I-myeloid leukemia engapheli (CML) ingenye yezigaba ezine ezinkulu ze- leukemia . Ezinye ezintathu ziyi-leukemia ephawulekayo ye-myeloid, i-acute lymphoblastic leukemia, ne-lymphocytic leukemia engapheli.
Kungakhathaliseki uhlobo, wonke ama-leukemi aqala amangqamuzana awenza igazi emangeni lomthambo. Uhlobo ngalunye lwe-leukemia lubizwa ngegama lokuthi umdlavuza ophuthumayo uvame ukukhula kanjani (umdlavuza we-acute ukhula ngokushesha; ukugula okungapheliyo kuyakhula kancane) kanye nohlobo lwamaseli okwenza igazi avela kuwo.
I-CML iyi- leukemia engapheli , okusho ukuthi ithambe ukukhula futhi ithuthuke kancane. I-CML nayo i- leukemia engamangqamuzana , okusho ukuthi iqala emangqamuzaneni amhlophe egazi amhlophe aziwa njengamaseli ase-myeloid.
Yini eyenza i-CML?
Izinguquko ezithile ku-DNA zingabangela ukuba amaseli avamile omnyofu abe ngamaseli e-leukemia. Abantu abane-CML ngokuvamile babe ne-chromosome ye-Philadelphia , equkethe isakhi se-BCR-ABL engavamile. Ifulethi le-BCR-ABL lidala amangqamuzana amhlophe egazi ukuba akhule ngendlela engavamile, engalawulwa, ebangela i-leukemia.
Ubani othola i-CML?
I-CML ingenzeka kunoma yisiphi ubudala, kodwa ivame kakhulu kubantu abadala abangaphezu kweminyaka engama-50, ababalwa ngamaphesenti angaba ngu-70 azo zonke izimo. U-Kareem Abdul-Jabbar ungowomunye waseMelika owaziwayo onen CML.
I-CML ejwayelekile kangakanani?
I-CML ayifani neze. E-United States ngo-2017, ukulinganiselwa ukuthi kwakungenzeka amacala amasha angu-8 950 futhi abantu abangaba ngu-1 080 bazofa ngalesi sifo.
Izimpawu
Ngenxa yokuthi i-CML isifo somdlavuza okhula kancane, abantu abaningi abanayo izimpawu uma beqala ukutholakala.
Eqinisweni, iziguli ezingamaphesenti angaba ngu-40 kuya kwangu-50 azikho izimpawu nhlobo, futhi zithola ukuxilongwa kwazo emva komsebenzi wegazi njalo zibona ukungavamile.
I-CML ingabangela izimpawu njengoba iqhubeka nesikhathi, noma kunjalo. Njengoba kunikezwe lesi simo, uhlu lwe "izimpawu ezivamile" lungachazwa kanje:
- Azikho izimpawu (kuze kube ngamaphesenti angu-50 abantu abaxilongwa)
- Ukukhathala ngokweqile noma ukukhathala
- Ukungabi namandla
- Fever
- Izithukuthuku ebusuku
- Ukulahlekelwa kwesisindo esingachaziwe
- Ubuhlungu noma ukugcwala kwesisu esisenhla kwesokunxele, ngaphansi kwezimbambo.
Uphawu lokugcina kulolu hlu lubangelwa ipeni ekhulisiwe, ebizwa nangokuthi i-splenomegaly, ekhona ngamaphesenti angama-46 kuya kwangu-76 alabo abane-CML. Ukwandiswa okunjalo kwephethini kungabangela isikhala esingaphansi kwezinye izitho endaweni, njengesisu, okungase kube nomqondo wokuba ugcwalise ngokugcwele lapho udla ukudla.
Ubuthakathaka nokukhathala abanye abantu abanokuhlangenwe nakho kwe-CML abangathuthuka kusuka emininini yemithombo ehlukene. Omunye umthombo wobuthakathaka nokukhathala yi-anemia, okusho ukuthi umzimba awunawo okwanele amangqamuzana egazi abomvu athatha i-oxygen emahlakheni. I-anemia ingenza futhi uzizwe sengathi ngeke ukwazi ukuzikhandla noma ukusebenzisa imisipha yakho ngamandla ngendlela evamile.
Ukuxilongwa
Udokotela wakho uzothatha umlando wakho wezokwelapha futhi enze ukuhlola ngokomzimba, njenganoma yikuphi ukuhlolwa kokugula.
Ubukhulu besipeni
Ukuhlola ubukhulu besipeni sakho yingxenye ebalulekile yokuhlolwa ngokomzimba. I-spleen ejwayelekile evamile ayizwa, kodwa ipeni ekhulisiwe ingatholakala ngakwesokunxele kwesisu esisenhla, ngaphansi komngcele wezintambo zomhlanga.
I-spleen ngokuvamile igcina amangqamuzana egazi futhi ibhubhise amangqamuzana endala egazi. Ngo-CML, i-spleen ingahle ikhuliswe ngenxa yawo wonke amangqamuzana egazi amhlophe ahlala egazini.
Izivivinyo ze-Lab
Ukuhlolwa kwelabhulali nakho kuyadingeka. Igazi livame ukuthathwa emgqeni wesandla, futhi umthambo wethambo usampuliwe ngenqubo ebizwa ngokuthi i-bone marrow aspiration kanye ne-biopsy. Ama-sampuli akho athunyelwa ebhokisini futhi udokotela wezilwane ubahlola ngaphansi kwe-microscope futhi wenza ezinye izivivinyo, efuna ukuthola nokuchaza kabanzi amaseli we-leukemia, uma ekhona.
Amangqamuzana amaningi amhlophe wegazi namazinga angavamile amakhemikhali athile egazini angabonisa ukuthi yi-CML.
Emasampheni omnyo wesifuba, lapho amaseli amaningi okwenza igazi ekhona khona kulindeleke, umnkantsha kuthiwa ukhululekile. Umnkantsha we-bone uvame ukuxhuma kakhulu ku-CML ngoba ugcwele amangqamuzana e-leukemia.
Izivivinyo ze-Gene
Ukuhlolwa kwe-Gene kuzokwenziwa futhi ukuze ubuke "i-chromosome ye-Philadelphia" kanye / noma isakhi se-BCR-ABL. Lolu hlobo lokuhlolwa lusetshenziselwa ukuqinisekisa ukuxilongwa kwe-CML. Uma ungenayo i-chromosome ye-Philadelphia noma i-gene ye-BCR-ABL, awunayo i-CML.
Ukuhlolwa kwezilingo
Ukuhlola, noma ukuhlolwa kwe-imaging, akudingeki ukuxilonga i-CML. Kodwa-ke, zingase zenziwe njengengxenye yokusebenza kwakho, kwezinye izimo; isibonelo, ukuphenya izimpawu ezithile noma ukubona ukuthi kukhona yini ukwandiswa kwesipere noma isibindi.
Izigaba ze-CML
Amacala we-CML angahlukaniswa ngamaqembu amathathu ahlukene okuthiwa izigaba. Lesi sigaba sisekelwe enani lamaseli amhlophe egazi amhlophe, noma ukuqhuma, okukhona egazini nasomnothweni wezintambo. Ukwazi isigaba se-CML yakho kungakusiza ukuba uthole umqondo wokuthi ukugula kwakho kuzokuthinta kanjani esikhathini esizayo.
Isigaba Esingajwayelekile
Lesi yisigaba sokuqala se-CML. Kulesi sigaba, usuvele unenombolo ekhuphukile yamangqamuzana egazi amhlophe egazini kanye / noma ematheni. Nokho, lawa maseli amhlophe egazi amhlophe, noma amabhomu, enza amaphesenti angaphansi kuka-10 amaseli egazini kanye / noma ematheni.
Ngokuvamile, esigabeni esingapheli, azikho izimpawu, kodwa kungenzeka kube khona ukugcwala kwesisu kwesokunxele. Amasosha omzimba wakho asasebenza kahle kakhulu esigabeni esingapheli, ngakho-ke usenakho ikhono lokulwa okuhle nokulwa nezifo. Umuntu angaba isigaba esingapheli isikhathi esifushane njengezinyanga ezimbalwa kuze kube iminyaka eminingi, eminingi.
Isigaba esisheshayo
Esigabeni esiphuthumayo, inani lokuqhuma kwamangqamuzana egazini kanye / noma emangeni lamathambo liphakeme kunalesi sigaba esingapheli futhi amangqamuzana e-leukemia akhula ukudala izimpawu ezingase zibandakanye umkhuhlane, ukulahlekelwa isisindo, ukungabi nesilamba, nesipula esikhulisiwe.
Inani lamaseli amhlophe egazi liphakeme kunalesijwayelekile futhi ungaba nezinguquko ezibalweni zakho zegazi, njengenombolo ephezulu ye-basophil noma inombolo ephansi yamaplatelet.
Kunezigaba ezahlukene zezindlela ezisetshenziswa namuhla ezichaza isigaba esisheshayo. I-WHO (World Health Organisation) I-Criteria ichaza isigaba esisheshayo njengokuba khona kwalokhu okulandelayo:
- Amaphesenti angu-10 kuya ku-19 aqhuma emgodleni wegazi kanye / noma emathambo
- Amaphesenti angaphezu kuka-20 ase-basophil egazi
- Inani leplatelet eliphakeme kakhulu noma eliphansi kakhulu elingathinti ukwelashwa
- Ukwandisa usayizi wepen kanye nokubala kwegazi elimhlophe naphezu kokwelashwa
- Izinguquko ezintsha zofuzo noma izinguquko
Isigaba sokuqhuma
Lokhu kubhekiselwa nangokuthi "ukuhlukunyezwa kokuqhuma," ngoba kuyisigaba sesithathu futhi sokugcina futhi sinamandla okusongela ukuphila. Inombolo yokuqhuma amangqamuzana egazini kanye / noma ematheni amathambo eba phezulu kakhulu futhi lawa maseli aqhuma ngaphandle kwegazi kanye / noma umongo wezinye izicubu. Izimpawu zivame ngokwengeziwe esigabeni sokuqhuma, okungase kuhlanganise izifo, ukuphuma kwegazi, ubuhlungu besisu, nobuhlungu besifuba.
I-CML isigaba sokuqhuma singase sibonakale sinjenge-leukemia enesifo esiyingozi kune-leukemia engapheli. Esigabeni sokuqhuma, amangqamuzana e-CML angase aziphathe njenge-AML (i-acute myeloid leukemia) noma ngaphezulu njenge-ALL (i-acute lymphoblastic leukemia).
Incazelo ye-WHO yesigaba sokuqhuma ikhulu kunezingamaphesenti angu-20 ibhula amangqamuzana egazini legazi noma ematheni. Incazelo ye-International Bone Marrow Transplant yokubhalisa kwesigaba sokuqhuma ingaphezu kwamaphesenti angu-30 aqhuma amangqamuzana egazini kanye / noma ematheni. Zombili izincazelo zifaka ukuthi kukhona ukuqhuma kwamangqamuzana ngaphandle kwegazi noma umthambo we-bone.
Isibikezelo
Uma uzama ukubikezela ukubikezela kwakho, isigaba se-CML yakho siyinto ebalulekile, kodwa akuyona into eyodwa kuphela.
Kunezinye izinto eziningana eziye zaboniswa ukuthi ziqondane nobungozi bakho njengesiguli ngasinye, kuhlanganise nobudala bakho, ubukhulu besitezi sakho, nesilinganiso segazi. Ngokusekelwe ezimweni ezinjalo, umuntu angawela kwesinye sezigaba ezintathu: okuphansi, okuphakathi, noma engozini enkulu.
Abantu abasesigabeni esisodwa sengozi bangase baphendule ngokufanayo ngendlela yokwelashwa. Abantu abaseqenjini elisezingeni eliphansi ngokuvamile baphendula kangcono ukwelashwa. Noma kunjalo, lezi ziqoqo ngamathuluzi, hhayi izinkomba eziphelele.
Ukwelashwa kwe-CML
Zonke izifo zinezingozi nezinzuzo ezingase zibe khona, futhi isinqumo sokuphatha i-CML senziwa ngokuxoxa ngezigulane nodokotela futhi sihlolisise isiguli esithile nesifo sakhe kanye nempilo yonke. Akuwona wonke umuntu one-CML othola yonke imithi ye-CML exoxwe ngezansi.
I-Tyrosine Kinase Inhibitor Therapy
I-Tyrosine kinase inhibitor therapy yindlela yokwelashwa okuhlosiwe. Iyini injongo? Leli qembu lezidakamizwa lihlose iphrotheni engavamile ye-BCR-ABL esiza amangqamuzana e-CML akhule.
Lezi zidakamizwa zivimbela iphrotheni ye-BCR-ABL ekuthumeleni izimpawu ezibangelwa amangqamuzana amaningi kakhulu we-CML. Lezi zidakamizwa ziza ngesimo samaphilisi angagwinywa.
Ukwelapha | Incazelo |
Imatinib | Ingabe i-tyrosine kinase inhibitor yokuqala eyamukelwa yi-FDA ukuphatha i-CML; avunyelwe ngo-2001. |
I-Dasatinib | Uvunyelwe ukwelashwa kwe-CML ngo-2006. |
Nilotinib | Kwamukelwa kuqala ukuphatha i-CML ngo-2007. |
I-Bosutinib | Kuvunyelwe ukwelapha i-CML ngo-2012, kodwa kuvunyelwe kuphela kubantu abaye baphathwa omunye umshini wokuvimbela u-tyrosine kinase oye wayeka ukusebenza noma obangele imiphumela emibi kakhulu. |
I-Ponatinib | Kuvunyelwe ukuphatha i-CML ngo-2012 kodwa ivunyiwe kuphela ezigulini ezine-T315I noma i-CML engavumelani noma engavumelani namanye ama-inhibitors we-tyrosine kinase. |
Immunotherapy
I-interferon iyisisetshenziswa esenza ngokwemvelo isistimu yomzimba. I-PEG (pegylated) i-interferon ifomu elibambe iqhaza lomuthi.
I-Interferon ayisetshenziswanga ukwelashwa kokuqala kwe-CML, kodwa kwezinye iziguli, lokhu kungaba yinketho uma bengakwazi ukubekezelela ukwelashwa kwe-tyrosine kinase inhibitor. I-Interferon yilezi zinhlayiya ezijova ngaphansi kwesikhumba noma zibe yisisipha esinaliti.
I-Chemotherapy
I-Omacetaxine iyisidakamizwa esisha esakhiwa yi-CML ngo-2012, kwiziguli ezinokuphikisana kanye / noma ukungaboni ngaso sonke isikhathi ku-inhibitors ezimbili noma ngaphezulu ze-tyrosine kinase. Ukuphikiswa lapho i-CML ingaphenduli khona ekwelapheni. Ukungaboni ngaso sonke isikhathi lapho ukwelashwa nomuthi kufanele kumiswe ngenxa yemiphumela emibi kakhulu.
I-Omacetaxine inikezwa njengetshezi elitshalwe ngaphansi kwesikhumba ngenaliti. Ezinye izidakamizwa ze-chemotherapy zingase zijojowe emthanjeni noma zinganikezwa njengephilisi ukugwinya.
I-Hematopoietic Transplant Cell (HCT)
Ngaphambi kwe-tyrosine kinase inhibitors, lokhu kwakubhekwa ukwelashwa okukhethwa yi-CML, kodwa i-HCT ye-allogeneic iyinkimbinkimbi futhi ingabangela imiphumela emibi kakhulu. Ngakho-ke, kungase kungabi ukhetho olungcono lwezokwelashwa kuzo zonke iziguli ezine-CML, futhi izikhungo eziningi zokwelapha namuhla zicabangela lokhu kuphela ukwelashwa kweziguli ezingaphansi kweminyaka engama-65 ubudala.
I-high-dose chemotherapy inikezwa kuqala ukubhubhisa kokubili amangqamuzana avamile namangqamuzana e-CML emnothweni wethambo. I-HCT iyindlela eyenza amangqamuzana abhubhise emnothweni wakho ngamangqamuzana amasha, aphilile enegazi.
Izivivinyo Zomtholampilo: Ukwelashwa Kwezokuphenya
Izidakamizwa ezintsha zicwaningwa ngokuqhubekayo. Ukuhlolwa kwemitholampilo yezokwelapha ezintsha kungase kube ukhetho kwezinye iziguli. Ungakwazi njalo ukucela ithimba lakho lokwelashwa uma kunesivivinyo sokuvuleka emtholampilo ongayijoyina nokuthi ngabe bakholelwa yini ukuthi uzoba ngumuntu omele ukhetho lololu hlelo.
Izwi elivela
Kulowo muntu nge-CML, ukubikezela kungancika ezintweni ezifana nobudala, isigaba se-CML, inani lokuqhuma egazini noma emathambo, ubukhulu be-spleen ekuxilongweni, kanye nempilo yonke.
Ngokufakwa kwezidakamizwa ezibizwa ngokuthi i-tyrosine kinase inhibitors kusukela ngo-2001, abantu abaningi abane-CML benze kahle kakhulu, futhi lesi sifo singagcinwa isigaba esingapheli iminyaka.
Noma kunjalo, kunezinselele eziningana eziqhubekayo: kungaba nzima ukubikezela, kusukela ekuqaleni, iziphi iziguli ezine-CML cishe ezizoba nemiphumela empofu. Ukwengeza, iziguli eziningi zidinga ukuthi i-CML iphathwe ngokungapheli, futhi ukwelashwa okucindezelayo akusikho imiphumela emibi. Ngakho-ke, ngenkathi intuthuko ibalulekile phakathi neminyaka eminyaka yamuva, kusekhona indawo yokuthuthukiswa okuqhubekayo.
> Imithombo:
> National Cancer Institute. Ukwelashwa Okungapheli Kwama-Myelogenous Leukemia.
> Thompson PA, Kantarjian HM, Cortes JE. Ukuxilongwa nokwelashwa kwe-Myeloid Leukemia engapheli ngo-2015. I- Mayo Clin Proc . 201 5; 90 (10): 1440-54.
> Faderl S, Talpaz M, Estrov Z, O'Brien S, Kurzrock R, HM Kantarjian. I-biology ye-chronic myeloid leukemia. N Engl J Med . 1999; 341 (3): 164-172.