Kurwara myeloid yeukemia (CML) ndeimwe yezvikamu zvina zvakakura zveukemia . Zvimwe zvitatu zvinosanganisira myeloid leukemia, acute lymphoblastic leukemia, uye chronic lymphocytic leukemia.
Pasinei nemhando, maeukemias ose anotanga mumasero ekugadzira ropa mumapfupa emapfupa. Mhando ipi neipi yeukwepa inowana zita rayo kubva mukurwara kwekenza kunowedzera kukura (kanaya yakaoma inokura nokukurumidza; chisingagumi chinokura zvishoma) uyewo rudzi rwemasero ekugadzira ropa kubva apo kuipa kwemavara kwakagadzirwa.
CML inorwara neukemia , zvinoreva kuti inotanga kukura nekufambira mberi zvishoma. CML iine mutezo wemukemikari , zvinoreva kuti unotanga mumasero matsvuku eropa anozivikanwa semaseroid myeloid.
Chii Chinokonzera CML?
Zvimwe zvinoshandiswa muDNA zvinogona kuita kuti muviri wose wemasora masero ave masero eukemia. Vanhu vane CML vanowanzove neChristelphia chromosome , iyo ine maitiro asinganzwisisiki BCR-ABL geni. Iyo BCR-ABL jena inokonzera masero eropa eropa kuti akure nenzira isina kufanira, isina kudzora, inokonzera kuberekwa kweukemia.
Ndiani Anowana CML?
CML inogona kuitika chero ipi zvayo, asi inonyanya kuonekwa kune vakwegura kupfuura makore makumi mashanu, vanoverengera makumi manomwe kubva muzana ematambudziko ose. Kareem Abdul-Jabbar mumwe muAmerica anozivikanwa ane CML.
Ko CML Inowanikwa Sei?
CML haina kuwanana. MuUnited States muna 2017, kuongororwa kwaive kuti 8 950 mitsva itsva yaizoitika uye inofungidzirwa kuti vanhu 1080 vaizofa nehosha iyi.
Zviratidzo
Nemhaka yokuti CML inotanga kuwedzera-kenza, vanhu vazhinji havana zviratidzo apo vanotanga kuonekwa.
Mukati, kusvika ku40 kusvika ku50 muzana yevarwere vane zviratidzo zvacho zvachose, uye vanogamuchira kuongororwa kwavo mushure mekuita basa reropa ropa rinowona kusakanganisika.
CML inogona kukonzera zviratidzo sezvainoenderera mberi nenguva, zvakadaro. Zvichipa mamiriro ezvinhu aya, urongwa hwe "zviratidzo zvinowanzoonekwa" hunogona kurondedzerwa sezvinotevera:
- Hapana zviratidzo (kusvika ku50 muzana yevanhu vanorwara)
- Kuneta zvakanyanya kana kuneta
- Kushaya simba
- Fever
- Night sweats
- Unexplained kuora kurasikirwa
- Nhamo kana kuzara mukati-kuruboshwe kurutivi, pasi pemiti.
Chiratidzo chekupedzisira mune urongwa chikonzero nekuda kwepleen yakawedzerwa, inonziwo splenomegaly, iyo iripo mune 46 kusvika 76 muzana yevaya vane CML. Kuwedzerwa kwakadaro kwepleen kunogona kuunza nzvimbo shomanana kune dzimwe nhengo dzenharaunda yacho, kufanana nedumbu, izvo zvinogona kuita kuti pfungwa dzive dzakakwana kare pavanodya kudya.
Kuvhiringidzika uye kuneta izvo vamwe vanhu vane CML ruzivo vanogona kukura kubva kune dzakawanda zvakasiyana-siyana. Chimwe chezvinyorwa zveutera uye kuneta isemia, izvo zvinoreva kuti muviri hauna masero masero matsvuku eropa anotakura okisijeni kumatumbu. Anemia inogonawo kuita kuti unzwe sokuti iwe haugone kuzviedza kana kushandisa masumbu ako zvakasimba sekugara.
Kuziva
Chiremba wako achatora nhoroondo yako yezvokurapa uye anoita chiitiko chemuviri, sekunge chero imwe yeruzhinji rwechirwere.
Spleen Size
Kuchengeta ukuru hwepleen yako chikamu chinokosha chekuongorora kwemuviri. A spleen-size spleen haisi inowanzoonekwa, asi spleen yakawedzerwa inogona kuonekwa kurutivi rworuboshwe rwemucheche kumusoro, pasi pemucheto webambo rembambo.
Iyo spleen inowanzochengeta masero eropa uye inoparadza masero eropa rekare. Mu CML, chirwere chinogona kuwedzerwa nekuda kwemamwe masero matsvuku eropa ari muiri.
Dhiyabhorosi Miedzo
Kuongororwa kwebhizinesi kunodawo. Ropa rinowanzobviswa kubva mumhino muhuni, uye marara emapfupa anonyorwa kuburikidza nenzira inonzi bone marrow aspiration uye biopsy. Zviratidzo zvako zvinotumirwa kubhizi uye chiremba anozvidziongorora pasi pe microscope uye anoita zvimwe zviedzo, achitarisa kuwana uye kutsanangurazve mapepa eukemia, kana aripo.
Mamwe masero machena eropa uye maitiro asina kukwana emamwe makemikari muropa angave ari kuratidza CML.
Mune mapfupa emasera, apo mamwe masero ekugadzira ropa aripo anotarisirwa, marora anonzi anonzi hypercellular. Bone marrow inowanzonyanya kuwanda mu CML nokuti yakazadza masero eukemia.
Gene Test
Gene kuedza kuchaitwawo kutarisa "Philadelphia Chromosome" uye / kana BCR-ABL geni. Icho chiyero chekuedzwa chinoshandiswa kusimbisa kuongororwa kwe CML. Kana iwe usina Philadelphia chromosome kana BCR-ABL geni, iwe hauna CML.
Kufungidzira Miedzo
Zvidzidzo, kana kuti mifananidzo yekufungidzira, hazvidiwi kuti uongorore CML. Zvisinei, inogona kuitwa sechikamu chekubata kwako, pane dzimwe nguva; somuenzaniso, kuongorora zvimwe zviratidzo kana kuti kuona kana kune kuwedzera kwepleen kana chiropa.
Zvikamu zve CML
Mhosva dze CML dzinogona kuiswa muzvikamu zvitatu zvakasiyana zvinonzi phases. Chikamu chakavakirwa pauwandu hwemasero matsvuku eropa, kana kuputika, iwe une ropa neropa remafuta. Kuziva chikamu che CML yako kunogona kukubatsira kuti uwane pfungwa yekuti chirwere chako chichakukanganisa sei mune ramangwana.
Chronic Phase
Ichi ndicho chikamu chekutanga che CML. Muchikamu ichi, iwe unotova nenhamba inowedzera yemachena masero eropa muropa uye / kana pfupa marora. Zvisinei, aya masero matsvuku eropa, kana kuputika, anoumba zvishoma kudarika 10 kubva muzana yemasero muropa uye / kana mumarara.
Kazhinji, muchikamu chisingagumi, hapana zviratidzo, asi kunogona kuve nechokumusoro kwakasara kuzara kwepamuviri. Zvirwere zvemuviri wako zvichiri kushanda zvakajeka muchikamu chisingagumi, saka unenge uchikwanisa kukwanisa kurwa kwakanaka nezvirwere. Munhu anogona kuva muchikamu chisingagumi kwenguva shoma semwedzi shomanana kusvika kwenguva yakawanda seyakawanda, makore mazhinji.
Kufambisa Phase
Muchikamu chinowedzera, nhamba yekuputika masero muropa uye / kana marora emapfupa yakakwirira kudarika muchigarire chisingaperi uye masero eukemia anokura kuti atange zviratidzo zvinogona kusanganisira fivha, kuora muviri, kusanzwa nzara, uye chirwere chakawedzerwa.
Nhamba yemasero machena epamusoro pane akakwana uye iwe unogona kuchinja kuwedzerwa ropa rako, sekuwanda kwenhamba ye basophil kana nhamba yakaderera yeplatelets.
Kune zvikamu zvakasiyana zvezvirevo zvinoshandiswa mazuva ano zvinotsanangura chikamu chinowedzera. IWorld (World Health Organisation) Criteria inotsanangura chikamu chinokurumidza kufanana nekuvapo kwechinhu chipi nechipi chinotevera:
- 10 kusvika ku19 kubva muzana inoputika muropa neropa kana musara
- Zvinopfuura makumi maviri zvezana zvebasophils muropa
- Yakakwirira kwazvo kana yakadzika kwazvo platelet count iyo isiri yechirwere
- Kuwedzerwa kupenya kwepleen uye kuchena kwemajeri eropa pasinei nekurapa
- Zvitsva zvema genetic kana kuchinja
Blast Phase
Izvi zvinonziwo "kuputika kwekutambudzika," sezvo iri chikamu chechitatu uye chekupedzisira uye chinogona kuisa upenyu pangozi. Nhamba yemafuta ekufema muropa uye / kana marora emapfupa anova akakwirira zvikuru uye izvi zvinoputika masero akapararira kunze kweropa uye / kana mapfupa emapfupa kune mamwe maviri. Zviratidzo zvinowanzova zvakajairika mumuviri wechikamu, izvo zvinogona kusanganisira zvirwere, kubuda ropa, kurwadziwa mumimba, uye kurwadziwa kwepfupa.
CML mukufema chikamu inogona kuonekwa seyakanyanya kufanana neukemia kupfuura yenguva refu yeukemia. Muchikamu chechikamu, CML masero anogona kuita zvakadai se AML (acute myeloid leukemia) kana kupfuura zvakafanana ALL (acute lymphoblastic leukemia).
Tsanangudzo yeO WHO yekubvisa chikamu yakakura kudarika 20 muzana inokonzera masero muropa romuviri kana marota. I International Bone Marrow Transplant Registry tsanangudzo yekuputika kwechikamu yakakura kudarika 30 muzana inoputika masero muropa uye / kana mutema wemafuta. Tsanangudzo mbiri idzi dzinosanganisira kuvapo kwemajeri masero kunze kweropa kana marora.
Prognosis
Paunenge uchiedza kufanotaura zvaunofunga, chikamu che CML yako chinhu chinokosha, asi haisi iye chete chinhu.
Pane zvimwe zvinhu zvakawanda zvakaratidzwa kuti zvibatane nehutano hwako somunhu mumwe murwere, kusanganisira yezera rako, ukuru hwepleen yako, neropa rinoverengeka. Kubva pane izvozvi, munhu anogona kuwira mune chimwe chezvikamu zvitatu: zvakaderera, pakati, kana pangozi.
Vanhu vari muchikwata chimwechete chezvipingamupinyi vanowanzopindura nenzira yakafanana kurapwa. Vanhu vari muboka risina njodzi vanowanzoita zviri nani kurapwa. Zvisinei, mapoka aya mabhuku, kwete zviratidzo zvachose.
CML Zvirwere
Mishonga yose ine mungozi uye inobatsira, uye chisarudzo chekutora CML chimwe chinoitwa nekuve nekukurukurirana kwechiremba-murwere uye kuongorora mumwe munhu murwere uye chirwere chake uye hutano hwakakwana. Haasi munhu wose ane CML inogamuchira mishonga yose ye CML inokurukurwa pasi apa.
Tyrosine Kinase Inhibitor Therapy
Tyrosine kinase inhibitor therapy chirwere chechirwere. Chinangwa chii? Iri boka rezvinodhaka rinotarisa kusakosha BCR-ABL protein inobatsira CML masero kukura.
Aya mishonga inodzivisa protein ye BCR-ABL kubva kutumira zviratidzo zvinokonzera masero akawanda eM CML kuumba. Iyi mishonga inouya nenzira yepiritsi inogona kumedzwa.
Therapy | Tsanangudzo |
Imatinib | Yaiva wekutanga tyrosine kinase inhibitor yakagamuchirwa neRDA kuti iite CML; yakagamuchirwa muna 2001. |
Dasatinib | Yakagamuchirwa kuitira kurapwa kwe CML muna 2006. |
Nilotinib | Yakatanga kugamuchirwa kurapa CML muna 2007. |
Bosutinib | Yakagamuchirwa kurapa CML muna 2012, asi inogamuchirwa chete kune vanhu vakamborapwa neimwe tyrosine kinase inhibitor iyo yakamira kushanda kana kuti yakakonzera madhara akaipa. |
Ponatinib | Yakagamuchirwa kurapa CML muna 2012 asi inogamuchirwa chete kune varwere vane T315I mutation kana CML isingagadziri kana kusagadzikana kune imwe tyrosine kinase inhibitors. |
Immunotherapy
Interferon chinhu chinoshandiswa nemasikirwo emasikirwo. PEG (pegylated) interferon iyo inogara kwenguva yakaita chimiro chemishonga.
Interferon haishandisi sekurapa kwokutanga kwe CML, asi kune vamwe varwere, izvi zvingava sarudzo kana vasingagoni kuregerera tyrosine kinase inhibitor therapy. Interferon isiri inopindwa mujecha pasi peganda kana mu musumbu ine nheyo.
Chemotherapy
Omacetaxine inobva mishonga yemakemotherapi itsva yakagamuchirwa ku CML muna 2012, mune varwere vanorwisana uye / kana kusawirirana kune maviri kana kupfuura tyrosine kinase inhibitors. Kurwisana apo CML haina kupindura kune kurapwa. Kusagadzikana ndechokuti kurapwa nemishonga kunofanira kumira pamusana pemigumisiro yakakomba.
Omacetaxine inopiwa semvura inoshandiswa pasi peganda ine nheyo. Mimwe chemotherapy mishonga inogona kuiswa muvhein kana kuti inogona kupiwa sepiritsi kuti inye.
Hematopoietic Cell Transplant (HCT)
Pamberi pe tyrosine kinase inhibitors, izvi zvaifungidzirwa sarudzo yesarudzo ye CML, asi HCT yakagadzirwa nehutano yakaoma uye inogona kukonzera madhara akaipa. Nokudaro, zvingave zvisina kunaka kurapa kwakanaka kune varwere vose vane CML, uye mazhinji mazano ekurapa nhasi anongororere kurapwa uku kwevarwere vasati vava nemakore makumi matanhatu nemashanu.
High-dose chemotherapy inopiwa kutanga kuparadza zvose masero masero uye CML masero mumapfupa emapfupa. HCT ndiyo nzira inotsiva masero akaparadzwa mumapfupa emapfupa ako nemasero matsva, ane utano weropa.
Clinical Trials: Investigational Therapies
Mishonga mitsva inoramba ichiongororwa. Mishonga yemakiriniki emishonga mitsva inogona kuve sarudzo kune vamwe varwere. Iwe unogona kugara uchibvunza boka rako rekurapa kana kune chiremba chekuvhara muchipatara kuti iwe unogona kubatana uye kuti kana kwete ivo vanotenda iwe ungadai uri mukana wakanaka kune chiremba chakadai.
Shoko Rinobva
Kune munhu ane CML, kusabvumirana kunogona kuvimba nezvinhu zvakadai sezera, chikamu cheM CML, nhamba yezvipembenene muropa kana pfupa remukati, ukuru hwepleen pakuonekwa, uye hutano hwakakwana.
Nekuiswa kwezvinodhaka zvinonzi tyrosine kinase inhibitors kutanga muna 2001, vanhu vakawanda vane CML vakaita zvakanaka kwazvo, uye chirwere chinogona kugara chiri muchikamu chisingaperi chemakore.
Kunyange zvakadaro, zvinetso zvinoramba zvichiripo: zvinogona kuva zvakaoma kufanotaura, kubvira pakutanga, izvo varwere vane CML vangangodaro vane mabudiro akaipa. Uyezve, varwere vakawanda vanoda CML inotarirwa nekusingaperi, uye kurapa kunopesana hakusi kunze kwemigumisiro. Saka, apo kufambira mberi kwave kwakakosha mumakumi emakore apfuura, kuchine nzvimbo yekufambira mberi.
> Sources:
> National Cancer Institute. Chirwere Chisingaperi Chirwere Chirwere Chirwere.
> Thompson PA, Kantarjian HM, Cortes JE. Kuongororwa uye kurapwa kwechirwere chisingaperi chinonzi Leukemia muna 2015. Mayo Clin Proc . 201 5; 90 (10): 1440-54.
> Faderl S, Talpaz M, Estrov Z, O'Brien S, Kurzrock R, Kantarjian HM. Biology yekusingaperi myeloid leukemia. N Engl J Med . 1999; 341 (3): 164-172.