamisa ukuba abantu abangama-352,000 behlabathi be-AML, kwaye eso sifo siya kuba sisigxina kakhulu njengabantu abadala.
Iimpawu neempawu ze-AML ziquka:
- Fever
- Intlungu yesifo
- I-Lethargy kunye nokukhathala
- Ukuqhawukelwa ngumphefumlo
- Isikhumba
- Ukusulela rhoqo
- Ukululaza
- Ukuphuma kwamagqabi, njengama-nosebleeds rhoqo kunye nokuphuma kwizinsini
Iincinci
Ukuhlelwa kwe-AML esekelwe kumbonakalo omncinci weeseli zesifo somhlaza, okanye i-morphology, kwandiswa ngabantu abatsha malunga nokuguquka kwemfuzo okanye ukuguqulwa kwezinto ezibandakanyekayo kwiindlela ezahlukeneyo zesi sifo.
I-Institute of Wellcome Trust Sanger kunye nabaququzeleli abasanda kudibanisa kwisiseko solwazi, ukunika ingxelo malunga nokuguquka kwemvelo ekunceda ukuma ukuqonda kwe-AML-ngokuqhubekayo ukuguqula ingcamango ye-AML kweso sifo esisodwa kwizinto ezili-11 ezahlukeneyo zofuzo ukuhlambalaza , ngokwahlukileyo okunokukunceda ukuchazela amaxesha athile okuphila phakathi kwezigulane eziselula ezine-AML.
Abaphandi bapapasha isifundo sabo kwi-genetic ye-AML kwiphepha le-Juni 2016 le-"New England Journal of Medicine," kunye neengcali zikholelwa ukuba ezi ziphumo zingaphucula izilingo zeklinikhi kwaye zichaphazela indlela abagulane be-AML bafunyanwa kwaye baphathwa ngayo kwixesha elizayo.
Isifundo se-NEJM
Abaphandi bafunda izigulane ezingama-1,540 ezine-AML ezazibhaliswe kwizilingo zonyango. Bahlalutya ama-geni angaphezu kwe-100 awaziwayo okwenza i-leukemia ngenjongo yokuchonga "iimpawu zofuzo" emva kokuphuhliswa kwesi sifo.
Bafumanise ukuba izigulane ezine-AML zingahlulwa zibe ngamaqela amakhulu angama-11, ngalinye linamaqela ahlukeneyo ekutshintshelweni kwezityalo kunye neempawu ezahlukahlukeneyo kunye nezixhobo. Ngokwalowo uphando, ezininzi izigulane zineentlobo ezikhethekileyo zokutshintshwa kweemfuyo eziqhuba i-leukemia, ezingasinceda ukucacisa ukuba kutheni i-AML ibonisa ukuhluka okunjalo kwimilinganiselo yokusinda.
Impembelelo
Ukukwazi ukuveliswa kwemfuyo ye-leukemia yesigulane kunokuphucula amandla okuqikelela ukuba ngaba unyango lwangoku luya kusebenza. Ulwazi lolu hlobo lungasetyenziselwa ukuseka izilingo ezintsha zeeklinikhi ukuphucula iindlela eziphambili zonyango kwi-subtype nganye ye-AML; kwaye ekugqibeleni, ukuvavanya okubanzi kwemfuyo ye-AML ekuxilongweni kungaba yinto eqhelekileyo.
Inkqubo yesigaba se-World Health Organisation (WHO) ye-2008, izazinzulu sele ziqalise ukuhlela i-AML yabantu abadala kwii-"molecule" ezahlukeneyo, kubandakanywa utshintsho oluthile lomzimba okanye ukulimala kuma-chromosomes echazwe ngolu hlobo lulandelayo: t (15; 17), t (8; 21; ), umbuzo (16) -t (16; 16), t (6; 9), im (3) -t (3; 3), i-MLL i-genetic fusion, kunye nexesha elifutshane, i-CEBPA okanye i-NPM1 iinguqulelo.
Nangona kunjalo, njengoko kuboniswe kwisifundo se-NEJM sakutshanje, ukuhlelwa kwee-molecular ze-WHO akusebenzi kakuhle inani elikhulu le-AML. Kuhlolisiso, izigulane ezingama-736 ezine-AML, okanye iipesenti ezingama-48 zazo, zazingayi kuhlatyathwa ngokusekelwe kwii-molecule ze-WHO, nangona iipesenti ezingama-96 zezigulane zenze, ngokwenene, zithiwa utshintsho lomtshintshi-utshintsho lwezo ukugula.
Ukufunyanwa kwamagciwane amaninzi omhlaza we-leukemia, ukuguqulwa kwamatshintshi amaninzi ngesigulane, kunye neendlela eziguquguqukayo zoguqulelo kwenza abaphandi baphinde baphinde bahlalutye i-AML kusukela ekuqaleni.
UkuVavanywa kwe-AML kunye noHlelo, ngokusekelwe kwiinguqu ze-Genetic Mutations
Ngaloo ndlela, abaphandi babuyela ebhodini lokudweba ukuzama ukuphuhlisa inkqubo entsha yokukhetha i-AML eyenza ukusetyenziswa kolwazi olukhulayo.
Ukwahluleka kwamanye amazwe kunye nokucetyiswa kwe-AML kusetyenziswa udidi lwe-WHO-kuquka okubizwa ngokuba yi-cytogenetic lesions-umzekelo t (15; 17) -nonke ne-NPM1, FLT3ITD, kunye ne-CEBP, njengoko kubhalwe apha ngasentla.
Ngokubhekisele kweso sifundo esitsha, abalobi bancomele ukuba, ngexesha elifutshane, i-TP53, i-SRSF2, i-ASXL1, i-DNMT3A, kunye ne-IDH2 kufuneka iqwalaselwe ngokubandakanywa kwizikhokelo zesigxina kuba ziqhelekileyo kwaye zineempembelelo ezinamandla kwiziphumo zeklinikhi.
Ukuhlelwa kwe-AML, ukuhlolwa kweengxaki ze-genetic factor "RUNX1, ASXL1, kunye ne-MLLPTD ekuxilongweni kuya kubona izigulane" kwiqela le-chromatin-spliceosome. "Le yayisiyigumbi lesibini elikhulu kunazo zonke izigulane ze-AML kwisifundo, kwaye ngokungafani Iiklasi ze-WHO ze-AML, akukho ntsho ye-genetic ye-genetic ichaza eli qela.
Ukusebenzisa le nkqubo ephakanyisiweyo, i-1,236 yezigulane eziyi-1,540 ezinezitshintsho zomqhubi zingafakwa kwicandelo elinye, kwaye izigulane ezingama-56 zidibene neendlela zokubaluleka ezimbini okanye ngaphezulu. Iigulane ezingama-166 ezinezitshintsho zomqhubi zihlala zingekho phantsi.
Imvelaphi yeeNkqubo zoHlelo olukhoyo
I-AML ayifakwe kwisiganeko njengamanye amaninzi emdlavuza. Imbono yomntu onelungelo le-AML incike kunolwazi ngolunye ulwazi, njenge-subtype njengoko kumiselwe iimvavanyo zebhabhi, kunye neminyaka yesigulane, kunye nezinye iziphumo zokuhlolwa kwebhu.
I-AML subtypes inokubambisana nesimo sengqondo somntu ngamnye kunye nonyango olungcono. Ngokomzekelo, i-subypeia ye-lemonemic i-promyelocytic (APL) edlalwa ngokuqhelekileyo iphathwa ngokusetyenziswa kweziyobisi ezihlukileyo kwazo ezisetyenziselwa ezinye i-subtype ze-AML.
Iinkqubo ezimbini ezibalulekileyo ezisetyenziselwe ukuhlukanisa i-AML zibe yi-subtypes yi-classification yaseFransi-American-British (FAB) kunye ne-World Health Organization (WHO).
Udidi lweFrench-American-British (FAB) lwe-AML
Ngee-1970, iqela leengcali zeFrench, iMelika kunye neBrithani zihlukanisa i-AML zibe ngamacandelwana, i-M0 ukuya kwi-M7, ngokusekelwe kwimizimba yeseli apho i-leukemia ikhula kwaye ikhula kangakanani iiseli. Oku kwakusekelwe ngokubanzi malunga nendlela iiseli ze-leukemia ezijonge ngayo ngaphantsi kwe-microscope emva kwebala eliqhelekileyo.
Igama le-FAB subtype
M0 Undifferentiated acute myeloblastic leukemia
M1 I-leyemia ye-myeloblastic enamandla kunye nokukhula okuncinci
I-M2 i-leyeloemlastic leukemia ehambelana nokukhula
I-M3 i-leukemia ye-promyelocytic (APL)
I-M4 i-myelomonocytic leukemia
I-M4 eos I-myelomonocytic leukemia ene-eosinophilia
I-M5 i-leocemical leukemia
I-M6 i-leryemia ye-leyemia
I-M7 I-megakaryoblastic leukemia
Amanqaku angama-M0 ukuya kwi-M5 onke aqala kwiifom ezingapheliyo zeeseli ezimhlophe zegazi. I-M6 AML iqala kwiifom ezibomvu zeeseli zegazi, ngelixa i-M7 i-AML iqalisa kwiifom ezingapheliyo zeeseli ezenza iiplatelet.
I-World Health Organisation (WHO) udidi lwe-AML
Inkqubo yesigaba se-FAB iluncedo kwaye isasetyenziselwa ukuqoqa i-AML ibe ngamacandelo, nangona ulwazi oluqhubekileyo ngokubhekiselele kwisigxina kunye nembono yeentlobo ezahlukeneyo ze-AML, kwaye ezinye zezi ntshukumo ziboniswe kwinkqubo ye-World Health Organisation (WHO) ka-2008.
Inkqubo ye-WHO ihlula i-AML kumaqela amaninzi:
I-AML eneempawu ezithile ezingafaniyo
- I-AML ene-translocation phakathi kwama-chromosomes 8 no-21
- I-AML ene-translocation okanye i-inversion kwi-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 okanye i-inversion kwi-chromosome 3
- I-AML (megakaryoblastic) kunye nokufuduka phakathi kwama-chromosomes 1 no-22
I-AML kunye neenguqulelo ezinxulumene ne-myelodysplasia
I-AML enxulumene nekhemotherapy yangaphambili okanye i-radiation
I-AML ayichazwe ngenye indlela (i-AML engayi kwiqela elikhankanywe apha ngasentla kwaye ihlelwe ngokubanzi njengokwenziwe kwindlela ye-FAB):
- I-AML enexabiso elincinci (M0)
- AML ngaphandle kokuvuthwa (M1)
- I-AML kunye nokuvuthwa (M2)
- I-myelomonocytic leukemia (M4)
- I-leocematic leukemia (M5)
- I-leryemia ye-erythroid (M6)
- I-acaryoblastic leukemia (M7)
- I-leukemia ye-basophilic
- I-panmyelosis enobuchule kunye ne-fibrosis
I-myeloid sarcoma (eyaziwa ngokuba yi-granulocytic sarcoma okanye i-chloroma)
Ukwanda kweMyeloid ehlobene ne-Down syndrome
I-leukemias e-Undifferentiated ne-biphenotypic
Lezi zi-leukemia ezinezo zimbini ze-lymphocytic ne-myeloid. Ngamanye amaxesha ubizwa ngokuba nguBONKE abaneempawu ze-myeloid, i-AML eneempawu ze-lymphoid, okanye i-leukemi.
Ngaphezulu kweentlobo ze-WHO zatshintshwa kwi-American Cancer Society.
Imithombo:
I-Papaemmanuil E, uGerstung M, et al. Uhlobo lweGenomic kunye nokugxekwa kwi-leukemia engummangaliso. N Engl J Med . 2016; 374 (23): 2209-21.
I-Wellcome Trust Sanger Institute. I-leukemia enesifo esilungileyo isifo okungenani izifo ezi-11. Ufikelele ngoJuni 2016.
American Cancer Society. Unjani i-leukemia ekhangelekile? Ufikelele ngoJuni 2016.