Kodi Myelofibrosis Yoyamba Ndi Chiyani?

Zamkati

• Zizindikiro zoyambirira za myelofibrosis
• Magawo oyambira a myelofibrosis
• Nchiyani chimayambitsa myelofibrosis yoyamba?
• Zowopsa za myelofibrosis yoyamba
• Njira zoyambirira zochizira myelofibrosis
• Mankhwala othandizira kuthana ndi matenda
• Zoletsa za JAK
• Kusintha kwama cell
• Chemotherapy ndi radiation
• Kuikidwa magazi
• Opaleshoni
• Mayesero amakono azachipatala
• Zosintha m'moyo
• Chiwonetsero
• Tengera kwina

Primary myelofibrosis (MF) ndi khansa yosowa yomwe imayambitsa minofu yambiri, yotchedwa fibrosis, m'mafupa. Izi zimalepheretsa mafupa anu kupanga maselo ochuluka amwazi.

Pulayimale MF ndi mtundu wa khansa yamagazi. Ndi imodzi mwamitundu itatu yamatenda a myeloproliferative neoplasms (MPN) omwe amapezeka pomwe maselo amagawika pafupipafupi kapena samafa pafupipafupi momwe amayenera kukhalira. Ma MPN ena amaphatikizapo polycythemia vera ndi thrombocythemia yofunikira.

Madokotala amayang'ana zinthu zingapo kuti apeze oyambira MF. Mutha kuyezetsa magazi komanso kusanthula m'mafupa kuti mupeze MF.

Zizindikiro zoyambirira za myelofibrosis

Simungakhale ndi zizindikilo zilizonse kwazaka zambiri. Zizindikiro nthawi zambiri zimayamba kuchitika pang'onopang'ono pambuyo poti mabala am'mafupa awonjezeka ndikuyamba kusokoneza makina amwazi.

Zizindikiro zoyambirira za myelofibrosis zitha kuphatikiza:

• kutopa
• kupuma movutikira
• khungu lotumbululuka
• malungo
• matenda pafupipafupi
• kuvulaza kosavuta
• thukuta usiku
• kusowa chilakolako
• kuonda kosadziwika
• nkhama zotuluka magazi
• Kutuluka magazi pafupipafupi
• chidzalo kapena kupweteka m'mimba kumbali yakumanzere (chifukwa cha ntchafu)
• mavuto ndi chiwindi
• kuyabwa
• kupweteka kwa mafupa kapena mafupa
• gout

Anthu omwe ali ndi MF nthawi zambiri amakhala ndi maselo ofiira ochepa kwambiri. Angakhalenso ndi kuchuluka kwa maselo oyera a magazi omwe ndi okwera kwambiri kapena otsika kwambiri. Dokotala wanu amangopeza zodabwitsazi pakuwunika nthawi zonse kutsatira kuwerengera kwathunthu kwamagazi.

Magawo oyambira a myelofibrosis

Mosiyana ndi mitundu ina ya khansa, MF yoyamba ilibe magawo omveka bwino. Dokotala wanu atha kugwiritsa ntchito Dynamic International Prognostic Scoring System (DIPSS) kuti akupatseni gulu laling'ono, lapakatikati, kapena loopsa.

Awona ngati mungachite izi:

• khalani ndi mulingo wa hemoglobin wochepera magalamu 10 pa desilita imodzi
• khalani ndi kuchuluka kwama cell oyera omwe amaposa 25 × 10⁹ pa lita imodzi
• ali ndi zaka zoposa 65
• khalani ndi maselo ophulika ofanana kapena ochepera 1%
• amakumana ndi zizindikilo monga kutopa, thukuta usiku, malungo, ndi kuonda

Mumayesedwa kuti ndinu oopsa ngati palibe zomwe zili pamwambazi zomwe zikukukhudzani. Kukwaniritsa chimodzi kapena ziwiri mwanjira izi kumakuikani m'gulu lomwe lili pachiwopsezo chapakati. Kukwaniritsa izi kapena zitatu mwa izi kumakuikani m'gulu lomwe lili pachiwopsezo chachikulu.

Nchiyani chimayambitsa myelofibrosis yoyamba?

Ofufuza samvetsa kwenikweni zomwe zimayambitsa MF. Nthawi zambiri sichimatengera kubadwa. Izi zikutanthauza kuti simungatenge matendawa kuchokera kwa makolo anu ndipo simungathe kuwapatsira ana anu, ngakhale MF imakonda kuyenda m'mabanja. Kafukufuku wina akuwonetsa kuti mwina zimayambitsidwa ndi kusintha kwa majini komwe kumakhudza mayendedwe am'maselo.

mwa anthu omwe ali ndi MF amasintha majini otchedwa janus-associated kinase 2 (JAK2) yomwe imakhudza maselo am'magazi. Pulogalamu ya JAK2 Kusintha kumabweretsa vuto m'mene mafupa amapangira maselo ofiira.

Maselo achilengedwe am'magazi am'mafupa amapanga maselo amwazi okhwima omwe amabwereza mwachangu ndikulanda mafupa. Kuchuluka kwa maselo amwazi kumayambitsa zipsera ndi kutupa komwe kumakhudza kuthekera kwa mafupa kupanga maselo abwinobwino amwazi. Izi nthawi zambiri zimatulutsa maselo ofiira ochepa komanso maselo oyera oyera ambiri.

Ofufuza agwirizanitsa MF ndi kusintha kwina kwa majini. Pafupifupi 5 mpaka 10 peresenti ya anthu omwe ali ndi MF ali ndi MPL kusintha kwa majini. Pafupifupi 23.5% ali ndi kusintha kwa majini kotchedwa calreticulin (CALR).

Zowopsa za myelofibrosis yoyamba

Pulayimale MF ndiyosowa kwambiri. Amapezeka pafupifupi 1.5 pa anthu 100,000 aliwonse ku United States. Matendawa amatha kukhudza amuna ndi akazi.

Zinthu zingapo zitha kuwonjezera chiopsezo cha munthu kuti apeze MF yoyamba, kuphatikiza:

• kukhala wazaka zopitilira 60
• kukhudzana ndi ma petrochemicals monga benzene ndi toluene
• kukhudzana ndi ma radiation
• kukhala ndi JAK2 kusintha kwa majini

Njira zoyambirira zochizira myelofibrosis

Ngati mulibe zizindikiro za MF, dokotala wanu sangakupatseni mankhwala aliwonse m'malo mwake amayang'anitsitsa mosamala mosamala. Zizindikiro zikangoyamba, chithandizo chimayesetsa kuthana ndi matenda ndikukhala ndi moyo wabwino.

Njira zoyambirira zochiritsira myelofibrosis zimaphatikizapo mankhwala, chemotherapy, radiation, kuziika kwama cell, kuthira magazi, ndi opaleshoni.

Mankhwala othandizira kuthana ndi matenda

Mankhwala angapo amatha kuthandiza kuthana ndi kutopa ndi kutseka.

Dokotala wanu angakulimbikitseni aspirin yaing'ono kapena hydroxyurea kuti muchepetse chiopsezo cha venous thrombosis (DVT).

Mankhwala ochizira kuchepa kwa magazi ofiira (magazi m'thupi) olumikizidwa ndi MF ndi awa:

• mankhwala a androgen
• steroids, monga prednisone
• thalidomide (Thalomid)
• lenalidomide (Wowonjezera)
• erythropoiesis zolimbikitsa othandizira (ESAs)

Zoletsa za JAK

Ma JAK inhibitors amathandizira zizindikiritso za MF poletsa zochitika za JAK2 geni ndi puloteni ya JAK1. Ruxolitinib (Jakafi) ndi fedratinib (Inrebic) ndi mankhwala awiri ovomerezeka ndi Food and Drug Administration (FDA) kuti athetse MF wapakati kapena wowopsa. Ma Jhibit inhibitors ena angapo akuyesedwa m'mayesero azachipatala.

Ruxolitinib wasonyezedwa kuti achepetse kukulira kwa ndulu ndikuchepetsa zizindikilo zingapo za MF, monga kusapeza bwino m'mimba, kupweteka kwa mafupa, ndi kuyabwa. Amachepetsanso kuchuluka kwa ma cytokines omwe amatulutsa magazi m'magazi. Izi zitha kuthandiza kuchepetsa zizolowezi za MF kuphatikiza kutopa, malungo, thukuta usiku, ndi kuonda.

Fedratinib nthawi zambiri amaperekedwa pamene ruxolitinib sagwira ntchito. Ndi cholimba kwambiri chosankha JAK2. Amakhala pachiwopsezo chochepa chowononga bongo komanso chowopsa chomwe chimadziwika kuti encephalopathy.

Kusintha kwama cell

Kuthira kwa cell cell ya allogeneic (ASCT) ndiye njira yokhayo yothetsera MF. Zomwe zimadziwikanso kuti kupatsira mafuta m'mafupa, zimaphatikizapo kulandira kulowetsedwa kwa maselo am'maso kuchokera kwa wopereka wathanzi. Maselo oterewa amalowa m'malo mwa maselo osagwira ntchito.

Njirayi ili pachiwopsezo chachikulu chowopsa. Mudzafufuzidwa mosamala musanakwane ndi wopereka. ASCT imangoganiziridwa kwa anthu omwe ali pachiwopsezo chapakati kapena MF omwe ali pachiwopsezo cha zaka 70.

Chemotherapy ndi radiation

Mankhwala a chemotherapy kuphatikizapo hydroxyurea amatha kuthandiza kuchepetsa nthenda yotakata yolumikizidwa ndi MF. Mankhwala a radiation amagwiritsidwanso ntchito pomwe JAK inhibitors ndi chemotherapy sizokwanira kuchepetsa kukula kwa ndulu.

Kuikidwa magazi

Kuikidwa magazi kwa maselo ofiira ofiira atha kugwiritsidwa ntchito kuwonjezera kuchuluka kwa maselo ofiira ndikuchiza kuchepa kwa magazi.

Opaleshoni

Ngati nthenda yotambalala ikuyambitsa zizindikilo zoopsa, dokotala nthawi zina amalimbikitsa kuchotsedwa kwa ndulu. Njirayi imadziwika kuti splenectomy.

Mayesero amakono azachipatala

Mankhwala ambiri pano akufufuzidwa pochiza myelofibrosis yoyamba. Izi zikuphatikiza mankhwala ena ambiri omwe amaletsa JAK2.

MPN Research Foundation imasunga mndandanda wamayeso azachipatala a MF. Ena mwa mayeserowa adayamba kale kuyesa. Ena pakadali pano akulemba odwala. Chisankho cholowa nawo pachipatala chiyenera kupangidwa mosamala ndi dokotala komanso banja.

Mankhwala osokoneza bongo amapyola magawo anayi azamayeso azachipatala asanavomerezedwe ndi FDA. Mankhwala ochepa okha ndi omwe ali mgawo lachitatu la mayesero azachipatala, kuphatikiza pacritinib ndi momelotinib.

Mayeso azachipatala a Phase I ndi II akuwonetsa kuti everolimus (RAD001) itha kuthandiza kuchepetsa zizindikilo ndi kukula kwa ndulu mwa anthu omwe ali ndi MF. Mankhwalawa amaletsa njira yopanga magazi omwe angayambitse kukula kwa maselo mu MF.

Zosintha m'moyo

Mutha kukhala ndi nkhawa m'maganizo mutalandira chithandizo choyambirira cha MF, ngakhale mutakhala kuti mulibe zizindikiro zilizonse. Ndikofunika kupempha thandizo kuchokera kwa abale ndi abwenzi.

Kukumana ndi namwino kapena wogwira nawo ntchito kumatha kukupatsirani zambiri zakomwe kudziwa za khansa kumakhudzira moyo wanu. Mwinanso mungafunike dokotala wanu za kugwira ntchito ndi katswiri wazachipatala.

Zosintha zina pamoyo wanu zitha kukuthandizani kuti muchepetse kupsinjika. Kusinkhasinkha, yoga, kuyenda kwachilengedwe, kapena ngakhale kumvera nyimbo kumatha kukuthandizani kukhala osangalala.

Chiwonetsero

Pulayimale MF mwina siyimayambitsa zizindikilo kumayambiliro ndipo imatha kuyang'aniridwa ndimankhwala osiyanasiyana. Kuneneratu momwe adzapulumukire MF kungakhale kovuta. Matendawa samakula kwa nthawi yayitali mwa anthu ena.

Zomwe zimapulumuka zimayambira kutengera ngati munthu ali mgulu laling'ono, lapakatikati, kapena loopsa. Kafukufuku wina akuwonetsa kuti omwe ali pachiwopsezo chochepa ali ndi ziwopsezo zofananira zaka zisanu zoyambirira atazindikira kuti ndi anthu ambiri, pomwe mitengo yomwe imapulumuka imayamba kuchepa. Anthu omwe ali pachiwopsezo chachikulu adapulumuka mpaka zaka 7.

MF imatha kubweretsa zovuta zazikulu pakapita nthawi. Primary MF ikupita ku khansa yayikulu komanso yovuta kuchiza magazi yotchedwa acute myeloid leukemia (AML) pafupifupi 15 mpaka 20% ya milandu.

Mankhwala ambiri a MF oyambira amayang'ana kuthana ndi zovuta zolumikizidwa ndi MF. Izi zimaphatikizapo kuchepa kwa magazi m'thupi, nthenda yotakulitsa, zovuta zamagazi, kukhala ndi maselo oyera oyera ambiri kapena ma platelet, komanso kukhala ndi kuchuluka kwa ma platelet. Mankhwala amathandizanso kuthana ndi kutopa, kutuluka thukuta usiku, khungu loyabwa, malungo, kupweteka kwamagulu, ndi gout.

Tengera kwina

Pulayimale MF ndi khansa yosowa yomwe imakhudza ma cell anu amwazi. Anthu ambiri sadzakhala ndi zizindikilo poyamba mpaka khansa itakula. Chithandizo chokhacho chomwe mungachiritse MF yoyamba ndikumangika kwa tsinde, koma pali mitundu ingapo yamankhwala ndi mayesero azachipatala omwe akuchitika kuti athetse zizindikiritso ndikukhala ndi moyo wabwino.