Matenda a myeloid a m'thupi (CML) ndi amodzi mwa magulu anayi akuluakulu a khansa ya m'magazi . Zina zitatuzi ndi myeloid leukemia, acute lymphoblastic leukemia, komanso matenda aakulu a khansa ya m'magazi.
Mosasamala mtundu, ma leukemia onse amayamba mu maselo opanga magazi mu mafupa. Mtundu uliwonse wa khansa ya m'magazi imatchedwa kuti khansa yachangu imayamba kukula (khansa yoopsa imakula mofulumira) komanso mtundu wa maselo omwe amapanga magazi.
CML ndi matenda a khansa ya m'magazi , kutanthauza kuti imakula ndikukula pang'onopang'ono. CML imakhalanso ndi khansa ya myelogenous , yomwe imatanthauza kuti imayambira m'maselo oyera achizungu omwe amadziwika kuti maselo a myeloid.
Nchiyani Chimachititsa CML?
Zina mwa kusintha kwa DNA zingayambitse maselo amtundu wokhala ndi mafupa a m'magazi. Anthu omwe ali ndi CML amakhala ndi chromosome ya Philadelphia , yomwe ili ndi jini lachilendo la BCR-ABL. Jini la BCR-ABL limayambitsa maselo oyera a magazi kukula mu njira yachilendo, yosayendetsedwa, yochititsa khansa ya m'magazi.
Ndani Amalandira CML?
CML ikhoza kuchitika pa msinkhu uliwonse, koma imakhala yachilendo kwa anthu akuluakulu oposa zaka 50, omwe amawerengera pafupifupi 70 peresenti ya milandu yonse. Kareem Abdul-Jabbar ndi American wotchuka yemwe ali ndi CML.
Kodi Makompyuta Amagwirizana Bwanji?
CML ndi yochepa. Ku United States mu 2017, chiŵerengerochi chinali chakuti anthu 8,950 atsopano adzachitika ndipo anthu pafupifupi 1,080 adzafa ndi matendawa.
Zizindikiro
Chifukwa CML ndi khansara yofulumira kukula, anthu ambiri alibe zizindikiro pamene amayamba kupeza.
Ndipotu, pafupifupi 40 mpaka 50 peresenti ya odwala alibe zizindikiro zilizonse, ndipo amalandira matendawa pambuyo pa ntchito yamagazi nthawi zonse amadziwika kuti ndi olakwika.
CML ikhoza kuyambitsa zizindikiro pamene ikupita ndi nthawi, komabe. Chifukwa cha ichi, mndandanda wa "zizindikiro zambiri" zingathe kufotokozedwa motere:
- Palibe zizindikiro (mpaka 50 peresenti ya anthu omwe amawapeza)
- Kutopa kwambiri kapena kutopa
- Kufooka
- Fever
- Kutuluka kwa usiku
- Kutaya kosawerengeka kosadziwika
- Ululu kapena umphumphu kumtunda kumanzere pamimba, pansi pa nthiti.
Chizindikiro chotsiriza m'ndandanda ndi chifukwa cha nthonje yofutukuka, yomwe imatchedwanso splenomegaly, yomwe ilipo 46 mpaka 76 peresenti ya anthu omwe ali ndi CML. Kutsekula kotere kwa nthendayi kungapangitse malo ochepa kuti ziwalo zina m'deralo, monga mimba, zomwe zingathandize kuti azikhala odzala msanga pamene adya chakudya.
Kufooka ndi kutopa kumene anthu ena omwe ali ndi CML amatha kukhala nawo kuchokera ku malo osiyanasiyana. Chitsime chimodzi chafooka ndi kutopa ndi kuperewera kwa magazi, kutanthauza kuti thupi liribe maselo ofiira a magazi ofunika kwambiri omwe amanyamula mpweya wabwino. Kuchepa kwa magazi kungakuchititseni kumva ngati simungathe kudzigwiritsa ntchito kapena kugwiritsa ntchito minofu yanu mwakhama.
Kudziwa
Dokotala wanu adzatenga mbiri yanu ya zachipatala ndikuyesera thupi, monga momwe ena akuyendera pa matenda.
Uphungu wamatumbo
Kuyang'ana kukula kwa mpeni wanu ndi gawo lofunikira la kuunika kwa thupi. Nkhumba yachilendo kawirikawiri siimamvekanso, koma nthenda yotambasula imatha kudziwika kumbali ya kumanzere kwa m'mimba, pamtunda wa nthiti.
Nthaka kawirikawiri imasunga maselo a magazi ndi kuwononga maselo akale a magazi. Mu CML, nthendayi imakula chifukwa cha maselo oyera omwe amapezeka m'thupi.
Mayesero a Lab
Kuyesedwa kwa Laboratory kukufunikanso. Magazi amachotsedwa pamtunda mdzanja, ndipo fupa la mafupa limapangidwira kudzera mu njira yotchedwa fupa la mafupa ndi biopsy. Zitsanzo zanu zimatumizidwa ku labu ndipo katswiri wamagetsi amawayesa pansi pa microscope ndikupanga mayesero ena, kuyang'ana kupeza ndi kufotokoza maselo a khansa ya m'magazi, ngati alipo.
Maselo oyera ambiri amagazi ndi magulu osadziwika a mankhwala ena m'magazi angakhale chizindikiro cha CML.
Mu mafupa a mafupa, pamene maselo ambiri opanga magazi alipo omwe akuyembekezeredwa, mafuta amtunduwu amatchedwa hypercellular. Msuzi wa mafupa nthawi zambiri umakhala wochuluka mu CML chifukwa uli wodzaza ndi maselo a khansa ya m'magazi.
Mayeso a Gene
Kuyezetsa magazi kudzachitanso kuyang'ana "chromosome ya Philadelphia" ndi / kapena kachilombo ka BCR-ABL. Mayeso amtundu uwu amagwiritsidwa ntchito kutsimikizira kuti matenda a CML ndi otani. Ngati mulibe chromosome ya Philadelphia kapena BC gene-ABL, ndiye kuti mulibe CML.
Mayesero Ojambula
Zojambula, kapena zojambula, sizikufunika kuti muzindikire CML. Komabe, iwo akhoza kuchitidwa ngati gawo la kupuma kwanu, muzochitika zina; Mwachitsanzo, kufufuza zizindikiro zina kapena kuona ngati palikulitsa kwa peni kapena chiwindi.
Miyeso ya CML
Milandu ya CML ikhoza kusankhidwa kukhala magulu atatu osiyana otchedwa magawo. Gawoli limachokera ku chiwerengero cha maselo oyera a magazi, kapena kuphulika kumene mumakhala nawo m'magazi ndi mafupa. Kudziwa gawo la CML lanu lingakuthandizeni kudziwa momwe matenda anu adzakukhudzireni mtsogolomu.
Phazi Lalikulu
Iyi ndi gawo loyamba la CML. Mu gawoli, muli ndi kuchuluka kwa maselo oyera m'magazi ndi / kapena mafupa. Komabe, maselo oyera a magazi oyera, kapena kuphulika, amapanga osachepera 10 peresenti ya maselo m'magazi ndi / kapena mafupa.
Kawirikawiri, mu gawo lachilendo, palibe zizindikilo, koma pangakhale zina zam'mwamba zotsalira za m'mimba. Chitetezo chanu cha mthupi chimagwirabe bwino kwambiri mu gawo lachilendo, kotero mumathabe kulimbana bwino ndi matenda. Munthu akhoza kukhala mu gawo losatha laling'ono ngati miyezi ingapo mpaka zaka zambiri, zaka zambiri.
Gawo lofulumizitsa
Pakapita nthawi, chiwerengero cha kuphulika maselo m'magazi ndi / kapena mafupa ndi apamwamba kuposa maselo akuluakulu komanso maselo a khansa ya m'magazi amakula kuti adziwe zizindikiro zomwe zimaphatikizapo kutentha thupi, kutaya thupi, osamva njala, ndi nthenda yotambasula.
Chiwerengero cha maselo oyera a m'magazi ndi apamwamba kusiyana ndi chizoloŵezi ndipo mukhoza kusintha m'magazi anu, monga nambala yambiri ya basophil kapena chiwerengero chochepa cha mapaleti.
Pali zigawo zosiyana siyana zomwe zimagwiritsidwa ntchito masiku ano zomwe zimatanthawuzira kuti nthawi yayitali. Bungwe la WHO (World Health Organisation) Criteria limatanthawuza kuti nthawi yowonjezereka ndiyo kukhalapo kwa zotsatirazi:
- 10 mpaka 19 peresenti ikuphulika m'magazi ndi / kapena mafupa
- Oposa 20 peresenti ya basophils mu magazi
- Chiŵerengero chapamwamba kwambiri kapena chotsika kwambiri cha platelet chomwe sichigwirizana ndi mankhwala
- Kuchulukitsa kukula kwa mapeyala ndi kuchuluka kwa maselo oyera a magazi ngakhale mutalandira chithandizo
- Zatsopano zatsopano zimasintha kapena kusintha
Kuphulika Phase
Izi zimatchedwanso "kupweteka koopsa," chifukwa ndilo gawo lachitatu ndi lotsiriza ndipo lingathe kupha moyo. Chiwerengero cha kuphulika maselo m'magazi ndi / kapena fupa la mafupa chimakhala chokwanira ndipo maselowa amafalikira kunja kwa mwazi ndi / kapena mafupa a mafupa ena. Zizindikiro zimakhala zofala kwambiri mu kuphulika, zomwe zimaphatikizapo matenda, kutuluka m'magazi, ululu m'mimba, ndi kupweteka kwa mafupa.
CML kuphulika gawo angawoneke ngati akudwala khansa ya m'magazi kuposa matenda aakulu a khansa ya m'magazi. Kuphulika kwake, maselo a CML akhoza kukhala ngati AML (acute myeloid leukemia) kapena zambiri ngati ALL (acute lymphoblastic leukemia).
Tanthauzo la WHO la kupweteka kwa magazi ndiloposa 20 peresenti likuphulika maselo m'magazi kapena mafupa. Dipatimenti ya International Bone Marrow Transplant Transplant definition ya kuphulika gawo ndiposa 30 peresenti ikuphulika maselo m'magazi ndi / kapena mafupa. Zonsezi zikuphatikizapo kupezeka kwa maselo kunja kwa magazi kapena mafupa a mafupa.
Kuthamangitsani
Poyesera kufotokozera zamankhwala anu, gawo la CML yanu ndilofunikira, koma silolo chokhacho.
Pali zina zambiri zomwe zawonetsedwa kuti zigwirizanitse ndi chiopsezo chanu ngati wodwalayo, kuphatikizapo msinkhu wanu, kukula kwa nthata yanu, ndi chiwerengero cha magazi. Pogwiritsa ntchito zinthu ngati zimenezi, munthu akhoza kugwera m'modzi mwa magawo atatu: otsika, pakati, kapena oopsa.
Anthu omwe ali mumagulu omwe ali ndi chiopsezo chotere amatha kuchitanso chimodzimodzi kuchipatala. Anthu omwe ali m'magulumagulu omwe amapezeka pangozi amawoneka bwino kuposa mankhwala. Komabe, maguluwa ndi zida, osati zizindikiro zenizeni.
Mankhwala a CML
Mankhwala onse ali ndi pangozi komanso phindu, ndipo chisankho chochizira CML ndi chimodzi chokha chimene chimapangidwa pokhala ndi odwala ndi odwala komanso kukambirana za wodwalayo komanso matenda ake onse. Osati munthu aliyense ndi CML amalandira mankhwala onse a CML omwe akufotokozedwa pansipa.
Tyrosine Kinase Inhibitor Therapy
Tyrosine kinase inhibitor therapy ndi mtundu wa mankhwala opatsirana. Kodi cholinga chake n'chiyani? Gulu la mankhwalawa likuwombera mapuloteni osapitirira BCR-ABL omwe amathandiza maselo a CML kukula.
Mankhwalawa amaletsa mapuloteni a BCR-ABL kuchokera kutumiza zizindikiro zomwe zimapangitsa maselo ambirimbiri a CML kupanga. Mankhwalawa amabwera ngati ma mapiritsi omwe angamezedwe.
Thandizo | Kufotokozera |
Imatinib | Kodi woyamba tyrosine kinase inhibitor amavomereza ndi FDA kuti awononge CML; inavomerezedwa mu 2001. |
Dasatinib | Anavomerezedwa kuchipatala cha CML mu 2006. |
Nilotinib | Analoledwa kulandira CML mu 2007. |
Bosutinib | Amavomerezedwa kuti asamalire CML mu 2012, koma amavomerezedwa kwa anthu amene adachiritsidwa ndi tyrosine kinase inhibitor yomwe yasiya kugwira ntchito kapena kuvulaza zotsatira zoipa. |
Ponatinib | Amavomerezedwa kuti azisamalira CML mu 2012 koma amavomerezedwa kwa odwala omwe ali ndi T315I osinthika kapena CML omwe sagonjetsedwa kapena osavomerezeka kwa ena tyrosine kinase inhibitors. |
Immunotherapy
Interferon ndi chinthu chimene chitetezo cha m'thupi chimapanga mwachibadwa. Peg (pegylated) interferon ndi mawonekedwe a nthawi yaitali a mankhwala.
Interferon siigwiritsidwe ntchito ngati mankhwala oyambirira a CML, koma kwa odwala ena, izi zingakhale zosankha pamene sangathe kulekerera tyrosine kinase inhibitor therapy. Interferon ndi madzi omwe amajambulidwa pansi pa khungu kapena mu minofu ndi singano.
Chemotherapy
Omacetaxine ndi mankhwala atsopano omwe amavomerezedwa ku CML mu 2012, odwala omwe amatsutsa komanso / kapena kusagwirizana ndi awiri kapena kuposa ena omwe ali ndi tyrosine kinase inhibitors. Kukanika ndi pamene CML imayankha mankhwala. Kusasalana ndi pamene mankhwala akumwa ndi mankhwala ayenera kuimitsidwa chifukwa cha zotsatira zoyipa.
Omacetaxine amaperekedwa ngati madzi omwe amalowa mkati mwa khungu ndi singano. Mankhwala ena amatha kupatsirana mu mitsempha kapena angaperekedwe ngati mapiritsi kuti amalize.
Hematopoietic Kuika Cell (HCT)
Pambuyo pa tyrosine kinase inhibitors, izi zimatengedwa ngati mankhwala opangira CML, koma matenda a HCT ndi ovuta komanso angayambitse mavuto aakulu. Choncho, sizingakhale chisankho chabwino cha chithandizo kwa wodwala aliyense ndi CML, ndipo malo ambiri opatsirana masiku ano amangoganizira chithandizo ichi kwa odwala osakwana 65.
Mankhwala apamwamba a mankhwala amtunduwu amaperekedwa koyamba kuti awononge maselo onse obadwa ndi maselo a CML mu fupa. HCT ndi njira yomwe imalowetseratu maselo owonongeka mumtambo wako ndi maselo atsopano, owongolera magazi.
Zochitika Zachipatala: Mankhwala Opaleshoni
Mankhwala atsopano amafufuzidwa mosalekeza. Zovuta zachipatala za mankhwala atsopano zingakhale zosankha kwa odwala ena. Nthawi zonse mukhoza kufunsa gulu lanu la mankhwala ngati muli ndi mayesero otseguka omwe mungagwirizane nawo kapena ngati sakukhulupirira kuti mungakhale woyenera pa yesero lamakono.
Mawu Ochokera
Kwa munthu amene ali ndi CML, matendawa amatha kudalira zinthu monga zaka, gawo la CML, chiwerengero cha kuphulika m'magazi kapena mafupa, kukula kwa nthendayi, komanso thanzi labwino.
Poyamba mankhwala omwe amatchedwa tyrosine kinase inhibitors kuyambira mu 2001, anthu ambiri omwe ali ndi CML achita bwino kwambiri, ndipo matendawa amatha kukhala osapitirira zaka zambiri.
Komabe, pali mavuto angapo otsala: zingakhale zovuta kufotokoza, kuyambira pachiyambi, omwe odwala omwe ali ndi CML amakhala ndi zotsatira zosauka. Kuonjezerapo, odwala ambiri amafunikira mankhwala a CML osatha, ndipo mankhwala opondereza amakhala opanda zotsatira. Choncho, ngakhale kupita patsogolo kwakhala kofunikira m'zaka makumi angapo zapitazi, pakadalibe malo okwanira.
> Zotsatira:
> National Cancer Institute. Kuchiza kwa Myelogenous Leukemia Treatment.
Thompson PA, Kantarjian HM, Cortes JE. Kuzindikira ndi chithandizo cha matenda aakulu a myeloid Leukemia mu 2015. Mayo Clin Proc . 201 5; 90 (10): 1440-54.
> Faderl S, Talpaz M, Estrov Z, O'Brien S, Kurzrock R, HM Kantarjian. Biology ya matenda aakulu a myeloid khansa ya m'magazi. N Engl J Med . 1999; 341 (3): 164-172.