Mtundu wa Khansara Yamagazi Umayang'aniratu Okalamba Achikulire
Mankhwala oopsa a myeloid (AML) ndi mtundu wa khansara umene umayambira poyamba m'mafupa kumene maselo amagazi amapangidwa ndiyeno amasunthira msangamsanga ku maselo enieniwo. Kuyambira pamenepo, khansara ikhoza kufalikira kumbali zina za thupi kuphatikizapo chiwindi, nthenda, khungu, ubongo, ndi msana.
AML amakhudza anthu mamiliyoni miliyoni chaka chilichonse ndipo imapha anthu 150,000.
Ku United States kokha, milandu pakati pa 10,000 ndi 18,000 imapezeka chaka chilichonse.
Mosiyana ndi mitundu ina ya khansa ya m'magazi , yomwe imakonda kugunda achinyamata, AML imakhudza anthu akuluakulu oposa 65. Pakati pa zaka zino, zaka zisanu zomwe zimapulumuka ndizosauka, zimangoyenda pafupifupi 5 peresenti. Machiritso ochiritsira pakati pa achinyamata akuluakulu amakhala bwino ndi kulikonse kuchokera pa 25 peresenti kufika pa 70 peresenti pokwaniritsa chikhululukiro chonse pambuyo chemotherapy.
Zizindikiro za Matenda
Khansa ya m'magazi ndi gulu losiyanasiyana la khansa limene limakhudza ziwalo zonse zopanga magazi ndi maselo enieni. Ngakhale kuti matendawa amakhudza maselo oyera a m'magazi , mitundu ina ya matendawa imayambitsa mitundu ina ya maselo.
Pankhani ya AML, mawu akuti "pachimake" amagwiritsidwa ntchito chifukwa khansa ikukula mofulumira, pamene "myeloid" amatanthauza mafupa onse ndi mafupa ena omwe amapanga mafupa amapanga.
AML imayamba m'selo yambiri yamagazi yomwe imatchedwa myeloblast.
Awa ndiwo maselo omwe, mwachikhalidwe, angakhale okhwima mu maselo oyera a magazi oyera monga granulocytes kapena monocytes . Komabe, ndi AML, myeloblasts idzakhala yabwino "yozizira" mu chikhalidwe chawo chachinyamatayo koma akupitiriza kuchulukana osasunthika.
Mosiyana ndi maselo achibadwa omwe ali ndi moyo wapadera, maselo a khansa amakhala "osakhoza kufa" ndipo adzapitiriza kufotokoza mosalekeza.
Ndi ma AML, maselo a magazi a khansa amatha kutulutsa maselo omwe amawazungulira komanso amachititsa kuti maselo atsopano a magazi, maselo ofiira (erythrocytes), ndi maplatelet ( thrombocytes ) akhalepo .
AML ndi wosiyana ndi msuweni wake wamadzimadzi oopsa a khansa ya m'magazi (ALL) yomwe imakhudza mtundu wina wa selo loyera la magazi lotchedwa lymphocyte . Ngakhale kuti AML makamaka imakhudza akuluakulu, ONSE amawakantha ana pakati pa zaka ziwiri ndi zisanu.
Zizindikiro Zoyambirira ndi Zizindikiro
Zizindikiro za AML zimagwirizana kwambiri ndi kusuntha kwa maselo achibadwa ndi khansa. Kupezeka kwa maselo ambiri a magazi kumatha kuchoka kwa munthu amene ali pachiopsezo ku matenda ndi matenda ena omwe thupi lingalepheretse.
Mwachifanizo, maselo oyera amagazi ndi ofunika kwambiri ku chitetezo cha mthupi. Maselo ofiira ofiira, mosiyana, ali ndi udindo wonyamula oksijeni ndi kuchotsa mpweya woipa kuchokera ku minofu, pamene mapaleti ndi ofunika kwambiri kuti magazi asatseke.
Kutaya kwa maselo onsewa kungachititse kuti zizindikiro zisawonongeke, nthawi zambiri zosakhala zovuta komanso zovuta kuzipeza. Zitsanzo zikuphatikizapo:
- Kuperewera kwa maselo oyera a magazi kungapangitse chiopsezo cha matenda omwe satha. Izi zikuphatikizapo zizindikiro zokhudzana ndi kusowa kwa lekocyte (leukopenia) kapena neutrophils ( neutropenia ).
- Kuperewera kwa maselo ofiira a magazi kungayambitse kuperewera kwa magazi m'thupi kumene kungasonyeze ndi zizindikiro za kutopa, kupweteka, kupuma pang'ono, kumutu, chizungulire, ndi kufooka.
- Kuperewera kwa mapuloleteni kungapangitse thrombocytopenia ndi kukula kwa mimba yamagazi, kupweteka kwambiri kapena kutaya mwazi, kapena kuphulika kwafupipafupi kapena koopsa.
Zizindikiro Zotsatira Zotsatira
Pamene matendawa akupita, zizindikiro zina zowonjezera zingayambe kukula. Chifukwa maselo a khansa ya m'magazi ndi aakulu kuposa maselo oyera a mwazi oyera, amakhala otetezeka m'zitsulo zing'onozing'ono zozungulira kapena kusonkhanitsa ziwalo zosiyanasiyana za thupi.
Malinga ndi kumene kutsekedwa kumachitika, munthu akhoza kupeza:
- Chloromas , maselo olimbitsa thupi omwe angapangidwe kukhala osakanikirana ndi minofu, kupwetekedwa, kapena kupweteka kwa chifuwa
- Leukostasis , vuto linalake lachipatala lomwe likhoza kutengera zizindikiro zofanana ndi kupwetekedwa
- Mphuno yotchedwa Sweet's syndrome , yomwe imapweteka kwambiri pakhungu, makamaka pamitengo, mutu, miyendo, ndi thunthu
- Pansi pa mitsempha ya thrombosis (DVT) imene mitsempha imatsekedwa, nthawi zambiri pamlendo
- Embolism yamapulism (PE) , kutsekedwa kwa mitsempha m'mapapo
- Kutsekedwa kwa m'mimba chifukwa cha kusungunuka kwa maselo m'matumbo ndi chiwindi
- Maninge a khansa ya m'magazi yomwe ikuwonetsa matenda aakulu monga mitu, kusanza, maso osokonezeka, kugunda, vuto lolimbana, ndi kusowa kwa nkhope
Nthawi zambiri, AML ikhoza kukhudza impso, maselo, maso, kapena mawere.
Zifukwa ndi Zoopsa
Pali zifukwa zingapo zoopsya zogwirizana ndi AML. Komabe, kukhala ndi chinthu chimodzi kapena zingapo izi sizikutanthauza kuti mudzalandira khansa ya m'magazi. Pakadali pano, sitimvetsetse bwino chifukwa chake maselo ena angasinthe mwadzidzidzi pamene ena samatero.
Chimene tikudziwa ndi chakuti khansa imayambitsidwa ndi zolakwika zomwe zimachitika pamene selo limagawanika. Timatchula izi ngati kusintha. Ngakhale kuti kusintha kwakukulu sikungayambitse khansa, nthawi zina vuto linalake lidzatha "kutseka" chinthu china chotchedwa chotupa choyambitsa matenda omwe amachititsa kuti selo likhale lalitali. Ngati izi zikuchitika, maselo osadziwika akhoza kusinthidwa mosavuta.
Pali zifukwa zingapo zoopsya zokhudzana ndi izi:
- Kusuta
- Kugwira ntchito kwa mankhwala, makamaka kwa benzene
- Mankhwala enaake okhudza kansalu, kuphatikizapo cyclophosphamide , mechlorethamine, procarbazine, chlorambucil, melphalan, busulfan, carmustine, cisplatin, ndi carboplatin
- Kuwopsa kwa ma radiation, monga khansa radiotherapy
- Kukhala ndi matenda ena aakulu monga matenda a myeloproliferative (MPS) kapena myelodysplastic syndromes (MDS)
- Kukhala ndi matenda ena obadwa monga Down syndrome , Fanconi anemia , ndi neurofibromatosis mtundu 1
Pazifukwa zosadziwika, amuna ndi 67 peresenti yowonjezera kupeza AML kuposa akazi.
Kudziwa
Ngati AML akukayikira, matendawa amayamba ndi kuunika kwa thupi ndikuyang'ana mbiri yake ya mankhwala ndi banja. Panthawi yoyezetsa magazi, dokotalayo amamvetsera mwachidwi zizindikiro monga kupweteka kwambiri, kutuluka m'magazi, matenda, kapena kupweteka kwa maso, mkamwa, chiwindi, nthata, kapena zam'mimba. Chiwerengero cha magazi chokwanira (CBC) chidzachitsidwanso kuti chizindikiritse zosawonongeka zomwe zili m'magazi.
Malinga ndi zomwe apezazi, dokotala akhoza kulamula mayeso angapo kuti atsimikizidwe kuti ali ndi vutoli. Izi ziphatikizapo:
- Mafupa a mafupa omwe mafupa a mafupa amachotsedwa mwa kuika singano yaitali mu fupa, kawirikawiri kuzungulira mchiuno
- Mphuno wa mafupa umakhala ndi singano yaikulu yomwe imalowetsedwa mkati mwa fupa kuti achoke maselo
- Kutsekedwa kwa maluwa (mmphepete mwa msana) yomwe singano yaing'ono imayikidwa pakati pa mafupa o Mzere wa msana kuti uchotse cerebrospinal fluid (CSF )
- Zojambula zojambula monga X-ray, ultrasound, kapena computed tomography (CT )
- Mwazi wamagazi omwe magazi amafufuzidwa pansi pa microscope, makamaka ndi mazira omwe samangotulutsa maselo a m'magazi koma kuthandiza kusiyanitsa pakati pa AML ndi ALL
- Mpweya wotchedwa cytometry womwe umatetezera mapuloteni, otchedwa AML antibodies, amawoneka m'magazi kapena CSF chitsanzo chotsimikizira kuti alipo AML maselo
- Cytogenetics yomwe maselo a khansa ya m'magazi "amakula" mu labu ndikuyang'aniranso pansi pa microscope ya electron kuti azindikire kusintha kwasinthidwe ndi ma chromosomal patterns
Kusinthana
Kafukufuku wa khansa amachitidwa kuti adziwe momwe khansa yafalikira. Izi zimathandizanso dokotala kudziwa njira yoyenera yothandizira kuti munthuyo asaphedwe kapena kuponderezedwa. Maphunzirowa amathandizanso kudziwa momwe munthu angapulumutsidwire nthawi yaitali chithandizo.
Chifukwa AML siimaphatikizapo kupanga mapangidwe oopsa a khansa, silingathe kukhazikitsidwa ndi njira ya TNM (njira ya chifuwa / lymph node / malignancy ).
Pali njira ziwiri zosiyana zomwe zikugwiritsidwa ntchito pokonzekera AML: Chikhalidwe cha French-American-British (FAB) cha AML ndi gulu la World Health Organization (WHO) la AML.
Maofesi a FAB
Chikhalidwe cha French-American-British (FAB) chinakhazikitsidwa m'zaka za m'ma 1970 ndi magawo a matendawa pogwiritsa ntchito mtundu ndi kukula kwa selo yokhudzidwayo.
Zomwe zimapangitsa kuti masewerowa akhale osavuta: AML nthawi zambiri imatsatira chitsanzo chomwe ma myeloblasts amodzi ndiwo maselo oyambirira omwe angakhudzidwe. Pamene nthendayi ikupita, idzayamba kukhudza myeloblasts m'kupita kwa nthawi kusasitsa ndiyeno kupita patsogolo ku maselo oyera achizungu (monga monocytes ndi eosinophils) asanasamuke ku maselo ofiira (erythrocytes) ndipo potsirizira pake megakaryoblasts (maselo ofiira a platelet).
Kupita patsogolo kumeneku kumapatsa odwala matenda okhudzana ndi khansa.
Mapepala a FAB ochokera ku M0 (kwa AML oyambirira) kupita ku M7 (kwa AML apamwamba) motere:
- M0: osakhudzidwa kwambiri ndi myeloblastic leukemia
- M1: mitsempha yambiri ya myeloblastic ndi kutseketsa kochepa
- M2: mitsempha yambiri ya myeloblastic ndi kusasitsa
- M3: pachimake promyelocytic khansa ya m'magazi
- M4: pachimake myelomonocytic khansa ya m'magazi
- M4 eos: mwamphamvu myelomonocytic leukemia ndi eosinophilia
- M5: pachimake monocytic khansa ya m'magazi
- M6: pachimake erythrocytic khansa ya m'magazi
- M7: Mthempha ya m'magazi yambiri
Khalidwe la WHO
Bungwe la World Health Organization linakhazikitsa njira zatsopano zosinthira AML mu 2008. Mosiyana ndi FAB dongosolo, gulu la WHO limaganizira za kusintha kwakukulu kwa chromosomal komwe kunapezeka panthawi yafukufuku wa cytogenetic. Zimathandizanso pazochitika zachipatala zomwe zingasinthe kapena kuonongeka kwambiri ndi momwe munthu akukhudzidwa.
Ndondomeko ya WHO imakhala yowonjezereka kwambiri poyesa matendawa ndipo ikhoza kusweka kwambiri motere:
- AML ndi zosawerengeka zachibadwa zamoyo (kutanthauzira, chizindikiro cha genetic mutations)
- AML ndi kusintha kwa myelodysplasia (kutanthauza kukhalapo kwa MDS, MDP, kapena matenda ena a myeloblastic)
- Matenda otchedwa myeloid neoplasms (okhudzana ndi mankhwala opatsirana kale kapena mankhwala opatsirana pogwiritsa ntchito mankhwala)
- Myeloid sarcoma (kutanthauza AML pamodzi ndi chloroma)
- Kuwonjezeka kwa Myeloid wokhudzana ndi Down syndrome
- Blastic plasmacytoid dendritic cell neoplasm (mtundu woopsa wa khansa yodziwika ndi zilonda za khungu)
- AML osagwiritsidwa ntchito mosagwiritsidwa ntchito (makamaka FAB njira zisanu ndi ziwiri ndi zigawo zina zoonjezera za matenda)
Chithandizo
Ngati atapezeka ndi AML, mawonekedwe ndi nthawi ya chithandizo adzakhala makamaka ndi siteji ya khansa komanso thanzi la munthu aliyense.
Kawirikawiri kulankhula, mankhwala amayamba ndi chemotherapy. Izi zingaphatikizepo mankhwala okalamba omwe amatha kukhudza maselo a khansa komanso osagwidwa ndi khansa komanso mankhwala osokoneza bongo atsopano omwe amapezeka pa maselo a khansa okha.
Mankhwala a chemotherapy regimen amatchulidwa kuti "7 + 3" chifukwa mankhwala osokoneza bongo omwe amadziwika kuti cytarabine amaperekedwa ngati mankhwala opatsirana (IV) osakanikirana masiku asanu ndi awiri otsatiridwa ndi masiku atatu otsatizana a mankhwala ena otchedwa anthracycline . Kufikira 70 peresenti ya anthu omwe ali ndi AML adzalandira chikhululukiro motsatira "mankhwala" 7 + 3.
Ndikunena kuti, khungu laching'ono la khansa ya m'magazi likhoza kukhala likutsatira chemotherapy, zomwe zimayambitsa kubwerera m'mbuyo. Pofuna kupewa izi, madokotala amapereka chithandizo chamankhwala chokhazikika potsatira zotsatira za mankhwala pambuyo pa chithandizo komanso thanzi labwino.
Kwa anthu omwe ali ndi zizindikiro zabwino zothandizira, mankhwala angaphatikizepo maphunziro atatu kapena asanu a mankhwala amphamvu, omwe amatchedwa chemotherapy.
Kwa omwe ali pachiopsezo chachikulu chobwezeretsa, zina, mankhwala oopsa angapangidwe kuphatikizapo kusamba kwa tsinde ngati wopereka angapezeke. Kawirikawiri, opaleshoni kapena mankhwala opatsirana pogwiritsa ntchito mankhwala ochizira matenda angapangidwe.
Chifukwa chakuti aml chemotherapy amachititsa kuti thupi lizipewa kwambiri, odwala okalamba sangathe kulekerera chithandizo ndipo akhoza kupatsidwa chithandizo chochepa chaching'ono kapena cheliative .
Kupulumuka
Malingaliro kwa munthu yemwe adalandira mankhwala a AML akhoza kukhala osiyana kwambiri pa siteji ya khansa panthawi yomwe akudwala matendawa. Koma, palinso zinthu zina zomwe zingathe kufotokozeratu zotsatira zake. Mwa iwo:
- Anthu omwe ali ndi MDS ndi MPD amakhala ndi moyo kuyambira nthawi ya miyezi isanu ndi iwiri kufika pa 11.8 malinga ndi kuopsa kwa matendawa.
- Zosintha za chromosomal zomwe zimapezeka ndi cytogenetics zingapangitse kuti zaka zisanu zapakati pazaka zisanu ndi zisanu zikhale zosakwana makumi asanu ndi limodzi.
- Anthu oposa 60 amene apanga mavitamini a lactate awonongeke (akusonyeza kuwonongeka kwa minofu yambiri) amakhala ndi zotsatira zosauka.
Zonsezi, chiwerengero cha machiritso a AML chiri pakati pa 20 peresenti ndi 45 peresenti. Mipikisano yowonjezera yowonjezera imakhala yabwino kwambiri kwa achinyamata omwe amatha kulekerera chithandizo.
Mawu Ochokera
Ngati mwapezeka kuti muli ndi AML, mudzakumana ndi zovuta zamaganizo ndi zakuthupi zomwe zingakhale zovuta kugonjetsa. Musati mupite nokha. Zomwe mungachite kuti zinthu zizikuyenderani bwino zidzasintha kwambiri ngati mumanga gulu lothandizira lopangidwa ndi okondedwa, akatswiri azaumoyo, ndi ena omwe adutsa kapena akudwala khansa.
Ngakhale mutatha kuchipatala, mantha otha kubwerera angakhalepo kwa miyezi kapena zaka. Pothandizidwa, pamapeto pake mudzagonjetsa nkhaŵa izi ndipo phunzirani kuwonetsa thanzi lanu ndi maulendo omwe nthawi zonse mumapita. Kawirikawiri, ngati kubwereranso sikuchitika pakapita zaka zochepa, sizingatheke kuti AML idzabweranso.
Ngakhale palibe chimene mungachite kuti mutha kubwezeretsanso, moyo wathanzi ukhoza kukuthandizani kwambiri. Izi zikuphatikizapo kupereka zizoloŵezi zabwino zoyenera kudya, kuchita masewera olimbitsa thupi, kusiya kusuta, ndi kupuma mokwanira kupeŵa kupanikizika ndi kutopa.
Pamapeto pake, nkofunika kutenga zinthu tsiku ndi tsiku ndikukhala ndi munthu yemwe mungathe kumuthandizira ngati mukufunikira thandizo.
> Zotsatira:
> American Cancer Society. "Kupulumuka Zambiri za Myelodysplastic Syndromes." Washington, DC; zasinthidwa pa January 22, 2018.
> De Kouchenovsky, I. ndi Abdul Hay, M. "Mthemphayo yambiri ya myeloid: ndondomeko yambiri ndi 2016." Magazi a Can Can 2016; 6; e441.
> Döhner, H ;; Weisdorf, D .; ndi Bloomfield, C. "Acute Myeloid Leukemia." New Engl J Med . 2015; 373 (12): 1136-52. DOI: 10.1056 / NEJMra1406184.