Myelofibrosis ndi matenda a mafupa omwe amachititsa kuti mafutawa akhale amtundu wambiri. Minofu yofiira imakhala mkati mwa minofu yopanga magazi, m'makona opatulika a mafupa ena. Komabe, "kupweteka" mu myelofibrosis, sikusiyana ndi kupweteka kochokera ku chilonda cha khungu. Mafupa a mafupa ndi malo opangira maselo onse a thupi, kotero kuti njira yofiira yomwe imayambira pamenepo siilibwino, ndipo ikhoza kutsogolera mavuto ndi maselo a maselo, mavuto aakulu ndikufupikitsa nthawi ya moyo.
Pamene fibrosis imatha, izi zimayambitsa kupangika kwa maselo a magazi, ndipo zosawerengeka mu selo zimawerengeka ndipo nthawi zina mumaselo enieni. Pamapeto pake, mu myelofibrosis yapamwamba, izi zingachititse kuti mongole usagwire ntchito yake yonse. Pamene myelofibrosis ikupita kapena kusintha, zovuta zowopsa monga khansa ya m'magazi komanso zoopsa zowononga magazi ndi zozizira zimatha. N'zotheka kuti myelofibrosis ikhale ndi khansa yoopsa ya khansa ya magazi.
Msingi ndi Myelofibrosis ya Sekondi
Myelofibrosis ikhoza kukhala yoyamba kapena yachiwiri. Pomwe iyo imakhala yatsopano, kapena imawoneka yonse yokha kwa nthawi yoyamba, imatchedwa primary myelofibrosis. Pamene imayamba kugwirizana ndi matenda ena, kuvulala kapena magazi kumatchedwa secondary myelofibrosis. Mwachitsanzo, myelofibrosis pambuyo pa matenda a magazi otchedwa polycythemia vera ndi mtundu umodzi wa myelofibrosis wachiwiri.
Kodi Ndizofanana ndi Khansa?
Zili ndi zinthu zambiri zofanana ndi khansa, koma akatswiri amatchula kuti "myeloproliferative neoplasm ." Kuti zinthu zikhale zosavuta, mawu oti neoplasm angaganizedwe ngati mawu amodzi akuti "chotupa kapena kukula" ndipo zotupa kapena kukulako kungakhale kosavuta kapena zoipa. Myelofibrosis sizowonongeka, koma imakhalanso ndi makhalidwe omwe anthu amayembekezera pamene akuganiza za khansa, kapena mafinya oyipa.
Izi zikuti, ngakhale mutayang'ana myelofibrosis ngati khansara kapena kukula kwa minofu ya mafupa, pakalipano palibe mankhwala omwe amachiza matenda (koma kuperekera mafupa kungathe kukhala ochizira), ndipo ndi njira yomwe ingayende patsogolo zovulaza-ngakhale mofulumira mosiyana mwa anthu osiyanasiyana. Mafomu apamwamba a myelofibrosis amachepetsa nthawi ya moyo ndipo amapanga mtolo wathanzi kwa odwala.
Mudzapeza malo ambiri oleza mtima omwe amatanthauza myelofibrosis monga "khansa yambiri yamagazi." Izi zikhoza kukhala njira yabwino yolankhulira lingaliro lalikulu, koma palinso zambiri pa nkhaniyi. Myelofibrosis ikhoza kuchititsa khansa yamagazi, koma nthawi zina, myelofibrosis ikhoza chifukwa cha khansa ya magazi.
Mitundu
Kuwonjezera pa pulayimale ndi yachiwiri, pali njira zinanso zopangira myelofibrosis. Njira imodzi ndiyo kugwiritsira ntchito magulu a matendawa m'magulumagulu osiyanasiyana malinga ndi zomwe munthu amapeza pamene matendawa amapezeka. Zida zingapo zosiyana zimapezeka kuti zithandize madokotala kudziwa momwe mulili pangozi, kuti muthandize kutsogolera chithandizo ndi kupanga mawonekedwe anu.
Kukula
Malingana ndi Leukemia & Lymphoma Society , myelofibrosis imapezeka pafupifupi 1.5 mwa anthu 100,000 ku United States chaka chilichonse.
Zimakhudza amuna ndi akazi ndipo kawirikawiri zimapezeka mwa anthu oposa zaka 60, koma zikhoza kuchitika pa msinkhu uliwonse. Akuti anthu pafupifupi 16,000 mpaka 18,500 ku United States ali ndi myelofibrosis.
Zimayambitsa
Mitundu yambiri ya maselo ndi majini amadziwika kuti amagwiriridwa, komabe, chifukwa chenichenicho cha kuchepa kwambiri myelofibrosis sikunamveka bwino. Zambiri zamtundu ndi ma chromosomal zakhala zikupezeka, kuphatikizapo kusinthika kutchedwa "JAK2 V617F kusinthasintha," ngakhale kuti kusinthika uku sikukutanthauza kuti mumakhala ndi myelofibrosis yoyamba. Chifukwa cha kusintha kotereku sichikudziwika, ndipo palibe zifukwa zinazake zomwe zingathe kugwiritsidwa ntchito ndi myelofibrosis yoyamba nthawi zambiri.
Kuphatikiza pa khansa ya magazi, matenda ena osagwidwa ndi khansa omwe amatchedwa "myeloproliferative neoplasms" monga polycythemia vera komanso thrombocythemia yofunikira ikhoza kutenganso myelofibrosis yachiwiri. Myelofibrosis yachiwiri kapena yothandizana nayo imatha kuchitika chifukwa cha mankhwala kapena kuvulaza thupi, matenda kapena kutayika kwa magazi ku fupa la fupa.
Myelofibrosis oyambirira, myelofibrosis yokhudza polycythemia vera, ndi myelofibrosis yofunikira kwambiri ya thrombocythemia nthawi zina zimalumikizana pamodzi monga "myelofibrosis," koma asayansi amanena kuti pakhoza kukhala zambiri kuti aphunzire ndi kumvetsa za kusiyana pakati pa mtundu uliwonse.
Zizindikiro
Odwala ambiri alibe zizindikiro pa nthawi ya matenda, koma zizindikiro zofala ndi izi:
- Kutopa
- Kutaya thupi
- Kutuluka kwa usiku
- Fever
- Kumverera kovuta kupuma
- Kusokonezeka m'mimba (chifukwa cha phala lokulitsa)
Kuperewera kwa mafupa kungapangitse zizindikiro kuchokera kuchepa magazi, monga kutopa chifukwa chokhala ndi maselo ofiira a magazi ochepa kwambiri. Zomwe zimakhudza mapiritsi a magazi zingathenso kuwonetsa kupha magazi komanso kutseketsa mavuto.
Zizindikiro zina, monga kukhuta m'mimba kapena kupanikizika, zikhoza kukhala chifukwa cha zonse zomwe zikuchitika kunja kwa mitsempha yowopsya, kuti apange maselo atsopano a magazi:
- Kawirikawiri, kupanga maselo a magazi kumasunthira mafupa m'mimba mwa makanda ozungulira kapena nthawi yobadwa. Koma asanabadwe, makanda angapangitse maselo atsopano m'magazi monga ntchentche, chiwindi, ndi ma lymph nodes-malo awa opanda mafupa amatchedwa extramedullary.
- Kawirikawiri kwa akuluakulu, malo okhawo a maselo atsopano a magazi ndi mafupa. M'magazi ena a khansa ndi matenda a magazi, anthu amabwerera kubweretsa maselo a magazi kulikonse kumene angathe, pa malo otchedwa extramedullary sites. Mu myelofibrosis, kawirikawiri izi zimapezeka mu nthenda ndi chiwindi. Nthawi zina nthata ya munthu ikhoza kukhala yaikulu chifukwa cha extramedullary hematopoiesis mu myelofibrosis.
Zovuta zazikulu za myelofibrosis zimachokera ku kuperewera kwa mafupa ndi extramedullary hematopoiesis .
Pali chiopsezo chowonjezeka cha kusintha kwa mitsempha yambiri ya myeloid (AML) ndi myelofibrosis, ndipo pafupifupi 20 peresenti ya anthu omwe ali ndi myelofibrosis amakhala ndi khansa yaikulu ya m'magazi.
Kudziwa
Kuphatikiza pa zomwe adokotala amapeza kudzera mu zizindikiro zanu ndi kuyezetsa thupi, pali mayesero angapo omwe amapereka chidziwitso chofunika kwambiri cha matenda. Izi zimaphatikizapo ziwerengero za magazi, ntchito zina za magazi, zojambula zojambula monga x-rays ndi MRI, mayeso a fupa la bone , ndi mayesero a jini. Chitsanzo cha magazi kapena mafupa a mafupa chingatumizedwe ku labotore kuti ayang'ane kusintha kwa majini (monga JAK2, CALR, kapena kusintha kwa MPL) omwe nthawi zambiri amakhalapo kwa anthu omwe ali ndi myelofibrosis.
Zinthu zina zomwe zingawoneke ngati myelofibrosis, koma sizinaphatikizepo matenda aakulu a khansa ya m'magazi, matenda ena a myeloproliferative, matenda aakulu a khansa ya m'magazi, ndi khansa ya m'mitsempha yambiri.
Chithandizo
Pakalipano, palibe mankhwala osokoneza bongo omwe ali ochizira. Cholinga cha odwala ambiri ndi kuthetsa zizindikiro, kuchepetsa nthenda yotambasula ndi kusintha kuchuluka kwa maselo a magazi. Mogwirizana ndi zolinga izi, cholinga chachikulu ndikuchepetsanso mavuto.
Myelofibrosis ndi matenda omwe amachititsa chithandizo chamankhwala chochepa, koma ochita atsopano ambiri akufufuzidwa ndikupangidwa. Chithandizo chikutsogoleredwa ndi zifukwa zanu monga kukhalapo kwa zizindikiro, komanso chiopsezo chanu cha myelofibrosis, komanso pa msinkhu wanu wonse ndi thanzi lathunthu.
Kwa anthu omwe ali ndi chiwopsezo chochepa komanso opanda zizindikilo, kuwonetsetsa kungakhale koyenera. Kwa matenda oopsa kwambiri, kupalesedwa kwa maselo opatsirana kuchokera kwa wopereka nthawi zambiri kumaganiziridwa, koma si odwala onse omwe ali oyenerera chifukwa cha zoopsa. Odwala ena ndi oyenerera kuchipatala kapena kuchipatala.
Mu 2011, Food and Drug Administration (FDA) inavomereza ruxolitinib (Jakafi) pofuna kuchiza myelofibrosis, yomwe imaphatikizapo myelofibrosis, post-polycythemia vera myelofibrosis, ndi thrombocythemia myelofibrosis.
- Chivomerezo cha FDA chinachokera ku zotsatira za mayesero awiri olamuliridwa ndi odwala omwe ali ndi myelofibrosis omwe ali pakati kapena omwe ali pangozi poyerekezera ruxolitinib ndi placebo (Phunziro 1) kapena mankhwala othandiza omwe alipo (Phunziro 2). Mu Phunziro 1, 42 peresenti ya odwala amathandizidwa ndi ruxolitinib, poyerekeza ndi 1 peresenti ya odwala omwe ali ndi placebo, omwe amadziwa kuchepa kwaperesenti 35 peresenti pa masabata 24. Panthawi yolandila, odwala 75 peresenti ya Phunziro 1 ndi 67 peresenti pa Phunziro 2 omwe adapeza kuchepa kwa magawo 35 peresenti ya phulusa lidachitabe kuchepetsedwa kwa nthendayi.
- Ruxolitinib imathandiza kuchepetsa kukula kwa mpeni ndi kuchepetsa zizindikiro kwa odwala ambiri. Kuyezetsa magazi kwa azimayi omwe ali ndi myelofibrosis omwe ali ndi chiopsezo chachikulu, gulu lalikulu la anthu omwe ali ndi gawoli linakula bwino kwambiri chifukwa cha matendawa. Iwo analemba diary tsiku ndi tsiku polemba zizindikiro zofooketsa za myelofibrosis zomwe zimaphatikizapo kutaya kwa m'mimba, ululu pansi pa nthiti zamanzere, itchiness, usiku kutuluka ndi fupa / minofu ululu.
- Njira yowonongeka ya mankhwala osokoneza bongo, inachepera 1 peresenti ya odwala omwe amachizidwa ndi ruxolitinib, kuphatikizapo mapulaneti apansi, kuchepa magazi m'thupi, kuvulaza, chizungulire, ndi mutu. Kuipa kwa mankhwala osokoneza bongo omwe anali oposa omwe amapezeka ndi ruxolitinib poyerekeza ndi placebo mu Phunziro 1 anali ndi mapepala apansi (omwe anadziwika ndi 13 peresenti ya odwala omwe ali ndi ruxolitinib, poyerekeza ndi 1 peresenti ya odwala omwe ali ndi placebo) ndi kuchepa kwa magazi 45 peresenti ya odwala amachiritsidwa ndi ruxolitinib, poyerekeza ndi 19 peresenti ya odwala odwala ndi placebo). Zotsatira zofananako zinawonetsedwa mu Phunziro 2.
Njira zina zothandizira kuti zikhale zothandiza pa nthendayi yowonjezereka ndi kuchepetsa zizindikiro zimaphatikizapo chemotherapies, kuchotsa ntchentche kapena splenectomy, ndi mankhwala ochepetsa mphamvu ya mazira kwa nthata. Kuikidwa magazi kungaperekedwe chifukwa cha magazi m'thupi, komanso kwa odwala omwe ali ndi magazi ochepetsa magazi, mankhwala osokoneza bongo monga erythropoietin, androgens (mwachitsanzo, danazol), ndi ma immunomodulators (mwachitsanzo, lenalidomide) angagwiritsidwe ntchito.
Kuthamangitsani
Malingana ndi kafukufuku wakale, magulu ena a anthu omwe amapezeka kuti ali ndi myelofibrosis anakhala ndi moyo zaka zambiri, pomwe m'magulu ena, nthawi zamoyo zinali zosakwana zaka 3 mpaka zisanu kuchokera pa matenda. Pafupifupi 60 peresenti ya odwala ali ndi mayifibrosis oyambirira / adiopathic amakhala zaka zisanu. Pali gulu lalikulu la odwala, komabe, omwe amakhala zaka 10 kapena kuposa.
Anthu omwe amakonda kuchita bwino ndi awa omwe ma hemoglobine ali oposa 10 g / dL, chiwerengero cha mapiritsi chiposa 100x3 / L ndi omwe ali ndi chiwindi chochepa. Kukula kwa nthata ndi kugonana sizikuwoneka kuti zimakhudza kwambiri kupulumuka mu maphunziro, ngakhale mukuganiza, kuchepetsa kukula kwa nthonje kungatanthauzire ku zopindulitsa zapadera nthawi zina.
Mawu Ochokera
Pakalipano, kupulumuka kwa anthu omwe ali ndi myelofibrosis oyambirira kumawoneka kuti ali ndi zambiri zokhudzana ndi zizindikiro zawo ndi mawonetseredwe a matendawa kumayambiriro, ndipo sakhudzidwa kwambiri ndi mankhwala kapena mankhwala; Komabe, ichi ndi lingaliro la chisinthiko chomwe chingasinthe monga sayansi ikuwonekera. Mankhwala atsopano amayamba kutuluka ndipo sayansi m'derali ikukula mofulumira.
Zotsatira:
> Myelofibrosis: Mfundo Zatsopano za Healthcare Professional: Kopezeka mu 2013. ScholarlyEditions, Jul 22, 2013.
> Gangat N, Caramazza D, Vaidya R, et al. DIPSS-plus: Bungwe la Refined Dynamic International Prognostic Scoring System (DIPSS) la Primary Myelofibrosis limene limaphatikizapo Chidziwitso chachinyengo kuchokera ku Karyotype, Platelet Count ndi Transfusion Status. J Clin Oncol. 2011; 29: 392-397.
> Greenberg PL, Attar E, Bennett JM, et al. Myelodylastic syntndrome JNCCN. 2013; 11 (7): 838-874.