I-leukemia (Chronicle leukemia) engapheliyo (CML) yenye yeendidi ezine ezinkulu ze- leukemia . Ezinye ezithathu ziyi-leukemia enkulu, i-lymphoblastic leukemia, kunye ne-lymphocytic leukemia.
Kungakhathaliseki ukuba luhlobo luni, zonke i-leukemi ziqala kwiiseli ezenza igazi kwindawo yomnatha. Uhlobo ngalunye lwe-leukemia lufumana igama laso ekubeni umdlavuza okhawuleza ukhula njani (umdlavuza okhulayo ukhula ngokukhawuleza; ukungafihlayo kukhula ngokukhawuleza) kunye nohlobo lwama-cell form forming.
I-CML ingumdlavuza ongapheliyo , oku kuthetha ukuba ukhula kwaye uqhube phambili. I-CML nayo i -leukemia ye-myelogenous , oku kuthetha ukuba iqala kwiiseli ezimhlophe zegazi ezimhlophe ezibizwa ngokuba ngama-cell smeloid.
Yintoni eyenza i-CML?
Utshintsho oluthile kwi-DNA lunokubangela ukuba iiseli zomnxeba eziqhelekileyo zibe ngamaseli e-leukemia. Abantu abane-CML ngokubanzi banayo i-chromosome yakwa -Philadelphia , equle i-gene ye-BCR-ABL engavamile. Umzimba we-BCR-ABL wenza ukuba amaseli egazi amhlophe akhule ngendlela engavamile, engalawulwayo, ebangela i-leukemia.
Ngubani ofumana i-CML?
I-CML inokwenzeka nanini nayiphi na iminyaka, kodwa iyaqhelekileyo kubantu abadala abangaphezu kweminyaka engama-50, ababalelwa kuma-70 ekhulwini kuwo onke amacala. U-Kareem Abdul-Jabbar ungowomnye waseMerika owaziwayo ophethe i-CML.
I-CML eqhelekileyo?
I-CML ayifaneki. EUnited States ngo-2017, uqikelelo lwama-8,950 amatyala amatsha aya kwenzeka kwaye abantu abangama-1,080 baya kufa ngesi sifo.
Iimpawu
Ngenxa yokuba i-CML isifo somhlaza esikhulayo, abaninzi abantu abanalo iimpawu xa baqala ukuxilongwa.
Enyanisweni, ukuya kuma-40 ukuya kuma-50 ekhulwini kwezigulane abanalo naluphi na izimpawu, kwaye bafumana ukuxilongwa kwabo emva kokuba umsebenzi wegazi oqhelekileyo ufumanisa ukungaqhelekanga.
I-CML inokubangela iimpawu njengoko kuqhubeka kunye nexesha, nangona kunjalo. Ngenxa yolu meko, uluhlu "lweempawu eziqhelekileyo" lungachazwa ngale ndlela:
- Akukho zibonakaliso (kufika kuma-50 ekhulwini abantu abachazwayo)
- Ukukhathala okanye ukukhathala
- Ubuthathaka
- Fever
- Ukukhupha ebusuku
- Ukulahleka kwesisindo esingachazwanga
- Ubunzima okanye ukuzaliswa kwisisu esisentla sekhohlo, phantsi kwembambo.
Uphawu lokugqibela kuloluhlu lubangelwa ipeni ekhulisiweyo, ebizwa ngokuba yi-splenomegaly, ekhoyo kwi-46 ukuya kuma-76 ekhulwini kulabo abane-CML. Ukwandiswa okunjalo kwendiza kunokubangela indawo engaphantsi kwezinye izitho kwindawo, njengesisu, ezinokubangela ukuba ufikelele ngokukhawuleza xa udla ukutya.
Ubuthathaka kunye nokukhathala ukuba abanye abantu abanamava e-CML bangahlakulela kwimimandla eyahlukeneyo. Omnye umthombo wobuthathaka kunye nokukhathala yi-anemia, oku kuthetha ukuba umzimba awunalo okwaneleyo iiseli zegazi ezibomvu ezithwala i-oksijini kwizicubu. I-anemia inokukwenza ukuba uzive ngathi awukwazi ukuzikhandla okanye usebenzise imisipha yakho ngokukhawuleza njengesiqhelo.
Ukuxilongwa
Ugqirha wakho uya kuthatha imbali yakho yonyango kwaye enze uvavanyo lomzimba, njengawo nawuphi na olunye uvavanyo lokugula.
Ubukhulu bePenkile
Ukuhlola ubungakanani benteni yakho yingxenye ebalulekileyo yovavanyo lomzimba. I-spleen eqhelekileyo ayiqhelekanga, kodwa i-spleen ephakamileyo ingafunyanwa kwicala lasekhohlo lesisu esisenyakatho, ngaphantsi kwemida yembambo.
I-spleen ivame ukugcina iiseli zegazi kwaye ichithe iiseli zamandulo zegazi. Kwi-CML, i-spleen ingaba yanda ngenxa yazo zonke iiseli zegazi ezimhlophe ezihlala kwiqumrhu.
Uvavanyo lweBeb
Iimvavanyo zeLebhu ziyadingeka. Igazi lisoloko lithathwa kwi-vein engalweni, kwaye umongo wethambo unesampulu ngokusebenzisa inkqubo ebizwa ngokuba yi-bone marrow aspiration kunye ne-biopsy. Iisampula zakho zithunyelwa kwibhabhatri kwaye i-pathologist iwahlola phantsi kwe-microscope kwaye yenza ezinye iimvavanyo, efuna ukufumana nokuchaza kwakhona iiseli ze-leukemia, ukuba zikhoyo.
Uninzi lweeseli zegazi ezimhlophe kunye namanqanaba angavumelekanga kwiikhemikhali ezithile kwigazi zingabonakalisa i-CML.
Kwiesampuli zamathambo, xa kusetyenziswa ezinye iiseli zegazi ezilindelekileyo, umrho kuthiwa yi-hypercellular. Umnkantso we-Bone udla ngokubanzi kwi-CML kuba uzele iiseli ze-leukemia.
Uvavanyo lweGenes
Uvavanyo lwe-Gene luya kwenziwa kwakhona ukukhangela "i-chromosome yaseFiladelphia" kunye / okanye i-gene ye-BCR-ABL. Olu hlobo lovavanyo lusetyenziswa ukuqinisekisa ukuxilongwa kwe-CML. Ukuba awunayo i-chromosome yeFiladelphia okanye i-gene ye-BCR-ABL, ke awunayo i-CML.
Uvavanyo lweemvavanyo
Izicwangciso, okanye iimvavanyo zokucinga, akudingeki ukuba uhlolisise i-CML. Nangona kunjalo, zinokuthi zenziwe njengenxalenye yokukhupha kwakho, kwezinye iimeko; umzekelo, ukuphanda iimpawu ezithile okanye ukubona ukuba kukho ukwandiswa kwetyeni okanye isibindi.
Izigaba ze-CML
Iimeko ze-CML zingabalwa zibe ngamaqela amathathu ahlukeneyo abizwa ngokuba ngamaqela. Isigaba sisekelwe kwinani lamaseli egazini amhlophe, okanye ukuqhuma, onayo egazini nasentolongeni yethambo. Ukwazi isigaba se-CML yakho kunokukunceda ukuba ufumane ingqalelo indlela ukugula kwakho kukuchaphazela ngayo esikhathini esizayo.
IsiGaba esingapheliyo
Le yigaba yokuqala ye-CML. Kule nqanaba, sele usenayo inani elongeziweyo lamaseli egazi ezimhlophe egazini kunye / okanye ematyeni. Nangona kunjalo, la maseli egazini amhlophe egazini, okanye ukuqhuma, ayenzi ngaphantsi kweepesenti ezili-10 zeeseli egazini kunye / okanye ematyeni.
Ngokuqhelekileyo, kwisigaba esingasigxina, akukho zibonakaliso, kodwa kunokubakho ukugcwala kwesisu esisentla. Isistim somzimba sakho sisasebenza kakuhle kwisigaba esingapheliyo, ngoko unako ukukwazi ukubeka imfazwe elungileyo ngokuchasene nezifo. Umntu unokuba kwisigaba esingapheliyo esifutshane nje ngeenyanga ezimbalwa ukuya kudala, iminyaka emininzi.
IsiGaba esiKhuselekileyo
Kwinqanaba elikhawulezileyo, inani lokuqhuma kweeseli egazini kunye / okanye ematyeni amathambo liphezulu ngaphezu kwesigaba esingapheliyo kwaye iiseli ze-leukemia zikhula zibangele iimpawu ezinokubandakanya umkhuhlane, ukulahleka kwesisindo, ukungabi nalambile, kunye nepen ekhulisiwe.
Inani leeseli ezimhlophe zegazi liphezulu kunezinto eziqhelekileyo kwaye unokuba neenguqu kwiibalo zakho zegazi, njengenombolo ephezulu ye-basophil okanye inombolo ephantsi yeeplatelet.
Kukho iiseti ezahlukeneyo zeendlela ezisetyenziswayo namhlanje ezichaza isigaba esheshayo. I-WHO (i-World Health Organisation) Iimpawu ezichaza isigaba esiphuthumayo njengokuba kukho naziphi na ezi zilandelayo:
- Amaphesenti angama-10 ukuya kuma-19 aqhutywe kwi-bloodstream kunye / okanye ematyeni
- Amaphesenti angama-20 e-basophil egazini
- Inani eliphakamileyo kakhulu okanye eliphantsi kakhulu leplatelet elingaxhomekeke kunyango
- Ukwandisa ubungakanani bepen kunye nombolo yamhlophe yegazi ngaphandle kwonyango
- Utshintsho olutsha lwezityalo okanye utshintsho
Isigaba seBlast
Oku kubhekiselwa nangoku ngokuthi "ukuphazamiseka kwengxaki," kuba isigaba sesithathu nesigxina kwaye sinakho ukusongela ubomi. Inombolo yokufutha kweeseli egazini kunye / okanye ematyeni amathambo iba phezulu kakhulu kwaye la maqhekeza aqhuma ngaphandle kwegazi kunye / okanye umongo wethambo kwezinye iiscupu. Iimpawu zixhaphake kakhulu kwisigaba sokuqhuma, esinokubandakanya izifo, ukuphuma kwegazi, intlungu yesisu, kunye nentlungu yesifo.
I-CML kwisigaba sokuqhuma ibonakala ngathi i-leukemia ephazamisayo kune-leukemia engapheliyo. Kwisigaba sokuqhuma, ii-CML iiseli zinokuziphatha ngokufana ne-AML (i-leyemia ye-myeloid acute) okanye i-ALL (i-lymphoblastic leukemia).
Incazelo ye-WHO yesigaba sokuqhuma iphezulu kunama-20 ekhulwini aqhutywe ngamaseli egazini okanye emathambo. Inkcazo ye-International Marrow Marrow Transplant definition of phase blow is greater than 30 percent in cells shed in blood and / or bone. Zombini iinkcazo ziquka ubukho bokuqhuma kweeseli ngaphandle kwegazi okanye ithambo lomongo.
Prognosis
Xa uzama ukuqikelela ubungqina bakho, isigaba se-CML yakho yinto ebalulekileyo, kodwa akuyena kuphela umbandela.
Kukho ezinye izinto ezininzi eziye zaboniswa ukuba zihambelane nomngcipheko wakho njengesigulane ngasinye, kubandakanya ubudala bakho, ubukhulu bepleen yakho, kunye nokubala kwegazi. Ngokusekelwe kwimiba enjalo, umntu angawela kwelinye leentlobo zintathu: eziphantsi, eziphakathi, okanye eziphezulu.
Abantu abakwiqela elijongene nobungozi banokuthi baphendule ngendlela efanayo kwonyango. Abantu abakwiqela eliphantsi kweengozi ngokubanzi baphendule bhetele unyango. Nangona kunjalo, ezi qela zixhobo, kungekhona izikhombisi.
Zonyango zeMLL
Zonke iindlela zonyango zinobungozi kunye neenzuzo, kunye nesigqibo sokuphatha i-CML yinto eyenziwa ngokuba neengxoxo zogqirha nezigulane kunye nokuvavanya isigulane ngasinye nesifo sakhe kunye nempilo yonke. Akunjalo wonke umntu ophethe i-CML ufumana zonke iinkonzo ze-CML ezixutywe ngezantsi.
ITyrosine Kinase Inhibitor Therapy
I-Tyrosine kinase unyango lwe-inhibitor luhlobo lonyango olujoliswe kuyo. Yintoni ekujoliswe kuyo? Eli qela leziyobisi lijolise kwiprotheni engavumelekanga ye-BCR-ABL eyenza i-CML iseli ikhule.
Ezi zi yobisi zivimbela iprotheni ye-BCR-ABL ekuthumeleleni iimpawu ezibangelwa iiseli ezininzi ze-CML. Ezi zi yobisi ziza kwifom yeepilisi ezinokugwinya.
Unyango | Inkcazo |
Imatinib | Ngaba i-tyrosine kinase inhibitor yokuqala evunywe yi-FDA ukuphatha i-CML; kuvunywe ngo-2001. |
Dasatinib | Kuvunyiwe ukuba unyango lwe-CML ngo-2006. |
Nilotinib | Kuye kwavunyelwa kuqala ukuphatha i-CML ngo-2007. |
Bosutinib | Kuyavunyelwa ukunyanga i-CML ngo-2012, kodwa ivunyiwe kuphela kubantu abaye baphathwa ngenye inkunkuma ye-tyrosine kinase eyekile ukusebenza okanye yabangela imiphumo emibi kakhulu. |
Ponatinib | Uvunyelwe ukunyanga i-CML ngo-2012 kodwa ivunyiwe kuphela kwizigulane ezine-T315I okanye i-CML enqabileyo okanye engahambisani nayo nezinye i-tyrosine kinase inhibitors. |
Immunotherapy
I-Interferon yinto efanelekileyo eyenziwa ngumzimba. I-PEG (pegylated) i-interferon yindlela ebenzela ixesha elide leziyobisi.
I-Interferon ayisetyenziswanga njengonyango lokuqala lwe-CML, kodwa kwezinye izigulane, oku kungenokwenzeka xa bengakwazi ukunyamezela unyango lwe-inhibitor ye-tyrosine kinase. I-Interferon yilwelo ejoyiweyo phantsi kwesikhumba okanye kwi-muscle enealiti.
Chemotherapy
I-Omacetaxine yilezi zixhobo zonyango lwe-chemotherapy olutsha olwamkelwe kwi-CML ngo-2012, kwizigulane ezinokumelana kunye / okanye ukunyanzelana kwi-inhibitors ezimbini okanye ngaphezulu kwe-tyrosine kinase. Ukuchasana xa i-CML ingaphenduli kwonyango. Ukunganyamezelwanga xa unyango kunye neyeza kufuneka lugqitywe ngenxa yemiphumo emibi.
I-Omacetaxine inikezwa njengelwelo elityalwa phantsi kwesikhumba ngenaliti. Ezinye iziyobisi ze-chemotherapy zingafakwa kwi-vein okanye ziyakunikwa njengepilisi yokugwinya.
I-Hematopoietic Transplant Cell (HCT)
Ngaphambi kokuba i-tyrosine kinase inhibitors, oku kuthethwa ngonyango olukhethiweyo lwe-CML, kodwa i-HCT ye-allogeneki yinkqubo enzima kwaye ingabangela iziphumo ezibi kakhulu. Ngaloo ndlela, kungenakuba yinto efanelekileyo yokunyanga unyango kuwo wonke isigulane nge-CML, kwaye amaziko amaninzi okhathalelo namhlanje aqwalasela olu khetho unyango kwizigulane ezingaphantsi kweminyaka engama-65 ubudala.
I-high-dose ye-chemotherapy inikezelwa kuqala ukutshabalalisa zombini iiseli eziqhelekileyo kunye namaseli e-CML kwintolongo yethambo. I-HCT yinkqubo ethatha indawo yetsholongwane etshabalalayo emnzini wakho wethambo kunye neentsholongwane ezintsha, ezisempilweni ezenza igazi.
Uvavanyo lweZliniki: Uphando lwezoPhando
Amachiza amatsha aqhutyelwa uphando. Uvavanyo lwezonyango lwezilwanyana ezintsha luza kuba lukhetho lwezigulane ezithile. Unokuhlala ucela iqela lakho lonyango ukuba kukho ityala elivulekileyo lekliniki ongayifumana kunye nokuba ngaba bakholwa ukuba uya kuba ngumviwa ofanelekileyo kwilingo lekliniki.
ILizwi
Ngomntu ngamnye nge-CML, ukuxhaswa kwemali kunokuxhomekeka kwizinto ezifana nobudala, isigaba se-CML, inani lokuqhuma kwi-blood okanye ithambo lomnkantso, ubungakanani bepeni ekuxilongweni, kunye nempilo yonke.
Ngokuqaliswa kweziyobisi ezibizwa ngokuba yi-tyrosine kinase inhibitors ukususela ngo-2001, abantu abaninzi abane-CML benze kakuhle kakhulu, kwaye isifo sinokugcinwa kwisigaba esingapheliyo iminyaka.
Sekunjalo, kukho imingeni emininzi: kunokuba kunzima ukuqikelela, ukususela ekuqaleni, iziphi izigulane ezine-CML ziyakwazi ukuba neziphumo ezimbi. Ukongezelela, ezininzi izigulana zifuna i-CML iphathwe ngokungapheliyo, kwaye unyango oluxhasayo alukho ngaphandle kweempembelelo. Ngoko ke, ngelixa ukuqhubela phambili kuye kwaphawuleka kwiminyaka emva nje, kusekho indawo yokuphucula phambili.
> Imithombo:
> INational Cancer Institute. Ukwelashwa kwe-Leyoemia ye-Myelogenous Leukemia.
> Thompson PA, i-Kantarjian HM, iCortes IY. Ukuxilongwa kunye nokunyangwa kwe-Leukemia engapheliyo ngo-2015. I- Mayo Clin Proc . 201 5; 90 (10): 1440-54.
> Faderl S, Talpaz M, Estrov Z, O'Brien S, Kurzrock R, HM eKantarjian. I-biology ye-leukemia engapheliyo. N Engl J Med . 1999; 341 (3): 164-172.