Kurwara kwemaelomonocytic yeukemia, kana CMML, inomukenza wemasero-aita masero anogara mumusepa. MuCMML, kusakanganiswa mumasero eropa-kuumba hakungogumiri pfupa remapfupa-zvinokonzera munhu kuva nemoocytes akawanda-rudzi rwechichena cheropa-uye izvi zvinokanganisa zvimwe zvikamu zvemuviri zvakare.
CMML vs. CML
CMML inomira musingagumi myelomonocytic leukemia, asi CML inomiririra myeloid leukemia (iyo inonziwo isingagumi myelogenous leukemia).
Ikoko kunogona kufanana pakati pe CML ne CMML maererano nekutanga kuwanikwa, asi zvirwere zviviri izvi zvakasiyana.
Pakati pevaviri, CMML inowanzotadza kukura kupfuura CML, kunyange zvazvo zvinhu zvakasiyana-siyana zvinogona kuita kuti munhu agone kufambira mberi uye kupona uye kune mishonga inorapwa inowanikwa zvichienderana nemhosva.
Zvinokonzera uye Zviitiko Zvengozi
CMML inowanzoitika kuvanhu vakwegura, kazhinji vanhu vari pamusoro pemakore makumi matanhatu nemashanu.
Muzviitiko zvakawanda zve CMML, chikonzero hachizivikanwi, uye hapana nzira inozivikanwa yekuidzivirira. Vamwe vanhu vanogadzira CMML mushure mekugamuchira chemotherapy uye mazai echinhu chechikamu chekurapa kenza. Mune zvimwe zviitiko, kuedza kunogona kuitwa kudzivisa chemotherapy mishonga iyo inogona kutungamirira ku CMML, asi mishonga iyi inogona kudiwa, kurapa kuponesa mune dzimwe nguva.
Kuwanda
Icho chiitiko chaiyo che CMML hachizivikanwi. Zvose, CMML haisi kenza yakawanda kwazvo; zvisinei, iyo ndiyo inonyanyozivikanwa yeropa disorder mune chikamu chayo, MDS / MPN, iyo inotsanangurwa zvakare pasi apa.
Zviratidzo
Chikamu che CMML, chakafanana nemamwe makondombera akawanda eropa, chakave chichigadziridzwa pamusoro pemakore, zvakafanana nekuvandudzwa kwekunzwisisa kwesayenzi kwechirwere ichi.
Nhasi, CMML inoratidzika seine marongerwo emamiriro ezvinhu maviri akasiyana-siyana ematambudziko eropa: myelodysplastic syndrome uye myeloproliferative neoplasm .
- Myelodysplastic syndrome (MDS) iboka rezvirwere zvinokanganisa kushandiswa kwemasero eropa mumapfupa emapfupa. Mu MDS, marora emapfupa anounza zvisingaoneki-zvinotarisa, zvisingatauri masero eropa anonzi kuputika masero. Izvi zvinokonzera masero zvinokundikana kukura zvakakwana uye hazvikwanise kushanda mune zvavanoda kuita semasero eropa.
- Myeloproliferative neoplasm (MPN) inoreva zvirwere umo mafuta emapfupa anoita masero matsvuku eropa, maplatelets, kana marudzi akasiyana emasero machena.
Vanhu vane CMML vane masero asingatarisi (dysplastic) mumasese avo, uye, saka, kwenguva yakareba, CMML yaionekwa sechimiro chemyelodysplastic syndrome.
Zvisinei, vanhu vane CMML vanewo kuwandisa kwemasero matsvuku eropa, uye chimwe chinhu chinokosha cheMDC chinenge chiine masero mashoma eropa muropa, saka CMML haina kumbobvira yakanyatsokodzera boka reMDC.
Myelodysplastic / myeloproliferative neoplasms (MDS / MPN)
Nokuda kweizvi uye zvimwe, zvikonzero zveWorld Health Organization (WHO) zvinosanganisira CMML mune rumwe rutivi rwayo: MDS / MPN, sezvinoratidzwa pasi apa.
CMML ndeimwe yemhando dzakawanda dzeMDC / MPN.
- Chronic myelomonocytic leukemia (CMML)
- Atypical chronic myeloid leukemia (aCML), BCR-ABL1 -
- Juvenile myelomonocytic leukemia (JMML)
- MDS / MPN ine ring ringoblasts uye thrombocytosis (MDS / MPN-RS-T)
- MDS / MPN, isingazivikanwi
CMML ndiyo chirwere chinowanzoonekwa muboka iri. Zvirwere zvishoma zvishoma muboka iri ndezvokuti atypical chronic myeloid leukemia nevana vangu myelomonocytic leukemia. Zvose izvi zvirwere zvinokonzera masero akawanda eropa.
Mune zvinyorwa zvakabudiswa muna 2016 neWIV, chimwe chinhu chaimbova chinonzi refractory anemia nechende sideroblasts chakabatanidzwa nekaratidzirwa thrombocytosis (RARS-T) yakatangwa seyakagamuchirwa, nguva itsva yekuongorora, inonzi MDS / MPN neine ringeroblasts uye thrombocytosis.
Zviratidzo nezviratidzo
Kuva nemonocytes akawanda kwazvo kwenguva yakareba, kazhinji, mwedzi mitatu ndicho chiratidzo chinowanzoonekwa che CMML.
Kuwedzerwa kwemamonocytes ndiko kukanganisa kwezviratidzo zvakawanda. Mamwe maoocytes anogona kufamba uye kupinda mukati memimwe nhengo mbiri mumudumbu, chiropa, uye spleen, paanokwanisa kukonzera kukura uye zvimwe zviratidzo.
- Nyoka yakakura , kana kuti splenomegaly, inogona kukonzera marwadzo kumusoro kwekumucheto chikamu chemuviri. Kuwedzera munharaunda iyi kunogona kukonzerawo kuti vanhu vanzwe vakakwana zvakanyanya pavanodya.
- Kana chiropa chikawedzera, icho chinonzi hepatomegaly, izvi zvinogona kukonzera kusuruvara mumimba zvakare, kumusoro kwepamusoro.
Kunyange zvazvo vanhu vane CMML vachiita masero machena akawanda emonocyte, pane dzimwe nguva kungave nekushayikwa kwemamwe marudzi emasero matsvuku uye machena sechikamu chebasa iri rose, uye kushayikwa uku kunogonawo kubudisa zvimwe zviratidzo zve CMML.
- Kushayikwa kwemasero matsvuku eropa, kana kuperevedza ropa, kunogona kuita kuti uchinzwe wakaneta kwazvo, uyewo utera, kupera kwepfufu, uye ganda rinopera.
- Nhamba dzisina kukwana dzemasero matsvuku eropa, kana kuti leukopenia, dzinogona kutungamirira kune zvirwere zvinowanzoitika kana zvakanyanya.
- Kushayikwa kweplatelet yeropa, kana thrombocytopenia, kunogona kutungamirira kubuda ropa kusingawanisi uye kupwanya. Mune munhu ane CMML, izvi zvinogona kuonekwa sekugara kwenguva refu kana kwakanyanya nosebleeds kana kuti kubuda ropa.
Zvimwe zviratidzo zvinogona kusanganisira kusarirwa kwekurema kwechiremera, fivha, uye kurasikirwa kwechido.
Zviratidzo uye zviratidzo zvinogona kupa zvinyorwa, asi hazvina kukwana kuongororwa kwe CMML.
Kuongorora uye Kuongorora
Kuongororwa kwe CMML kunosanganisira kuongororwa kwakaitwa masero kubva kumaropa ako uye marora. Izvi zvinoreva kuti mapfupa emapfupa anonyanya chikamu chekuongororwa, kuwedzera kune ropa rinowanzozivikanwa rinotora kubva mumupfuva uchishandisa nete.
Kubva pane zvakawanikwa pakuedzwa kwekutanga, kunewo nzira yekuparadzanisa inopfuurira, sezvo zvirwere zvakawanda zvakasiyana zvinogona kubudisa zviratidzo izvi uye zvakanyanyisa mab laboratory zvakawanikwa.
2016 WHO Criteria CMML Kuziva
- Kuramba (kwemwedzi inopfuura 3) kudarika kwe monocytes (monocytosis) muropa reropa rinopfuura 1000 / microL.
- Monocytes account yakawanda kupfuura 10 muzana ye yese white white cell (WBC) yakasiyana.
- Kwete kusangana neE WHO yezvimwe zvinetso zveropa: BCR-ABL1 chitubu chine myeloid leukemia, primary myelofibrosis, polycythemia vera, kana thrombocytosis inokosha
- Percent of immature blast types in blood and marrow: under 20% myeloblasts + monoblasts + promonocytes muhutachipa ropa uye marora.
- Dysplastic change (zvisingaoneki maumbirwo uye maitiro pasi pe microscope) mune imwe kana mhuri dzakawanda dzinokura masero eropa mu "mumhuri myeloid." Mumhuri yemamiedidhi, "matavi" anosanganisira masero emagetsi anoparadzanisa uye anokura pakupedzisira kugadzira monocytes, macrophages, neutrophils, basophils, eosinophils, masero matsvuku eropa, masero dendritic, uye megakaryocytes.
Zvirwere nezvakawanikwa zvakafanana neCMML zvinowanzotarisirwa kunze, uye dzimwe nguva vanachiremba vachaita mimwe miedzo inotarisirwa kusati yatanga kurapwa nevarwere vane CMML, pamwe chete nekugadzirisa kwekutanga, sezvo vari kutotora fupa rako remafupa uye ropa samples kuti uongororwe.
Kana mamwe mazai ezvemagetsi akashandurwa kana kugadziriswa kwemashoko ega ega anowanikwa mumasero ane dambudziko (zvisingawanzoonekwa nevanhu vane monocytosis, asi vanosanganisira PDGFRA kana PDGFRB, FGFR1, kana PCM1-JAK2) zvino pane kusiyana kwakasiyana-siyana kwe WHO, uye kune zvinoreva kurapwa pamwe nemumwe muenzi anonzi imatinib.
CMML Zvikamu
Sangano re WHO rinowedzerawo vanhu vane CMML mumapoka matatu akasiyana anoenderana nekufananidzira:
- CMML-0: Pasi pema2 peresenti inoputika muropa rega roga uye pasi pe5 muzana inoputika mumusi.
- CMML-1: 2 kusvika ku4 muzana inoputika muropa reropa uye / kana 5 kusvika 9 muzana inoputika mumusi.
- CMML-2: 5 kusvika ku19 muzana inoputika muropa remhepo, 10 kusvika ku19 muzana inoputika mumusana, uye / kana kuvapo kweimwe kana kupfuura mamwe mazwi anonzi Auer (clumps ye burgundy yemifananidzo yakawanda yakaumbwa yakaumbwa, yakaonekwa mu cytoplasm ye myeloid blasts).
Kurapa
Pfupa remapfupa kubva kune anopa (allogeneic hematopoietic cell transplantation) ndiyo chete inogona kurapa kurapa kune varwere vane CMML. Kana iri chisarudzo, chisarudzo ichi chinoitwa semugumisiro wehurukuro yachiremba-murwere.
Clinical Trials
Mukureva chemotherapy, kusvika ikozvino, hapana "magic bullet ye CMML" ichigere kuwanikwa. Mishonga inowanikwa haibatsiri zvakanyanya kufambira mberi kwechirwere chechirwere ichi, uye saka vanhu vane CMML vanokurudzirwa zvakasimba kuti vaone kunyora muchipatara miedzo, kana iripo.
Kune vanhu vasingaendi kunyorera, uye kune avo vasingabvumi munjodzi, kune nzira dzakasiyana-siyana dzokurapa chirwere, kupfupika kwechirwere, kusanganisira zviratidzo-zvinorapwa.
Chirwere-Kutungamirirwa uye Kutsigira Therapy
Nyanzvi dzemitemo dzinotaura kuti varwere vasina zviratidzo vanofanira kuongororwa navanachiremba vavo vane nguva dzekuongorora uye mapurogiramu ekuongorora. Vanokurudzirawo kuti majekiseni agadziriswe uye kana murwere ari kusvuta, kuti kusvuta kunomira.
Kana pasina kliniki yekuongorora, kune zvimwe zvinodhaka zvinowanikwa zvinogona kushandiswa "kuputsa" nhamba dzakawandisa dzemasero asina kukodzera (cytoreductive therapy) akadai se hydroxyurea, azacitidine, kana decitabine.
Kutsigira kuchengetedza varwere vane CMML kwakafanana nezvakaitwa kune varwere vane MDS. Kudhindwa kwemasero matsvuku nemaplatelet kunowanzopiwa, uye kushandiswa kwema erythropoiesis-kukurudzira maitiro kunogona kufungwa, pamwe chete nemishonga inorwisa mabhakitiriya yezvirwere.
Nokuda kwekunyengedza CMML, kana zviitiko apo kurapwa kwakaedzwa asi kwakakundikana, varwere vanokurudzirwa kutora chikamu mumakiriniki emakirini chero pavanenge varipo.
Prognosis
Hapakanaka bhoppark chimiro chekupona, sezvo vanhu vane CMML vangave vane zviitiko zvakasiyana zvakasiyana nehosha.
A yakabudiswa median (kana, nhamba, nhamba yepakati mumutsara wenguva yekupona) inenge mamiriyoni makumi matatu ekupona kubva panguva yekuongororwa. Kune kusiyana kwakasiyana-siyana munharaunda iyi, kunyange zvakadaro, uye nhamba dzehupenyu, kazhinji, hazviratidze mushonga mutsva uyo usina data zvakare. Zviratidzo zvinotaridzirwa zvakanyanyisa pasi apa, kana majekiseni akasiyana-siyana e CMML achitsanangurwa.
Kuvhiringidzika pamusoro pezvikwereti kunogona kuita kuti unzwe uchinetseka. Kukurukurirana pachena nechiremba wako kuchakubatsira kuti unzwisise nyaya yako pachako. Kune mhando dzakasiyana dzemigumisiro uye nguva yekupfuura ne CMML, uye kune zvingasvika zvipfumbamwe zvakasiyana-siyana zvirongwa zvekuedza kubatsira kubatsira kutsvaga-masangano anoshandisa zvinhu zvakadai semakiriniki nezviwanikwa zve laboratori, uye vamwe vanonzverawo genetics yemasero anokonzera .
Ikoko kunogona kuve nekusiyana kukuru munzira iyo vanachiremba vanoenda kune ngozi yeM CMML, zvichienderana nesangano, asi hapana dhesi inoonekwa seyo inoratidza imwe nzira yakanakisisa yekuongorora njodzi.
Nhamba kubva kuAmerican Cancer Society iye zvino yava dated, zvisinei, dzinobatsira kuratidza kusawirirana ne CMML-1 uye CMML-2 zvikamu, uye dzinoratidzawo kuti mamwe mapoka mukati mehuwandu hwevanhu vose anoita seanoita zviri nani pane mamwe.
Mune rumwe ruzivo rweM CMML varwere vakaonekwa pakati pe1985 na2005, nguva dzekugara kwenguva refu neM CMML-1 uye CMML-2 dzaive mwedzi makumi maviri nemwedzi gumi nemashanu, zvichienderana. Zvisinei, vamwe varwere vakararama kwenguva refu. Inenge 20 muzana ye CMML-1 varwere uye vanenge 10 muzana ye CMML-2 varwere vakapona kwenguva refu kupfuura makore mashanu. Uyezve, varwere vane CMML-2 vane mikana yekuenderera mberi kukura maukemia akaoma kupfuura varwere vane CMML-1. Muchidzidzo chakafanana, 18 muzana ye CMML-1 varwere uye 63 muzana ye CMML-2 varwere vakagadzira myeloid yeukemia yakareba mumakore mashanu e CMML kuongororwa.
Shoko Rinobva
Kune mumwe munhu ane CMML, kufungidzira kunogona kuvimba pane zvakawanda zvakasiyana, uye munhu wezera uye hutano hwakakwana hunokosha. Nokuti chirwere ichi chinowanzokonzera vanhu vakawanda vakwegura vanogona kunge vane zvimwe zvirwere zvisingaperi, kurapwa kunosuruvara uye imwe iyo inogona kugadzirisa-bone marrow transplant-haisi nguva dzose.
Nhanho dzakanaka dzinotsigira dzinowanika, asi CMML chirwere chinoratidzika chiri pamucheto wezvinodiwa kurapa kurapa. Sezvo, izvo zvakadhindwa zuro zvingave kana zvisingasvike mangwana, saka tarisa pane zvese zvaunogona kuita uye funga kunyora muchipatara chekuedza.
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> Elena C, Gallì A, Such E, et al. Kubatanidza makiriniki ehutachiona uye zvipembenene zvine maitiro muhuongorori hwehutachiona hwevarwere vane chirwere chisingaperi chine myelomonocytic leukemia. Ropa 2016; 128 (10): 1408-1417.
> Zeidan AM, Hu X, Long JB, et al. Hypomethylating agent therapy kushandiswa uye kupona kune vakwegura vakura vane chirwere chisingaperi chine myelomonocytic leukemia muUnited States: Chidzidzo chikuru chevanhu. Cancer . 2017 Oct 1; 123 (19): 3754-3762.