I-Myelomonocytic Leukemia (CMML)

I-myelomonocytic leukemia, okanye i-CMML, ngumhlaza weeseli ezenziwe ngegazi ezihlala emnzini wethambo. Kwi-CMML, ukungaqhelekanga kwiiseli ezenziwe ngegazi akuchaphazeli nje kuphela umongo wentaba-bangela ukuba umntu abe ne-monocytes ezininzi-uhlobo lwe-white cell cell-kwaye oku kuyachaphazela nezinye iindawo zomzimba ngokunjalo.

CMML no CML

I-CMML imela i-myelomonocytic leukemia engapheliyo, ngelixa i-CML imele i- leukemia engapheliyo (ebizwa ngokuba yi-leukemia engapheliyo).

Kukho ukufana phakathi kwe-CML kunye ne-CMML ngokokufunyaniswa kokuqala, kodwa ezi zifo zibini zihluke.

Kule mibini, i-CMML ngokubanzi ihamba ngokugqithiseleyo kunokuba i-CML, nangona iimeko ezininzi ezahlukeneyo zinokufaka isandla kwisigxina somntu kunye nokusinda kwaye kunokukho unyango lwezokwelapha olufumanekayo kuxhomekeke kwimeko ethile.

Izizathu kunye neengozi

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

Kwiimeko ezininzi ze-CMML, isizathu asiyazi, kwaye akukho ndlela eyaziwayo yokukhusela. Abanye abantu bahlakulela i-CMML emva kokufumana i- chemotherapy kunye ne- radiation njengenxalenye yonyango lwabo lomhlaza. Kwezinye iimeko, iinzame zenziwa ukuba ziphephe iziyobisi zechemotherapy ezinokuthi zikhokelele kwi-CMML, kodwa ezi ngonyango zingadingeka, unyango olusindisa ubomi kwezinye iimeko.

Ukukhula

Isiganeko ngqo se-CMML asiyazi. Ngokubanzi, i-CMML ayiyona umdlavuza oqhelekileyo; Nangona kunjalo, luhlobo oluqhelekileyo lweengxaki zegazi kwicandelo lawo, i-MDS / MPN, echazwe ngaphezulu ngezantsi.

Iimbonakalo

Ukwahlulelwa kwe-CMML, njengaleyo eminye imihlaza yegazi, sele ihlaziywa ngaphaya kweminyaka, ngokuhambelana nokuphuhliswa kwengqondo engcono yesayensi.

Namhlanje, i-CMML ibonwa njengenomxube weempawu ezimbini ezihlukeneyo zeengxaki zegazi: i- myelodysplastic syndrome kunye neoplomi ye-myeloproliferative .

• I-Myelodysplastic syndrome (MDS) liqela lezifo ezichaphazela ukuveliswa kweeselfowuni yegazi kwisalathiso leso. Kwi-MDS, umnatha wethambo uvelisa amangqamuzana angabonakaliyo, ajikeleza igazi elibizwa ngokuba yi-cell cells. Ezi ziqhumane zomoya ziyahluleka ukuvuthwa kakuhle kwaye azikwazi ukusebenza kwiinjongo ezijoliswe kuzo njengegazi zeeseli.
• I-Myeloproliferative neoplasm (MPN) ibhekisela kwizifo apho umongo wethambo wenza amangqamuzana amaninzi obomvu, iiplatelet, okanye iintlobo ezahlukeneyo zeeseli zegazi ezimhlophe.

Abantu abane-CMML banamaseli angabonakaliyo (dysplastic) kwiibokhwe zabo zethambo, kwaye ngoko, ixesha elide, i-CMML ithathwa njengoluhlobo lwe-myelodysplastic syndrome.

Nangona kunjalo, abantu abane-CMML banokugqithisa okwedlula amangqamuzana egazi lamhlophe, kunye nenqaku eliphambili le-MDS linamaseli ambalwa kakhulu egazini, ngoko ke i-CMML ayizange ibe yinto efanelekileyo kakhulu kudidi lwe-MDS.

I-Myelodysplastic / imyeloproliferative neoplasms (MDS / MPN)

Ngenxa yezi zizathu nezizathu, iNkqubo yeMpilo yeHlabathi ye-World (I-WHO) iquka i-CMML kwicandelo layo yonke into: i-MDS / MPN, njengoko kuboniswe ngezantsi.

I-CMML yenye yeendidi ezininzi ze-MDS / MPN.

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

• MDS / MPN kunye ne-sideroblasts kunye ne-thrombocytosis (MDS / MPN-RS-T)
• MDS / MPN, engenakucatshulwa

I-CMML yisona sifo esiqhelekileyo kweli qela. Izifo eziqhelekileyo kangako kweli qela ziyi-leyemia ye- leyemia ye-atypical engapheliyo kunye ne-juvenile ye-myelomonocytic leukemia. Zonke ezi zifo zivelisa ezininzi zegazi ezingaqhelekanga.

Kuhlaziyo olukhutshwe ngo-2016 ngu-WHO, into eyayibizwa ngokuba yi-anemia yokuphazamiseka kunye ne-sideroblasts engxowaniswe kunye ne-thrombocytosis ephawulweyo (RARS-T) yasungulwa njengexesha elipheleleyo, i-diagnostic term, ebizwa ngokuba yi-MDS / MPN kunye ne-sideroblasts kunye ne-thrombocytosis.

Izimpawu kunye neMpawu

Ukuba ne-monocytes ezininzi kwixesha elide, ngokuqhelekileyo, iinyanga ezintathu zibonakaliso oluqhelekileyo lwe-CMML.

Ukugqitha kwee-monocytes kukugweba ezininzi iimpawu. I-monocytes eyongezelelweyo ihamba kwaye ihlale kwizitho zimbini kwisisu, isibindi, kunye nebhenda, apho zingabangela ukwandiswa kunye neempawu ezithile.

• Ipenti ekhulisiweyo , okanye i-splenomegaly, ingabangela intlungu kwi-upper part of the belly. Ukwandiswa kule ndawo kungabangela ukuba abantu bazive bezele ngokukhawuleza xa besidla.
• Ukuba isibindi sandisiwe, esibizwa ngokuba yi-hepatomegaly, oku kungabangela ukungathandeki kwisisu nangasentla.

Nangona abantu abaneCMML benza amaninzi e-white monocyte egazi, ngamanye amaxesha kukho ukungabikho kweminye iindidi zeebomvu ezibomvu kunye ezimhlophe njengenxalenye yale nkqubo yonke, kwaye le ntlupheko inokuvelisa ezinye iimpawu ze-CMML.

• Ukunqongophala kweeseli ezibomvu zegazi, okanye i-anemia, kunokukhokelela ekubeni uzive ukhathele kakhulu, kunye nobuthathaka, ukuphefumula okufutshane kunye nebala eliphaphakileyo.
• Inani elaneleyo leeseli ezimhlophe zegazi eliqhelekileyo, okanye i-leukopenia, kunokukhokelela kwizifo eziqhelekileyo okanye ezinzulu.
• Ukunqongophala kweeplatelet zegazi, okanye i-thrombocytopenia, kunokukhokelela ekuphumeni okungaqhelekanga kunye nokutyumla. Kwimeko yomntu onomdla we-CMML, oku kunokuthi kuqaphele njengeziphunga ezinomdla okanye ezigulisayo.

Ezinye iimpawu zingabandakanya ukulahlekelwa kwesisindo esingenasenzo, umkhuhlane kunye nokulahleka kwesidlo.

Iimpawu kunye neempawu zingabonisa izikhombisi, kodwa azinako ukuxilongwa kwe-CMML.

Ukuxilongwa nokuHlola

Ukuxilongwa kwe-CMML kuqukwa uphando olukhethekileyo lweeseli ukusuka kwimbali yakho yegazi kunye namathambo. Oku kuthetha ithambo le-marrow biopsy inxalenye yolu vavanyo, ngaphezu kwegazi eliqhelekileyo lithatha kwi-vein isebenzisa inaliti.

Ngokusekelwe kwiziphumo zokuvavanywa kokuqala, kukho inqubo yokuqedwa okuqhubekayo, ekubeni izifo ezininzi ezahlukeneyo zingavelisa le mpawu kunye neziphumo zebhubhoratri ezisisiseko.

Ngo-2016 Ngubani na umgaqo we-CMML Ukuxilongwa

1. Ukuqhubeka (ngaphezulu kweenyanga ezi-3) ngaphezulu kwama-monocytes (monocytosis) egazini legazi elingaphezulu kwe-1000 / microL.
2. I-akhawunti ye-Monocytes ibe ngaphezu kweepesenti ezili-10 ze-cell white (WBC).
3. Ayihlangabezane ne-WHO yezinye iinkathazo zegazi: i-BCR-ABL1 enomdlavuza we-myeloid, i-myelofibrosis ephambili, i-polycythemia vera, okanye i-thrombocytosis ebalulekileyo
4. Ipesenti zeentlobo ezingapheliyo zegazi kunye nomongo: ezingaphantsi kwama-20 ekhulwini i-myeloblasts + i-monoblasts + i-promonocytes egazini legazi kunye nomnatha wethambo.
5. Utshintsho lwamaDysplastiki (imilo engaqhelekanga kunye nemibonakalo phantsi kwe-microscope) kwintsapho enye okanye ngaphezulu kwimizimba yamanzi egazini "emthonjeni weentsapho." Kwimithi yentsapho edibeneyo, "amasebe" afaka imizila yeselwana eyahlula kwaye ikhulile ekugqibeleni ivelise i-monocytes, i-macrophages, i-neutrophils, i-basophil, i-eosinophil, iiseli zegazi ezibomvu, iiseli ze-dendritic kunye ne-megakaryocytes.

Izifo ezinokufumana iziphumo ezifana ne-CMML kufuneka zikhuphe ngaphandle, kwaye ngamanye amaxesha oogqirha baya kwenza iimvavanyo ezongezelelweyo ezicetyiswayo ngaphambi kokonyango lwezigulane ezine-CMML, kunye ne-initial repupation, kuba sele sele bethola umongo wakho weesalathisi kunye neesampula zegazi zokuxilongwa.

Ukuba ukuguqulwa kwezinto ezithile zofuzo okanye ukulungiswa kwakhona kwezilwanyana ezithile kufumaneka kwiiseli ezinengxaki (ezingaqhelekanga kubantu abane-monocytosis, kodwa zibandakanya i-PDGFRA okanye i-PDGFRB, i-FGFR1, okanye i-PCM1-JAK2) kwaye kukho uluhlu oluhlukeneyo lwe-WHO, kwaye kukho iingxaki zokonyango kunye ne-arhente ethile ebizwa i-imatinib.

Iimpawu zeCMML

Udidi lwe-WHO lukwahlula ngakumbi abantu abane-CMML kwiindidi ezintathu ezahlukeneyo ezihambelana nokuxela:

• I-CMML-0: Iingaphantsi kweepesenti ezi-2 ziphazamiseka kwigazi le-peripheral kwaye zingaphantsi kwama-5 eepesenti ezidlulayo emlonyeni.
• I-CMML-1: i- 2 ukuya kwi-4 ekhulwini igalelo kwigazi le-peripheral kunye / okanye i-5 ukuya kwe-9 ekhulwini ephezulu.
• I-CMML-2: i- 5 ukuya kwi-19 iphesenti yegazi i-peripheral, i-10 ukuya kwi-19 ekhulwini iqhutywe emnzini, kunye / okanye ubukho bomntu omnye okanye ngaphezulu kwee-Auer (ii-clumps ze-burgundy ezibonakalayo ezinobumba obubunjiweyo obunemibala ehlukeneyo, ebonwa kwi-cytoplasm myeloid blasts).

Unyango

Ukutsalwa komongo we-bone (i-allogeneic hematopoietic cell transplantation) yonyango kuphela yonyango olulindelekileyo lwezigulane ezine-CMML. Xa kukho ukhetho, esi sigqibo senziwa njengesiphumo sengxoxo yengcali.

Uvavanyo lweZliniki

Ngokwe-chemotherapy, ngoku kude kubekho, akukho "magic bullet kwi-CMML" ayitholakali. Uphulo olukhoyo aluchaphazeli kakhulu ukuqhubela phambili kweso sifo, ngoko ke abantu abane-CMML bakhuthazwa kakhulu ukuba baqwalasele ukubhalisa kwizilingo zeclini, xa zikhoyo.

Kubantu abangenayo ukutshintshwa, kunye nalabo abangabhalisi kwilingo, kukho iindlela ezininzi zokunyanga ukugula, okufutshane kwiphilisi, kuquka ne-cure-directed therapy.

Utyando lwe-Symptom-Guideline and Supportive Treatment

Izikhokelo zengcebiso zithi izigulane ezingenazo iimpawu zimele zihlolwe oogqirha babo ngokuhlolwa kwexesha kunye nezifundo zebhubhoratri. Bakhuthaza ukuba amagonyo ahlaziywe kwaye xa isigulane sibhema, ukuba ukutshaya kugqityiwe.

Ngaphandle kokuvavanywa kwetlinikiyo, kusekho izixhobo ezikhoyo ezinokusetyenziswa "ukubetha" iinombolo ezigqithisileyo zeeseli ezingaqhelekanga (i-cytoreductive therapy) njenge-hydroxyurea, i-azacitidine, okanye i-decitabine.

Ukunyamekela izigulane ezine-CMML kufana noko kwenziwa kwiigulane ezine-MDS. Ukutshintshwa kweeseli ezibomvu kunye neeplatelets zihlala zinikwa, kwaye ukusetyenziswa kwee-erythropoiesis-stimulating agents kungabonwa, kwakunye neyeza zokulwa nezifo.

Kwi-CMML yokukhangela, okanye amatyala apho kukho ukuzama ukunyanga kodwa aphumelelanga, izigulane zikhuthazwa ukuba zithathe inxaxheba kwizilingo zeclini xa zifumaneka.

Prognosis

Akukho nhlobo efanelekileyo ye-ballpark yokusinda, kuba abantu abane-CMML banokuba namava ahluke kakhulu nesifo.

Umpapasho (okanye, ngokulandelelanisa, inombolo ephakathi kwinqanaba lexesha lokusinda) lihlala malunga neenyanga ezingama-30 ukususela ngexesha lokuxilongwa. Kukho uguquko olubanzi kule miba, nangona kunjalo, kunye nenani lokuphila, ngokubanzi, alubonakali unyango olutsha olungenakho idatha okwamanje. Ukuxhamla ingqwalasela kuthethwa ngcaciso engezantsi, emva kokuba kuchazwe amaqela achaphazelekayo e-CMML.

Ukungaqiniseki malunga neenombolo kungenza uzive uxhalabile. Ingxoxo evulekileyo nodokotela wakho iya kunceda uqonde imeko yakho. Kukho uluhlu olubanzi lweziphumo kunye namaxesha okuphila kunye ne-CMML, kwaye kukho ubuncinane iinkqubo ezili-9 zokufaka amanqaku ahlukeneyo ukuzama ukukunceda ukucacisa iindlela zokusebenzisa izixhobo ezifana neziphumo zeklinikhi kunye neziphumo zebhubhoratri, kwaye ezinye zihlalutya i-genetics yeseli .

Kukho ukungafani okukhulu kwindlela iindlela oogqirha bezisebenzisa ngayo ingozi ye-CMML, kuxhomekeke kwinqanaba, kodwa akukho datha ebonakala ingabhekisela kwindlela enye efanelekileyo yokuhlola umngcipheko.

Izibalo ezivela kwi-American Cancer Society ngoku zikho, kodwa, zinceda ukubonisa ukungafani ngeentlobo ze-CMML-1 kunye ne-CMML-2, kwaye zibonisa indlela amaqela athile kuwo wonke umntu abonakala enza ngcono kunabanye.

Kuhlolisiso olulodwa lwezigulane ze-CMML ezifunyanwe phakathi kuka-1975 no-2005, amaxesha okuphila okuphakathi kunye ne-CMML-1 kunye ne-CMML-2 yayineenyanga ezingama-20 kunye neenyanga ezili-15, ngokulandelana. Nangona kunjalo, ezinye izigulane zazihlala ixesha elide. Amaphesenti angama-20 e-CMML-1 yezigulane kwaye malunga neepesenti ezili-10 zezigulane ze-CMML-2 zasinda ixesha elingaphezu kweminyaka emihlanu. Kwakhona, izigulane ezine-CMML-2 ziyakwazi ukuqhubeka nokuhlakulela i-leukemia ephezulu kunezigulane ezine-CMML-1. Kwisifundo esifanayo, iipesenti ezingama-18 zezigulane ze-CMML-1 kunye neepesenti ezingama-63 zezigulane ze-CMML-2 zavelisa i-leukemia ekhangelekayo emilonyeni kwiminyaka emihlanu ye-CMML yokuxilongwa.

ILizwi

Ngomntu ngamnye nge-CMML, ukuxela kwangaphambili kunokuxhomekeka kwimibandela eyahlukeneyo, kwaye ubudala bomntu kunye nempilo yonke ibalulekile. Ngenxa yokuba esi sifo sithintela abantu abaninzi asebekhulile abanokuba nezinye izifo ezingapheliyo, unyango olunefuthe kunye nolunye olunokuthi lusetshentshile-umthambo wokutshintsha umongo-akusoloko lukhethwa.

Amanyathelo omncedisayo afumaneka, kodwa i-CMML isifo esibonakala siphezu kwe-diagnostic efunekayo. Ngaloo nto, oko kwanyatheliswa izolo kungenokwenzeka okanye kungeke kube yinyani ngomso, ngoko jonga zonke iinketho zakho uze ucinge ukubhalisa kwilingo leklinikhi.

> Imithombo:

> Arber DA, Orazi A, Hasserjian R, et al. Ukuhlaziywa kwe-2016 kwi-World Health Organisation ye-World Health Organisation ye-semoid neoplasms kunye ne-acute leukemia. Igazi . 2016; 127: 2391-2405.

> Elena C, Gallì A, Eyo E, et al. Ukudibanisa iimpawu zekliniki kunye nezilonda zezofuzo ekuhloleni ingozi kwizigulane ezine-leelemia. Igazi 2016; 128 (10): 1408-1417.

> Zeidan AM, Hu X, Long JB, et al. Ukusetyenziswa kwonyango ye-hypomethylating kunye nokusinda kwizigulane ezithe zakuba ne-myelomonocytic leukemia e-United States: Isifundo esiphezulu sabantu. Cancer . 2017 Oktobha 1; 123 (19): 3754-3762.