Matenda a Myelomonocytic Leukemia (CMML)

Matenda a khansa ya myelomonocytic, kapena CMML, ndi khansara ya maselo opanga magazi amene amapezeka m'mafupa. Mu CMML, zoperewera m'maselo opanga magazi sizimangokhudza mafupa-zimapangitsa munthu kukhala ndi monocyte kwambiri-mtundu wa selo loyera-ndipo izi zimakhudza ziwalo zina za thupi.

CMML vs. CML

CMML imayimira matenda a khansa ya myelomonocytic, pamene CML imayimika matenda aakulu a myeloid leukemia (omwe amatchedwanso kuti matenda aakulu a m'magazi).

Zikhoza kufanana pakati pa CML ndi CMML malinga ndi zofufuza zoyamba, koma matenda awiriwa ndi osiyana.

Mmodzi mwa awiriwo, CMML imakhala yowonongeka kwambiri kuposa CML, ngakhale kuti pali zinthu zambiri zomwe zingapangitse kuti munthu adziwe momwe angakhalire ndi kupulumuka ndipo pakhoza kukhala chithandizo chamankhwala kuchipeza malinga ndi zomwe zilipo.

Zifukwa ndi Zoopsa

CMML imapezeka makamaka akuluakulu, kawirikawiri anthu omwe ali pamwamba pa zaka 65.

Nthawi zambiri CMML, chifukwa chake sichidziwika, ndipo palibe njira yodziwikiratu. Anthu ena amapanga CMML atalandira mankhwala a chemotherapy ndi ma radiation monga gawo la mankhwala awo. Nthawi zina, amayesetsanso kupeŵa mankhwala a chemotherapy amene angathe kutsogolera CMML, koma mankhwalawa amafunikira, mankhwala opulumutsa moyo nthawi zina.

Kukula

Zochitika zenizeni za CMML sizidziwika. Konseko, CMML si khansa yamba; Komabe, ndiwo mtundu wambiri wa matenda a magazi m'gulu lake, MDS / MPN, yomwe ikufotokozedwa pansipa.

Mawonekedwe

Mndandanda wa CMML, monga wa ma khansa ena ambiri a magazi, wakhala akusinthidwa zaka zambiri, mofananamo ndi chitukuko cha kumvetsa bwino za sayansi za matendawa.

Masiku ano, CMML imadziwika kukhala ndi maonekedwe a magawo awiri osiyana a matenda a magazi: myelodysplastic syndrome ndi myeloproliferative neoplasm .

• Myelodysplastic syndrome (MDS) ndi gulu la matenda omwe amakhudza maselo obadwa m'magazi. Mu MDS, fupa la mafupa limapanga maselo osawoneka, omwe amakhala ndi magazi omwe amatchedwa maselo. Maselo amenewa amalephera kukula bwino ndipo sangathe kugwira ntchito zawo monga maselo a magazi.
• Myeloproliferative neoplasm (MPN) amatanthauza matenda omwe mafupa amachititsa maselo ofiira ambiri, magazi, kapena mitundu yosiyanasiyana ya maselo oyera.

Anthu omwe ali ndi CMML ali ndi maselo osakongola (mafupa) omwe amapezeka m'mafupa awo, ndipo, kwa nthawi yaitali, CMML imatengedwa ngati mtundu wa myelodysplastic syndrome.

Komabe, anthu omwe ali ndi CMML amakhalanso ndi maselo oyera amagazi a monocyte, ndipo mbali yaikulu ya MDS ili ndi maselo ochepa a magazi m'magazi, kotero CMML sinali yabwino kwambiri ku gawo la MDS.

Myelodylastic / myeloproliferative neoplasms (MDS / MPN)

Pazifukwa izi ndi zina, bungwe la World Health Organization (WHO) likuphatikizapo CMML mu gulu lonse: MDS / MPN, monga momwe tawonetsera m'munsiyi.

CMML ndi imodzi mwa mitundu yosiyanasiyana ya MDS / MPN.

• Matenda a maelomonocytic a m'magazi (CMML)
• Mankhwala oopsa a myeloid leukemia (aCML), BCR-ABL1 -
• Matenda a khansa ya maelomonocytic (JMML)

• MDS / MPN ndi mphete zowonjezera ndi thrombocytosis (MDS / MPN-RS-T)
• MDS / MPN, yosawerengeka

CMML ndi matenda ambiri m'gulu lino. Matenda ochepa omwe amapezeka m'maguluwa ndi odwala matenda odwala matenda a m'mitsempha omwe amawopsa kwambiri. Matenda onsewa amabweretsa maselo ambiri osadziwika.

M'ndondomeko yomwe inalembedwa mu 2016 ndi WHO, chinachake chomwe poyamba chimatchedwa refractory anemia ndi ringeroblasts chogwirizana ndi thrombocytosis (RARS-T) yodziwika kuti inavomerezedwa kuti ndivomerezedwe, dzina lachidziwitso la MDS / MPN ndi ringeroblasts ndi thrombocytosis.

Zizindikiro ndi Zizindikiro

Pokhala ndi monocyte wochuluka kwambiri kwa nthawi yaitali, kawirikawiri, miyezi itatu ndi chizindikiro chofala kwambiri cha CMML.

Kuchuluka kwa monocytes ndiko chifukwa cha zizindikiro zambiri. Ma monocyte owonjezera amatha kuyenda ndi ziwalo ziwiri m'mimba, chiwindi, ndi ntchentche, zomwe zingayambitse zizindikiro zina.

• Mphuno yotukulidwa , kapena splenomegaly, ikhoza kupweteka kumtunda kumtunda kwa mbali ya mimba. Kuonjezera m'deralo kungathandizenso anthu kumverera mofulumira kwambiri akamadya.
• Ngati chiwindi chikulitsa, chomwe chimatchedwa hepatomegaly, izi zingachititse kuti munthu asamavutike pamimba komanso kumtunda.

Ngakhale anthu omwe ali ndi CMML amapanga maselo oyera amagazi ambiri, nthawi zina pangakhale kusowa kwa mitundu ina ya maselo ofiira ndi oyera monga gawo lonseli, ndipo kuchepa kumeneku kungatulutsenso zizindikiro za CMML.

• Kuperewera kwa maselo ofiira a magazi, kapena kuchepa kwa magazi, kungachititse kuti mukumva wotopa kwambiri, komanso kufooka, mpweya wochepa, ndi khungu lotumbululuka.
• Mawerengedwe ochepa a maselo oyera a mtundu woyera, kapena leukopenia, angayambitse matenda opatsirana kapena oopsa.
• Kuperewera kwa mapuloteni a magazi, kapena thrombocytopenia, kungachititse kuti magazi asasamalire komanso kuvulaza. Kwa munthu yemwe ali ndi CMML, izi zikhoza kuzindikiridwa ngati zowonjezereka kapena zowopsya kwambiri m'mphuno kapena m'mimba mwazi.

Zizindikiro zina zingaphatikizepo kutaya kwadzidzidzi, kutentha thupi, komanso kusowa kwa njala.

Zizindikilo zingapereke zizindikiro, koma sizikwanira kuti azindikire za CMML.

Kuzindikira ndi Kufufuza

Kupezeka kwa CMML kumaphatikizapo kufufuza kwapadera kwa maselo kuchokera m'magazi anu ndi mafupa. Izi zikutanthawuza kuti mafupa a chifuwa ndi gawo la kuunika, kuwonjezera pa magazi omwe amadziwika bwino akukoka kuchokera kumsana pogwiritsa ntchito singano.

Malinga ndi zomwe anapeza poyesa koyambirira, palinso njira yothetsera vutoli, chifukwa matenda ambiri angabweretse zizindikiro izi ndi zofunikira kwambiri za ma laboratory.

2016 Zowonongeka za WHO WHO CMML

1. Kupitirizabe (kwaposa miyezi itatu) kuchuluka kwa monocytes (monocytosis) m'magazi achilendo kuposa 1000 / microL.
2. Malipoti a monocytes amaposa 10 peresenti ya chiwerengero chonse cha magazi oyera (WBC).
3. Osayanjana ndi mayeso a WHO omwe ali ndi vuto lina la magazi: BCR-ABL1 chronic chronic myeloid leukemia, primary myelofibrosis, polycythemia vera, kapena thrombocytosis yofunikira
4. Peresenti ya kamwana kamene imayambitsa mitundu m'magazi ndi mumtambo: osachepera 20 peresenti ya myeloblasts + monoblasts + promonocytes m'magazi a magazi ndi mafupa.
5. Kusintha kwa Dysplastic (maonekedwe osamvetseka ndi maonekedwe a microscope) m'modzi kapena m'mabanja ambiri omwe amakhala ndi maselo a m'magazi "mumtundu wa myeloid." M "banja la myeloid," nthambi "zikuphatikizapo maselo omwe amagawanitsa ndi okhwima kuti athandize monocytes, macrophages, neutrophils, basophils, eosinophils, maselo ofiira a magazi, maselo a dendritic, ndi megakaryocytes.

Matenda omwe ali ndi zotsatira zofanana ndi CMML nthawi zambiri amayenera kutulutsidwa kunja, ndipo nthawizina madokotala amapanga mayesero oonjezera omwe amalangizidwa asanayambe kuchiza odwala CMML, komanso kupopera koyamba, popeza akupeza kale fupa ndi mafupa a magazi kuti adziwe.

Ngati zina zamoyo zimasintha kapena zowonongeka kwa majeremusi ena zimapezeka m'maselo ovuta (omwe sali ambiri mwa anthu omwe ali ndi monocytosis, koma a PDGFRA kapena PDGFRB, FGFR1, kapena PCM1-JAK2) ndiye pali kusiyana kosiyana kwa WHO, ndi wothandizira wina wotchedwa imatinib.

Magulu a CMML

Mndandanda wa WHO umaphatikizapo anthu omwe ali ndi CMML m'magulu atatu osiyana ndi omwe akugwirizana nawo:

• CMML-0: Peresenti zosakwana 2 peresenti ikuphulika m'magazi amagazi ndipo osachepera 5% amaphulika mu mafuta.
• CMML-1: 2 mpaka 4 peresenti ikuphulika m'magazi am'thupi ndipo / kapena 5 mpaka 9 peresenti ikuphulika m'mawa.
• CMML-2: 5 mpaka 19 peresenti ikuphulika m'magazi amagazi, 10 mpaka 19 peresenti ikufalikira mumtambo, ndi / kapena kukhalapo kwa ndodo imodzi ya Auer (makina a burgundy a mitundu yosiyanasiyana yomwe imapanga maonekedwe osiyana, omwe amawonekera mu cytoplasm ya kuphulika kwa myeloid).

Chithandizo

Kugwiritsira ntchito mafuta a mafupa kuchokera kwa wopereka (allogeneic hematopoietic cell transplantation) ndi njira yokhayo yothandizira odwala CMML. Ngati chiri chosankha, chisankho ichi chapangidwa monga zotsatira za zokambirana za dokotala-wodwala.

Mayesero Amakono

Ponena za chemotherapy, pakadali pano, palibe "magic bullet kwa CMML". Mankhwala omwe alipo samakhudza kwambiri chikhalidwe cha matenda, ndipo kotero anthu omwe ali ndi CMML amalimbikitsidwa kuti alembere kulembera ku mayesero a chipatala, akapezeka.

Kwa anthu omwe sapita kukadula, ndi omwe salembetsa mayesero, pali njira zingapo zothandizira kuchiza matendawa, kuphatikizapo mankhwala, kuphatikizapo mankhwala othandizira.

Thandizo lachizindikiro ndi Lothandizira

Malangizo a akatswiri amanena kuti odwala popanda zizindikiro ayenera kuyang'anitsidwa ndi madokotala awo ndi kufufuza nthawi ndi kafukufuku wa laboratori. Amalimbikitsanso kuti katemera asinthidwe komanso ngati wodwala akusuta, kusuta kwaimitsidwa.

Ngati palibe vuto la kuchipatala, palinso mankhwala omwe angagwiritsidwe ntchito "kugunda" maselo osadziwika (cytoreductive therapy) monga hydroxyurea, azacitidine, kapena decitabine.

Kusamalira odwala omwe ali ndi CMML ndi ofanana ndi zomwe zimachitikira odwala MDS. Nthawi zambiri amapatsidwa maselo ofiira ndi mapiritsi, ndipo amagwiritsidwa ntchito pogwiritsa ntchito mankhwala a erythropoiesis, komanso mankhwala opatsirana pogwiritsa ntchito maantibayotiki.

Kwa CMML yowonongeka, kapena milandu imene ayesedwa koma olephera, odwala amalimbikitsidwa kutenga nawo mbali pa mayesero akachipatala akapezeka.

Kuthamangitsani

Palibe chiwerengero chabwino cha ballpark chokhalira ndi moyo, popeza anthu omwe ali ndi CMML akhoza kukhala ndi zosiyana kwambiri ndi matendawa.

Nambala yapakatikati (kapena, chiwerengero, pakati pa nthawi yambiri yopulumuka) ndi pafupifupi miyezi 30 yomwe ikupulumuka nthawi yomwe yapezeka. Pali kusiyana kwakukulu pakati pa izi, komabe, komanso chiwerengero cha moyo, sizinapangidwe mankhwala atsopano omwe alibe deta. Kugonjetsa kumawonedwa mwatsatanetsatane pansipa, pamene magulu osiyanasiyana a CMML owopsa amafotokozedwa.

Kusakayikira pa ziwerengero kungakuchititseni kukhala ndi nkhawa. Kukambirana momasuka ndi dokotala kudzakuthandizani kumvetsetsa vuto lanu. Pali zotsatira zosiyanasiyana zosiyana ndi zomwe zimakhalapo ndi CMML, ndipo pali zolemba zisanu ndi zinayi zosiyana siyana zomwe zimayesetseratu kuti ziwathandize kupeza zizindikiro zomwe zimagwiritsa ntchito zinthu monga zochitika zachipatala ndi zofukufuku za ma laboratori, ndipo ena amaonanso ma genetic a maselo a khansa. .

Pakhoza kukhala kusiyana kwakukulu mwa njira imene madokotala akuyendera chiopsezo cha CMML, malingana ndi bungwe, koma palibe deta yomwe ikuwoneka kuti ikulozera njira imodzi yabwino yowunika chiopsezo.

Ziwerengero zochokera ku American Cancer Society panopa zilipo, koma zimathandizira kusonyeza kusiyana kwa magulu a CMML-1 ndi CMML-2, komanso zikuwonetseratu momwe magulu ena mwa anthu onse akuwonekera bwino kuposa ena.

Mu kafukufuku wina wa odwala CMML omwe anapezeka pakati pa 1975 ndi 2005, nthawi yamoyo yapakati pa CMML-1 ndi CMML-2 inali miyezi 20 ndi miyezi 15, motero. Komabe, odwala ena anakhala ndi nthawi yaitali. Pafupifupi 20 peresenti ya odwala CMML-1 ndipo pafupifupi 10 peresenti ya odwala CMML-2 anapulumuka zaka zoposa zisanu. Komanso, odwala omwe ali ndi CMML-2 amatha kukhala ndi khansa yoopsa kwambiri kuposa odwala omwe ali ndi CMML-1. Phunziro lomwelo, 18 peresenti ya odwala CMML-1 ndi 63 peresenti ya odwala CMML-2 ali ndi vuto la myeloid leukemia m'kati mwa zaka zisanu za CMML.

Mawu Ochokera

Kwa munthu amene ali ndi CMML, matendawa angadalire pazifukwa zambiri, ndipo zaka za munthu ndi thanzi labwino ndizofunikira. Chifukwa chakuti matendawa amakhudza anthu ambiri achikulire amene angakhale ndi matenda ena aakulu, mankhwala opweteka kwambiri komanso omwe amatha kuwombera mafupa-osakhala nthawi zonse.

Njira zabwino zothandizira zilipo, koma CMML ndi matenda omwe amawoneka kuti ali pamphepete mwa zofunikira zothandizira. Choncho, zomwe zidasindikizidwa dzulo zingakhale zoona kapena sizikhala zoona mawa, choncho yang'anani zomwe mungasankhe ndikuganiza kuti mukulembera mu mayesero a zachipatala.

> Zotsatira:

> Arber DA, Orazi A, Hasserjian R, et al. Kukonzanso kwa 2016 ku bungwe la World Health Organisation la myeloid neoplasms ndi chiwindi chachikulu cha khansa. Magazi . 2016; 127: 2391-2405.

Elena C, Gallì A, E E, et al. Kuphatikiza zochitika zachipatala ndi zilonda zam'mimba poyeza chiopsezo cha odwala omwe ali ndi matenda a khansa ya myelomonocytic ya m'magazi. Magazi 2016; 128 (10): 1408-1417.

> Zeidan AM, Hu X, Long JB, et al. Mankhwala a hypomethylating amagwiritsidwa ntchito ndi kupulumuka kwa okalamba omwe ali ndi matenda a khansa ya myelomonocytic ku United States: Kuphunzira kwakukulu kwa anthu. Khansa . 2017 Oct 1; 123 (19): 3754-3762.