I-Myelomonocytic Leukemia (CMML)

I-lemonemia ye-myelomonocytic, noma i-CMML, ingumdlavuza wamangqamuzana egazi ahlala emnothweni wethambo. Ku-CMML, ukungajwayelekile emangqamuzaneni akha igazi akusho nje ukuthinta umthambo wethambo-kubangele umuntu abe ne-monocyte eminingi kakhulu-uhlobo lwegciwane legazi elimhlophe-futhi lokhu kuthinta nezinye izingxenye zomzimba kanye.

I-CMML ne-CML

I-CMML imele i-myelomonocytic leukemia engapheli, kuyilapho i-CML imele i- myeloid leukemia engapheli (ebizwa nangokuthi i-lexemia engapheliyo i-myelogenous leukemia).

Kungaba khona ukufana phakathi kwe-CML ne-CMML ngokwezinto zokutholwa zokuqala, kodwa lezi zifo ezimbili zihlukile.

Kulezi zimbini, i-CMML ngokuvamile ibhekene nokuhlaselwa okubi nakakhulu kune-CML, nakuba izici eziningi ezihlukahlukene zingaba nomthelela ekutheni umuntu ahlasele futhi aphile futhi kungenzeka kube nemithi yokwelapha etholakalayo kuye ngokuthi kunjani.

Izimbangela Nezici Zengozi

I-CMML ivela ikakhulu kubantu abadala, ngokuvamile abantu abangaphezu kweminyaka engama-65.

Ezimweni eziningi ze-CMML, imbangela ayifakazi, futhi ayikho indlela eyaziwayo yokuyivimbela. Abanye abantu bathuthukisa i-CMML ngemuva kokuthola i- chemotherapy kanye nemisebe njengengxenye yokwelashwa kwabo komdlavuza. Kwezinye izimo, imizamo ingenziwa ukugwema izidakamizwa ze-chemotherapy ezingase ziholele ku-CMML, kodwa lezi zidakamizwa zingadingeka, ukwelashwa okusindisa impilo kwezinye izimo.

Ukuvama

Isimo esiqondile se-CMML asiziwa. Ngokuvamile, i-CMML akuyona umdlavuza ovamile kakhulu; Kodwa-ke, yilona uhlobo oluvame kakhulu lwe-disorder blood esigabeni salo, i-MDS / MPN, echazwe kabanzi ngezansi.

Izici

Ukuhlelwa kwe-CMML, njengokwamanye amagciwane amaningi egazi, kuye kwabuyekezwa eminyakeni edlule, kufana nokuthuthukiswa kokuqonda kwesayensi kangcono kwesifo.

Namuhla, i-CMML ibonakala njengenhlanganisela yezigaba ezimbili ezihlukene zezinkinga zegazi: i- myelodysplastic syndrome kanye neoplasm myeloproliferative .

• I-Myelodysplastic syndrome (i-MDS) yiqembu lezifo ezithinta ukukhiqizwa kwegazi okwejwayelekile emangeni. Ku-MDS, umnkantsha wamathambo ukhiqiza amangqamuzana angabonakali, ahlanzekile egazi okuthiwa aqhuma amaseli. Lezi zingqamuzana zokuqhuma zihluleka ukuvuthwa ngendlela efanele futhi azikwazi ukusebenza ezindimeni ezihlosiwe njengezakhi zegazi.
• I-neoplasm ye-Myeloproliferative (i-MPN) ibhekisela ezifweni lapho umongo we-bone wenza amangqamuzana amaningi egazi abomvu, amaplatelet, noma izinhlobo ezahlukene zamaseli ezimhlophe.

Abantu abane-CMML banamaseli angabonakali (ama-dysplastic) emangeni abo, futhi, ngakho, isikhathi eside, i-CMML ibhekwa njengoluhlobo lwe-myelodysplastic syndrome.

Kodwa-ke, abantu abane-CMML nabo banomthelela ngokweqile kwamangqamuzana egazi amhlophe, futhi isici esiyinhloko se-MDS sinamaseli ambalwa kakhulu egazini, ngakho-ke i-CMML ayizange ifaneleke kakhulu isigaba se-MDS.

Ama-neoplasms aseMyelodysplastic / myeloproliferative (MDS / MPN)

Ngenxa yalezi zizathu nezinye, isimiso sokuhlukanisa i-World Health Organization (WHO) sihlanganisa i-CMML esigabeni sayo sonke: MDS / MPN, njengoba kuboniswe ngezansi.

I-CMML ingenye yezinhlobo eziningi ze-MDS / MPN.

• I-myelomonocytic leukemia engapheli (CMML)
• I-atypical chronic myeloid leukemia (aCML), i- BCR-ABL1 -
• I-juvenile ye-myelomonocytic leukemia (JMML)

• I-MDS / MPN ene-ring sideroblasts ne-thrombocytosis (MDS / MPN-RS-T)
• I-MDS / MPN, ayikwazi ukuhlukaniswa

I-CMML yisifo esivame kakhulu kuleli qembu. Izifo ezingavamile kakhulu kuleli qembu ziyi-leyemia ye-mythroomonocytic ye-leyemia engavamile. Zonke lezi zifo ziveza amangqamuzana amaningi egazi angavamile.

Esikhathini esibuyekeziwe esakhishwe ngo-2016 yi-WHO, into ebizwa ngokuthi i-anemia ye-refractory ene-ring sideroblasts ehambisana ne-thrombocytosis ephawulekile (i-RARS-T) yasungulwa njengegama elihlonishwa ngokugcwele, elibizwa ngokuthi i-MDS / MPN nge-ringeroblasts ne-thrombocytosis.

Izimpawu Nezibonakaliso

Ukuba nama-monocyte amaningi kakhulu isikhathi eside, ngokuvamile, izinyanga ezintathu yilona uphawu oluvame kakhulu lwe-CMML.

Ukugqithisa kwama-monocytes ukusola izimpawu eziningi. I-monocytes eyengeziwe ingakwazi ukuhamba iphinde ibe nezinhlangothi ezimbili esiswini, isibindi, nesipere, lapho kungabangela ukwandiswa kanye nezimpawu ezithile.

• I- pleen ekhulisiwe , noma i-splenomegaly, ingabangela ubuhlungu engxenyeni engenhla kwesokunxele kwesisu. Ukwandiswa kule ndawo kungabangela abantu bazizwe begcwele ngokushesha kakhulu uma bedla.
• Uma isibindi sanda, esibizwa ngokuthi i-hepatomegaly, lokhu kungabangela ukungathandeki esiswini kanye nasenhla kwesokudla.

Ngisho noma abantu abane CMML benza amangqamuzana amhlophe egazi omhlophe amaningi, ngezinye izikhathi kungase kube nokuntuleka kwezinye izinhlobo zamaseli abomvu namhlophe njengengxenye yale nqubo yonke, futhi lokhu kunqongophala kungaveza nezinye izimpawu ze-CMML.

• Ukuntuleka kwamangqamuzana egazi abomvu, noma i-anemia, kungabangela ukuzwa ukhathele kakhulu, kanye nobuthakathaka, ukuphefumula, nokukhumba isikhumba.
• Izinombolo ezinganele zamangqamuzana egazi amhlophe ajwayelekile, noma i-leukopenia, zingaholela ezifweni ezivame kakhulu noma ezinzima.
• Ukuntuleka kwamaplatelet wegazi, noma i-thrombocytopenia, kungaholela ekuguleni okungavamile nokulimaza. Kumuntu onomdlavuza we-CMML, lokhu kungase kuqaphele njengezinkinga ze-noseblees ezinzima noma ezinzima noma izinsini eziphaphayo.

Ezinye izimpawu zingase zihlanganise ukulahlekelwa kwesisindo esingenasisekelo, umkhuhlane, nokulahlekelwa ukudla.

Izimpawu nezimpawu zingase zinikeze izinkomba, kodwa azanele ukuxilongwa kwe-CMML.

Ukuxilongwa Nokuhlola

Ukuxilongwa kwe-CMML kuhilela ucwaningo oluthile lwamaseli kusukela kokubili kwegazi lakho kanye nomnyo we-bone. Lokhu kusho ukuthi i- bone marrow biopsy iyingxenye yokuhlola, ngaphezu kwegazi elijwayele ukudweba kusuka emthanjeni usebenzisa inaliti.

Ngokusekelwe ekutholeni ukuhlolwa kokuqala, kukhona inqubo yokuqedwa okuqhubekayo, ngoba izifo eziningi ezihlukahlukene zingakhipha lezi zimpawu kanye nezibalo eziyisisekelo zokubhubhisa.

2016 I-WHO Inqubo Yokuthola Ukuhlonza I-CMML

1. Ukuphikelela (ngaphezulu kwezinyanga ezingu-3) ngokweqile kwe-monocytes (i-monocytosis) egazini legazi elingaphezu kuka-1000 / microL.
2. I-akhawunti ye-monocytes ngamaphesenti angaphezu kwezingu-10 we-whole white cell cell (WBC).
3. Akuhlangabezane ne-WHO yokuthola ezinye izinkinga zegazi: i-BCR-ABL1 enomsoco we-myeloid leukemia, i-myelofibrosis eyinhloko, i-polycythemia vera, noma i-thrombocytosis ebalulekile
4. Amaphesenti wezinhlobo ezingapheliyo ezivuthwa ngegazi nomnkantsha: ngaphansi kwamaphesenti angama-20 ama-myeloblasts + ama-monoblasts + ama-promonocytes ku-blood pipeline kanye nomnkantso wamathambo.
5. Izinguquko ze-Dysplastic (ubujamo obungavamile kanye nokubukeka ngaphansi kwe-microscope) emkhayeni owodwa noma ngaphezulu wezingqalasizinda zegazi "emndenini womndeni we-myeloid." Emndenini womndeni we-myeloid, "amagatsha" afaka imigqa yeselula ehlukanisa futhi evuthiwe ekugcineni ikhiqize ama-monocytes, ama-macrophages, ama-neutrophils, ama-basophil, ama-eosinophil, amangqamuzana egazi obomvu, amaseli e-dendritic, nama-megakaryocytes.

Izifo ezinokuthola okufana ne-CMML zivame ukukhishwa ngaphandle, futhi ngezinye izikhathi odokotela bazokwenza izivivinyo ezengeziwe eziphakanyisiwe ngaphambi kokwelashwa kweziguli ezine-CMML, kanye nokuphanga kokuqala, njengoba sebevele bethola umnkantsha wakho namasampuli egazi ukuze uhlolwe.

Uma ushintsho oluthile lwezakhi zofuzo noma ukulungiswa kabusha kwezakhi ezithile zezakhi zofuzo kutholakala emangqamuzaneni anzima (okuvamile kubantu abane-monocytosis, kodwa kufaka i-PDGFRA noma i-PDGFRB, i-FGFR1, noma i-PCM1-JAK2) bese kunesigaba esihlukile se-WHO esinikezwa, futhi kunezimpendulo zokwelapha nge-ejenti ethile ebizwa i-imatinib.

Izigaba ze-CMML

Isigaba se-WHO sisabalalisa abantu abane-CMML ngezigaba ezintathu ezahlukene ezihlobene nokubikezela:

• I-CMML-0: Amaphesenti angaphansi kwezingu-2 aqhuma egazini legazi futhi amaphesenti angaphansi kwamaphesenti angu-5 aphefumula emnkantso.
• I-CMML-1: amaphesenti angu-2 ukuya kwangu-4 aqhuma egazini legazi kanye / noma amaphesenti angu-5 kuya kwangu-9 emanzini.
• I-CMML-2: Amaphesenti angu-5 kuya kwangu-19 aqhuma egazini legazi, amaphesenti angu-10 kuya kwangu-19 aqhuma emnkantso, kanye / noma ukutholakala kwamakhodi ama-Auer amaningi (ama-clumps e-burgundy ezibonakalayo ezinemibala ehlanganiswayo, ebonakala ku-cytoplasm ukuqhuma kwemithi).

Ukwelapha

Ukufakelwa komnyoba wamathambo avela kumnikezi (i-allogeneic hematopoietic cell transplantation) yindlela yokwelashwa kuphela engase yenze ukwelashwa iziguli ezine-CMML. Uma kuyinketho, lesi sinqumo senziwa njengomphumela wesxoxo nodokotela-isiguli.

Izivivinyo zemitholampilo

Ngokuphathelene ne-chemotherapy, kuze kube manje, akukho "i-magic bullet ye-CMML" engatholakali. Izindlela zokwelashwa ezitholakalayo azithinti kakhulu ukuqhubeka kwemvelo yesifo, ngakho-ke abantu abane CMML bakhuthazwa kakhulu ukuba bacabangele ukubhalisa ekuvivinyweni komtholampilo, uma kutholakala.

Kubantu abangahambi ukufakelwa kabusha, nalabo abangabhalisi kulesi sivivinyo, kunezinketho eziningi zokwelapha lesi sifo, okuncane ukwelashwa, okufaka phakathi ukwelapha okuqondiswa uphawu.

Ukwelashwa Okuqondisiwe Nokuqondisa

Imihlahlandlela yocwaningo ithi iziguli ezingenazo izimpawu kufanele zihlolwe odokotela bazo ngokuhlolwa ngezikhathi ezithile kanye nezifundo zelabhorethri. Babuye batusa ukuthi ukugoma kuvuselelwe futhi uma isiguli sibhema, lokho kubhema kumile.

Uma kungekho ukuhlolwa komtholampilo, kusekhona izidakamizwa ezitholakalayo ezingase zisetshenziselwe "ukushaya" izinombolo ezidlulele zamangqamuzana angajwayelekile (i-cytoreductive therapy) njenge-hydroxyurea, i-azacitidine, noma i-decitabine.

Ukondla iziguli ezine-CMML kufana nalokho okwenzelwe iziguli ezine-MDS. Ukudluliselwa kwamaseli abomvu nama-platelet kuvame ukunikezwa, futhi ukusetshenziswa kwama-erythropoiesis-okuvuselela ama-agent kungacatshangwa, kanye nokwelashwa kwama-antibiotic kwezifo.

Ku-CMML engamangazayo, noma amacala lapho ukuzama ukuhlolwa khona kuye kodwa kwehlulekile, iziguli ziyakhuthazwa ukuba zihlanganyele ekuvivinyweni kwemitholampilo noma nini lapho zitholakala.

Isibikezelo

Ayikho isibalo esihle se-ballpark sokusinda, ngoba abantu abane-CMML bangase babe nakho okuhluke kakhulu nesifo.

I-median (noma, statistically, inombolo ephakathi kwenkambiso yezikhathi zokusinda) ingaba izinyanga ezingaba ngu-30 zisinda esikhathini sokuxilongwa. Kukhona ukuhlukahluka okubanzi kulezi zibalo, kodwa, futhi izibalo zokuphila, ngokuvamile, azibonakali izindlela ezintsha zokwelapha ezingekho idatha okwamanje. Ukubikezela kubhekwe ngokuningiliziwe ngezansi, uma kuchazwa amaqembu ahlukene we-CMML engozini.

Ukungaqiniseki ngezibalo kungenza uzizwe ukhathazekile. Ingxoxo evulekile nodokotela wakho izokusiza uqonde icala lakho ngabanye. Kunemiphumela eminingi ehlukahlukene kanye nezikhathi zokusinda nge-CMML, futhi kukhona okungenani izinhlelo eziyisishiyagalolunye zokufaka amagoli ukuzama ukusiza ukucacisa izinhlelo zokusebenzisa izibalo ezisebenzisa izinto ezifana nezici zemitholampilo kanye nokutholakala kwelabhutriki, kanti ezinye zihlaziya i-genetics yamangqamuzana anomdlavuza .

Kungaba khona umehluko omkhulu endleleni odokotela abenza ngayo ingozi ye-CMML, kuye ngokuthi isikhungo, kodwa akukho idatha ebonakala ibhekisela endleleni eyodwa yokuhlola ingozi.

Izibalo ezivela e-American Cancer Society manje ziyi-dated, kodwa zisiza ukukhombisa umehluko ngamagumbi we-CMML-1 no-CMML-2, futhi zibonisa ukuthi amanye amaqembu phakathi kwabantu bonke abonakala enza kangcono kunabanye.

Ucwaningo olulodwa lweziguli ze-CMML ezitholakale phakathi kuka-1975 no-2005, izikhathi zokuphila eziphakathi nendawo nge-CMML-1 no-CMML-2 zaziyizinyanga ezingu-20 nezinyanga ezingu-15, ngokulandelana. Nokho, ezinye iziguli zazihlala isikhathi eside. Amaphesenti angaba ngu-20 weziguli ze-CMML-1 kanti cishe amaphesenti angu-10 weziguli ze-CMML-2 zasinda isikhathi eside kuneminyaka emihlanu. Futhi, iziguli ezine-CMML-2 zingase ziqhubeke nokuthuthukisa i-leukemia elula kuneziguli ezine-CMML-1. Esikhathini esifundweni esifanayo, amaphesenti angu-18 weziguli ze-CMML-1 namaphesenti angu-63 weziguli ze-CMML-2 zenze i-acute myeloid leukemia engakapheli iminyaka emihlanu ye-CMML yokuxilongwa.

Izwi elivela

Kulowo muntu nge-CMML, ukubikezela kungancika ezintweni eziningi, futhi iminyaka yomuntu kanye nempilo yakhe yonke ibalulekile. Ngenxa yokuthi lesi sifo sithinta abantu abaningi asebekhulile abangase babe nezinye izifo ezingelapheki, ukwelashwa okunomthelela kunabo bonke kanye nokuguqulwa komnkantsha we-bone-okwamanje akuyona inketho.

Izindlela ezinhle zokusekela ziyatholakala, kodwa i-CMML yisifo esibonakala sisekupheleni kokutholakala okudinga ukwelapha. Njengalokhu, okushicilelwe izolo kungase kwenzeke noma kungabi yiqiniso kusasa, ngakho-ke bheka zonke izinketho zakho futhi ucabange ukubhalisa esilingo somtholampilo.

> Imithombo:

> Arber DA, Orazi A, Hasserjian R, et al. Ukubuyekezwa kuka-2016 ku-World Health Organisation ngezigaba ze-myeloid ne-acute leukemia. Igazi . 2016; 127: 2391-2405.

> Elena C, Gallì A, E-E, et al. Ukuhlanganisa izici zemitholampilo kanye nezilonda zofuzo ekuhloleni kwezinhlekelele zeziguli ezinomdlavuza we-myelomonocytic ongapheli. Igazi 2016; 128 (10): 1408-1417.

> Zeidan AM, Hu X, Long JB, et al. Ukusetshenziswa kwe-agent ye-hypomethylating kanye nokusinda ezigulini ezindala ezine-leelemia ye-myelomonocytic e-United States engapheli: Isifundo esikhulu esisekelwe kubantu. I-Cancer . 2017 Okthoba 1; 123 (19): 3754-3762.