Myelofibrosis chirwere chepfupa remafu chinokonzera marora kuva fibrotic, kana kuora. Mucheka mutsvuku mukati memaropa ekuumba marusi, mumakona matsvene emamwe mapfupa. Izvo "kuvhara" mu myelofibrosis, zvisinei, hazvifananidzi kupera kunokonzerwa nechironda cheganda rinoporesa. Bone marrow ndiyo nzvimbo yekugadzirwa kwemasero ese emuviri, uye naizvozvo tsvina inotanga ipapo haisi yanyanyisa, uye inogona kuguma kuendesa kune zvinetso nekero mazita, zvinetso zvakakomba uye kupfupisa kwehupenyu hwemazuva.
Apo fibrosis inotora, izvi zvinowedzera kuipa uye kuwedzera kukura kwemasero eropa, uye kusakanganiswa muchitokisi kuverenga uye dzimwe nguva mumasero eropa ivo pachavo. Pakupedzisira, mune myelofibrosis yakakwirira, izvi zvinogona kuita kuti marrow atadze kuita basa racho zvachose. Apo myelofibrosis inofambira mberi kana kuchinja, zvinetso zvekutarisa upenyu zvakadai seyeukemia uye zvakanyanya kuvhara ropa uye zviitiko zvekubuda ropa zvinobvira. Izvo zvinogona zvakare kuti myelofibrosis inotungamirira kune chirwere chinokonzerwa neukemia , kenza yeropa inouraya upenyu.
Primary Primary / Secondary Myelofibrosis
Myelofibrosis inogona kuva yekutanga kana yechipiri. Apo inomuka anew, kana kuti inoonekwa yose iri yoga kwekutanga, inonzi primary myelofibrosis. Apo inotanga kushamwaridzana nedzimwe zvirwere, kukuvadza kana chirwere cheropa kunonzi yechipiri myelofibrosis. Semuenzaniso, myelofibrosis mushure mehutano hweropa huzivikanwa se polycythemia vera imwe yerudzi rwechipiri myelofibrosis.
Ndizvo Zvakangofanana neCancer?
Iine zvinhu zvakawanda zvakafanana nekenza, asi nyanzvi dzinotaura nezvazvo se "myeloproliferative neoplasm ." Kuchengeta zvinhu zviri nyore, shoko rokuti neoplasm rinogona kufungidzirwa sezwi rakanaka kune "chirwere kana kukura" uye matumire kana kukura kunogona kunge kwakanaka kana yakaipa. Myelofibrosis haisi kuipa, asi hainawo humwe hutano hwevanhu hwave huri kutarisira pavanofunga nezvekenza, kana kuti maharaplasms akaipa.
Izvo zvakataura, pasinei nokuti iwe unotarisa myelofibrosis sekenza kana kukura kunokuvadza kwemapfupa emapfupa, pane ikozvino hapana mishonga inodhaka iyo inoporesa (asi bone marrow transplant inogona kunge ichirapa), uye iyo inogona kukura kuita zvinokuvadza-kunyangwe zvakasiyana zvakasiyana nevanhu vakasiyana. Nzira dzakapfurikidza dzemyelofibrosis dzinogadzirisa nguva yeupenyu uye dzinoita chiremera chikuru cheutano kune varwere.
Iwe uchawana zvakawanda zvemashoko anotsungirirwa anoreva myelofibrosis se "kenza yeropa isingawanzoitiki." Iyi inogona kunge iri nzira yakanakisisa yekukurukura pfungwa yakawanda, asi panewo zvakare kune nyaya yacho. Myelofibrosis inogona kutungamirira kune kenza yeropa, asi pane dzimwe nguva, myelofibrosis inogona kubva pamusana weropa.
Aina
Kunze kwekutanga uye yechipiri, kune dzimwe nzira dzekugadzirisa myelofibrosis. Imwe nzira ndeyokutora zvikwata zvechirwere ichi muzvipingamupinyi zvakasiyana zvakasiyana zvichienderana nezvakawanikwa nevanhu kana chirwere chacho chinotanga kuonekwa. Zvishandiso zvakasiyana-siyana zvakasiyana zvinowanikwa kubatsira vanachiremba kuti vaone huwandu hwehutano hwako, kubatsira kutungamirira kurapwa nekugadzirisa kushandiswa kwako.
Kuwanda
Maererano neLeukemia & Lymphoma Society , myelofibrosis inowanikwa munenge 1.5 kubva kune vanhu 100 000 muUnited States gore negore.
Inobata varume nevakadzi uye inowanzoonekwa kune vanhu vanopfuura makore makumi matanhatu, asi inogona kuitika chero ipi zvayo. Inofungidzirwa kuti vanenge 16 000 kusvika 18,500 vanhu muUnited States vane myelofibrosis.
Zvinokonzera
Mhando dzakawanda dzemakero masero nemagene anozivikanwa kuti akabatanidzwa, zvisinei, chikonzero chaiicho chekuvhara zvakanyanya myelofibrosis hachisi zvachose chakajeka. Zvizhinji zvemasvikiro uye chromosomal zvisiri izvo zvakawanikwa, kusanganisira kushandurwa kunonzi "JAK2 V617F kusina mutemo mutation," kunyange zvazvo kuva nekushanduka uku hakurevi kuti iwe pachako uchanyanya kukura myelofibrosis. Chikonzero chekuchinja kwakadaro hazvizivikanwi, uye hapana kujekesa kwakananga kana zvimwe zvinhu zvinogona kuisa mungozi zvinogona kubatanidzwa kune myelofibrosis yepamusoro mune dzimwe nguva.
Mukuwedzera kune mararamiro eropa, mamwe marwadzo asina ropa anokonzerwa nekenza anozivikanwa se "myeloproliferative neoplasms" akadai se polycythemia vera uye hutachiona hunokosha hunogona kutungamirira kune wechipiri myelofibrosis. Secondary or interactive myelofibrosis inogonawo kuitika mukugadzirisa kemikemikari kana kukuvadzwa mumuviri, utachiona kana kurasikirwa kweropa kunopa marora.
Primary myelofibrosis, polycythemia vera-yakabatana nemyelofibrosis, uye zvinokonzerwa ne-thyelocythemia-myelofibrosis yakabatana dzimwe nguva inobatanidzwa pamwe chete se "myelofibrosis," asi masayendisiti anoti pane zvimwe zvinogona kudzidza uye kunzwisisa nezvekusiyana pakati pemhando imwe neimwe.
Zviratidzo
Varwere vazhinji havana zviratidzo panguva yekuongororwa, asi zviratidzo zvinowanzosanganisira zvinotevera:
- Kuneta
- Kuonda
- Night sweats
- Fever
- Manzwiro ekuoma kufema kufema
- Kushungurudzika mudumbu (nemhaka yepleen yakawedzerwa)
Kukundikana kwemafupa kunogona kutungamirira kuzviratidzo kubva pasi pekushaya ropa, zvakadai sokuneta kubva kuva nemashoma masero masero matsvuku eropa. Kusvibiswa pamaplatelet eropa kunogonawo kutungamirira kumaropa uye kuvhara matambudziko.
Zvimwe zviratidzo, zvakadai sekuzara kwepamuviri kana kuomanikidza, zvinogona kuva nekuda kwezvose zviri kuitika kunze kwemafupa akaoma, kuitira kuti iite masero matsva eropa:
- Kazhinji, kushandiswa kwemasero eropa kunosvika kumapfupa emapfupa mucheche paanenge ari pedyo kana panguva yakaberekwa. Vasati vaberekwa, zvisinei, vana vanogona kubudisa masero matsva eropa panzvimbo dzakadai sa spleen, chiropa, uye lymph node-idzi nzvimbo dzisiri kunze kwemafupa dzinonzi extramedullary.
- Kazhinji muvanhu vakuru, nzvimbo yega yega yega masero ekugadzirwa kweropa ndiyo bone marrow. Mune dzimwe ropa dzekenza uye zvirwere zveropa, vanhu vanodzokera kubudisa masero eropa chero kupi zvavanogona, pane idzi dzinowanikwa pasi. Mu myelofibrosis, izvi zvinonyanya kuitika mumutsara uye chiropa. Dzimwe nguva chirwere chomunhu chinogona kuva chikuru nekuda kwe extramedullary hematopoiesis mu myelofibrosis.
Zvimwe zvinetso zvemyelofibrosis zvinowanzobva mukusakatika kwemapfupa uye extramedullary hematopoiesis .
Pane ngozi yakawedzerwa yekushandurwa kuhutachiona hwemyeloid leukemia (AML) ne myelofibrosis, uye vanenge 20 muzana yevanhu vane myelofibrosis vanokura maukemia akaoma.
Kuziva
Mukuwedzera kune ruzivo chiremba wako anowana zviratidzo zvako uye kuongorora mumuviri, kune mimwe miedzo inowedzera ruzivo rwekuongorora ruzivo. Izvi zvinosanganisira kuverenga ropa, mamwe mabasa eropa, zviitiko zvekufungidzira zvakadai se-ray-ray uye MRI, kuongorora marrow bone , uye kuedza kwejeni. Muenzaniso weropa kana mapfupa epafupa inogona kutumirwa ku laboratory kuti iongorore kuchinja kwemashoko (zvakadai seJAK2, CALR, kana kuti kuchinja kweMPL) iyo inowanzovapo kune vanhu vane myelofibrosis.
Zvimwe zvinhu zvinogona kutaridzika se myelofibrosis, asi hazvisi, zvinosanganisira marwadzo ane myelogenous leukemia, mamwe myeloproliferative syndromes, chisingararamiki myelomonocytic leukemia, uye chirwere chinokonzera myeloid yeukemia.
Kurapa
Parizvino, hapana chekushandisa zvinodhaka izvo zvinorapa. Chinangwa chevarwere vazhinji ndechekudzivirira zviratidzo, kuderedza chirwere chakawedzerwa nekuvandudza ropa rema cell. Mukubvumirana nezvinangwa izvi, chinangwa chikuru ndezvekuderedza ngozi yezvinetso.
Myelofibrosis chirwere chine maitiro mashoma anogamuchirwa ehutano, asi vazhinji vatsva vari kuongororwa uye kukurudzirwa. Kurapa kunotungamirirwa nezvimwe zvinhu zvakadai sokuvapo kwezviratidzo, uyewo pangozi yeimwe nyaya ye myelofibrosis, uyewo pazera rako uye hutano / hutano huzhinji.
Kune vanhu vane ngozi shoma uye vasina zviratidzo, kucherechedza kungangove kwakanaka. Nokuda kwechirwere chepamusoro, stem cell transplant kubva kune mupiro inowanzoonekwa, asi kwete varwere vose vakakodzera nekuda kwezvipingamupinyi. Vamwe varwere vakanyatsokodzera kugadziriswa kwechirwere chechirwere kana kuongororwa kwezvinodhaka mumishonga yechipatara.
Muna 2011, chirongwa cheDain and Drug Administration (FDA) chakagamuchirwa ruxolitinib (Jakafi) mukurapa myelofibrosis iri pakati nepakati uye yakawanda, kusanganisira myelofibrosis inokosha, post-polycythemia vera myelofibrosis, uye mushure mekukosha kweprombosemia myelofibrosis.
- Mvumo yeDAA yakabva pamigumisiro yemiedzo miviri yakarongedzwa nevarwere vane metefibrosis iri pakati nepakati kana yakawanda inofanidza ruxolitinib kune placebo (Chidzidzo 1) kana kuti kurapa kwakanakisisa kunoitwa (Chidzidzo 2). MuChidzidzo 1, 42 muzana yevarwere vanobatwa neRuxolitinib, vachienzaniswa ne1 muzana yevarwere vanobatwa ne placebo, vanoziva zvikamu zvinodarika makumi matatu kubva muzana kubva muzana yepleen size pamasvondo makumi maviri nemaviri. Panguva yekubvumirwa, 75 muzana yevarwere paChidzidzo 1 uye 67 muzana paChidzidzo 2 uyo akawana kudarika 35 kubva muzana kuderedzwa kwepleen volume yakachengetedza kuderedzwa kwepleen volume.
- Ruxolitinib inoshanda mukuderedza ukuru hwepleen uye kuderedza zviratidzo muzhinji rwevarwere. Mumakiriniki ekuedzwa kuongorora ruxolitinib kune varwere vane metefibrosis yakawanda, boka guru revatori vechikamu rakagadziridzwa zvikuru muzviratidzo zvinosangana nechirwere ichi: vakanyora diary zuva rega rega vachiona zviratidzo zvinokonzera myelofibrosis izvo zvaisanganisira kusuruvara kwepamuviri, kutanga kwekunzwa kwekuzara, kurwadziwa pasi pemiti yakasara, huchinyoro, husiku hunoputika uye bone / musonda kurwadziwa.
- Chiitiko chinowanzovhiringidza zvinodhaka, zvinowanikwa mune 1 muzana yevarwere vanobatwa neRuxolitinib, vaisanganisira pasi platelets, anemia, kupwanya, dutu, uye musoro. Kukanganisa kushandiswa kwezvinodhaka zvechirwere chechiremera chakasimba chakange chiri chepamusoro kune varwere vakabata ruxolitinib vachienzaniswa ne placebo muChidzidzo 1 chaisanganisira maplatelet maduku (akawana 13 muzana yevarwere vakabata ruxolitinib, vachienzanisa ne1 muzana yevarwere vaibatwa ne placebo) uye anemia (vakaona 45 muzana yevarwere vanobatwa neRuxolitinib, vachienzaniswa ne19 muzana yevarwere vanobatwa ne placebo). Zvimwe zvakawanikwa zvakaonekwa muChidzidzo 2.
Mimwe mishonga inowanikwa kuti inoshanda pakuwedzera kwepleen nekudzivirira kwezviratidzo zvinosanganisira chemotherapies, kubviswa kwepleen kana splenectomy, uye chirwere chakanyanya kuderedza mazai kune spleen. Kuwedzerwa ropa kunogona kupiwa nokuda kweanemia, uye kune varwere vane ropa rekuwedzerwa ropa, vanoita marrow-zvinokonzera zvinodhaka zvakadai se erythropoietin, uyerogens (somuenzaniso, danazol), uye immunomodulator (somuenzaniso, lenalidomide) inogona kushandiswa.
Prognosis
Kubva pane zvidzidzo zvekare, mamwe mapoka evanhu akaonekwa kuti ane myelofibrosis akararama kwemakore mazhinji, asi mune mamwe mapoka, nguva dzekupona dzakanga dzisingasviki makore matatu kusvika ku5 kubva pakuongororwa. Inenge 60 kubva muzana yevarwere vane primary / idiopathic myelofibrosis vanorarama makore mashanu. Kune boka guru revarwere, zvisinei, vanogara makore 10 kana kupfuura.
Avo vanowanzoita zvakanaka kwazvo vanosanganisira avo vane mahemoglobine mazana makuru kupfuura 10 g / dL, platelet inotakura kupfuura 100x3 / L uye avo vane kuderera kuduku kwechiropa. Simba hutachiona uye zvepabonde hazviratidzika kuva nechinonyanya kukosha mukurarama mune zvidzidzo, kunyange zvazvo muchirevo, kuderedza uswa hwepleen kunogona kushandura kuhupenyu huripo mune dzimwe nguva.
Shoko Rinobva
Kusvika ikozvino, kupona kwevanhu vane myelofibrosis yekutanga kunoratidzika kune zvakawanda zvine chokuita nezviratidzo zvavo zvega uye zviratidzo zvechirwere pakutanga, uye hazvirevi zvakanyanya kune imwe nzira yekurapa kana kurapa; zvisinei, ichi chirevo mune kushanduka kwezvinhu zvinogona kuchinja sezvo sainzi inowedzera. Zvitsva zvitsva zvinoramba zvichiri kubuda uye sayenzi munharaunda ino iri kuwedzera nokukurumidza.
Sources:
> Myelofibrosis: Mutsva Insights yeHealthcare Professional: Gwaro re2013. ScholarlyEditions, Jul 22, 2013.
> Gangat N, Caramazza D, Vaidya R, et al. DIPSS-plus: A Refined Dynamic International Prognostic Scoring System (DIPSS) ye Primary Primary Myelofibrosis Iyo Inosanganisira Mashoko Okuzivisa kubva Karyotype, Platelet Count uye Transfusion Status. J Clin Oncol. 2011; 29: 392-397.
> Greenberg PL, Attar E, Bennett JM, et al. Myelodysplastic Syndromes: Mishonga yeChidzo yeKunyorera muIncology. JNCCN. 2013; 11 (7): 838-874.