Ko mwongo wemapfupa ako unoburitsa fibrous tishu here? Ngatidzidzei nezve myelofibrosis!

Wakambonzwa nezvechirwere chinonzi myelofibrosis here? Chaizvoizvo igomarara reropa risingawanzoitiki. Zvichitaurwa zviri nyore, ndipo apo mupfupa wemapfupa , unova chikamu chakapfava, chine mapfupa, unotsiviwa nenyama ine mavanga efibrous . Irowo rudzi rweleukemia yenguva refu, uye iri muboka rinonzi myeloproliferative disorders. Myeloproliferative disorders inoitika kana masero eropa akawanda kwazvo achigadzirwa mupfupa, uko masero eropa anogadzirwa.

Chii chinonzi myelofibrosis chaizvo?

Ngatitarisei izvi zvakadzama. Mupfupa wako une masero ekutanga anogadzira ropa. Masero aya anozokura kuita masero matsvuku eropa, masero machena eropa, uye maplatelet. Asi mumunhu ane myelofibrosis, shanduko inoitika muzvinhu zvemajini, kana kuti DNA, mune rimwe remasero aya. Izvi zvinoita kuti sero rive sero rekenza risina kunaka. Sero iri risina kunaka rinozopatsanurana uye rinoendesa shanduko iyi kumasero matsva ese aro.

Nekufamba kwenguva, masero egomarara aya asingawanzoitiki anoungana akawanda. Mamwe emasero aya anokonzera kuzvimba mumongo wemapfupa. Kuzvimba uku ndiko kunoita kuti mavanga andambotaura aumbe. Saka, nekuda kweganda iri remavanga uye masero egomarara akawandisa, mungo wemapfupa unorasikirwa nekugona kwawo kugadzira masero eropa ane hutano.

Ndeapi marudzi makuru e myelofibrosis?

Kune mhando mbiri huru dze myelofibrosis.

• Myelofibrosis yekutanga: Iyi ndiyo mhando inoitika yoga, isina hukama nechimwe chirwere.
• Myelofibrosis yechipiri: Izvi zvinokonzerwa nechimwe chirwere cheropa. Semuenzaniso, zvinogona kukonzerwa nezvirwere zvakaita seprimary thrombocytosis kana polycythemia vera. Rudzi urwu rwechipiri runotora pakati pe10% ne20% yevarwere vakaonekwa kuti vane chirwere ichi.

Chirwere ichi chinonzi Myelofibrosis chakajairika zvakadii?

Myelofibrosis chirwere chisingawanzoitiki . Semuenzaniso, muUnited States, zvinonzi chirwere ichi chinoitika muvanhu vanosvika 1.5 pavanhu 100,000 pagore. Chinogona kubata chero munhu, zvisinei nezera. Hapana musiyano wezera, asi chinowanzoitika muvanhu vane makore anopfuura 50. Kana myelofibrosis ikaitika muvana vadiki, inowanzoonekwa vasati vasvitsa makore matatu.

Myelofibrosis inogona kukuvadza sei muviri wako?

Kana masero eropa akaumbwa zvisina kujairika nenzira iyi, matambudziko akasiyana-siyana anogona kuitika. Ngatitarisei kuti chii:

• Anemia: Iyi inyaya yekuti hapana masero matsvuku eropa akakwana. Sezvaunoziva, masero matsvuku eropa ndiwo anotakura okisijeni mumuviri wese. Saka kana pasina masero matsvuku akakwana, nyama dzemuviri hadziwani okisijeni yakakwana. Izvi zvinogona kukonzera zviratidzo zvakaita sekuneta , kushaya simba, uye kufema zvishoma .

• Thrombocytopenia: Ichi chirwere apo maplatelet mashoma. Maplatelet ndiwo anobatsira kuumba ropa kana paine kukuvara. Saka kana huwandu hwemaplatelet ako huri pasi, muviri wako unogona kuva nyore kubuda ropa uye kukuvadzwa .

• Splenomegaly: Chipfuva chako chinodzora kuwanda kwemasero eropa uye chinobvisa masero matsvuku eropa akakuvara mumuviri wako. Saka kana ukaita masero eropa akawanda asina kujairika, chipfuva chako chinofanira kushanda nesimba. Chinogona kukura. Kana chipfuva chako chikakura, unogona kunzwa kuzara kana kusagadzikana mudumbu rekumusoro kuruboshwe .

• Kubuda kweropa muropa kunze kwemupfupa: Uku ndiko kukura kwemasero anogadzira ropa kunze kwemupfupa , mune dzimwe nzvimbo dzemuviri. Masero aya anogona kuumbwa munzvimbo dzakadai semapapu, mudumbu, mumutsipa, muuropi, kana muma lymph nodes. Masero aya anogona kuungana pamwe chete kuti aumbe bundu , iro rinogona kupinda mune dzimwe nhengo dzemuviri kana kukanganisa mashandiro adzo.

• Kukwira kweropa mutsinga yeropa: Uku kuwedzera kweropa mutsinga inotakura ropa kubva muspleen kuenda kuchiropa. Izvi zvingangodaro zvichikonzerwa nekukuvara kwetsinga nekuda kweropa riri kunze kwemedullary rambotaurwa.

Chimwe chinhu ndechekuti kune vanhu vanosvika 12% vane myelofibrosis yekutanga, chirwere ichi chinogona kukura kuita mhando yekenza yeropa inonzi acute myeloid leukemia .

Chii chinokonzera myelofibrosis?

Masayendisiti haasati aziva kuti chii chinokonzera myelofibrosis. Asi vakaona kuti ine chekuita nekuchinja kweDNA yemajini mamwe . Rudzi rweprotein inonzi Janus-associated kinases (JAKs) rwunobatanidzwa. Mapuroteni aya eJAK anodzora kugadzirwa kwemasero eropa mumongo wemapfupa, achitumira zviratidzo kumasero kuti apatsanuke uye akure. Kana mapuroteni aya eJAK akanyanya kushanda, masero eropa akawanda kana mashoma anogona kugadzirwa.

Pakati pe60% ne65% yevanhu vane myelofibrosis vane shanduko mujini reJAK2 . Vamwe 5% kusvika 10%Pane shanduko mujini re myeloproliferative leukemia (MPL). Zvakare, shanduko inonzi Calreticulin (CALR) inoonekwa pakati pe20% ne25% yevarwere vane myelofibrosis.

Ndiani ari panjodzi huru yeizvi?

Unogona kunge uri panjodzi yakakura yekukura kwe myelofibrosis nekuda kwezvikonzero zvinotevera:

• Kana une makore anopfuura makumi mashanu .
• Kana uine chirwere cheropa chinonzi primary thrombocytosis kana polycythemia vera.
• Kana wakambosangana nemwaranzi ye ionizing kana petrochemicals senge benzene ne toluene.

Ndezvipi zviratidzo zve myelofibrosis?

Myelofibrosis chirwere chinokura zvishoma nezvishoma , saka ungasava nezviratidzo kwemakore. Chikamu chimwe muzvitatu chevarwere hachina zviratidzo pakutanga.

Kana zviratidzo zvikaitika, zvinonyanya kuitika kuneta zvakanyanya (nekuda kwekushaya ropa) uye chiropa chakakura . Zviratidzo zvinogona kusanganisirawo:

• Basa rakaoma
• Kupindwa nechando
• Kukwenya
• Ganda rakachenuruka
• Kuderedza huremu
• Kudikitira husiku
• Kurwadziwa kwemapfupa kana majoini
• Kutapukirwa kakawanda
• Chiropa chakakura kana kuti pleura
• Kuvhara ropa kusingatsananguriki
• Kubuda ropa kusingawanzoitiki kana mavanga
• Kukura kwetsinga dziri mudumbu nepagurokuro (tsinga idzi dzinogona kuputika nekubuda ropa).

Nzira yekuziva sei chirwere ichi?

Chiremba, kunyanya chiremba wekenza, achakuongorora muviri wako, achakubvunza nezvenhoroondo yako yehutano, uye achakubvunza nezvezviratidzo zvako zvemazuva ano. Vachaongororawo kana chiropa chako chakura uye zviratidzo zvekushaya ropa.

Zvadaro, bvunzo dzakasiyana-siyana dzinoitwa kuti pasave nedzimwe zvirwere uye kuti pave nechokwadi chekuti une myelofibrosis here.

Kuongororwa kweropa

• Kuverengwa kweropa kwakazara (CBC): Izvi zvinoyera huwandu hwemarudzi akasiyana emasero muropa rako. Kana masero matsvuku eropa ako ari pasi peakajairika, kana masero machena eropa rako nemaplatelet asina kujairika, zvinogona kuva chiratidzo che myelofibrosis.
• Kuongororwa kweropa (PBS): Izvi zvinoongorora saizi, chimiro, uye humwe hunhu hwemasero ako eropa kuti aone kana paine zvisina kumira zvakanaka. Kana paine masero asingatarisike zvakanaka uye masero eropa akawanda asina kukura zvakanaka, zvinogonawo kuva chiratidzo che myelofibrosis.
• Kuongororwa kwemakemikari eropa:Kuongororwa uku kunoyera huwandu hwezvinhu zvakasiyana-siyana zvinoburitswa muropa kubva munhengo dzako. Izvi zvinogona kukuudza kuti nhengo iri kushanda zvakanaka sei. Kuwanda kwezvinhu zvakaita se uric acid, bilirubin, uye lactic dehydrogenase muropa kunogonawo kuva chiratidzo che myelofibrosis.

Kuongororwa kwemwongo wemapfupa

• Kuongororwa kwemapfupa emubofu: Izvi zvinosanganisira kutora sampuli diki yemapfupa ako woiongorora uchishandisa maikorosikopu. Masero acho anoongororwa kuti aone kana uine myelofibrosis.
• Kutsvaga mupfupa wemapfupa: Izvi zvinosanganisira kubvisa mvura kubva mupfupa wemapfupa uye kuyedza zviratidzo zve myelofibrosis.

Mimwe miedzo ingangodiwa

Mimwe miedzo ingangodiwa kuti usimbise chirwere chako:

• Kuongororwa kwekuchinja kwemajini: Chiremba wako achaedza masero ako eropa nemapfupa kuti aone kana paine shanduko dzemajini dzine chekuita nemyelofibrosis, dzakadai seJAK2, CALR, uye MPL. Mamwe marapirwo anotarisana nemasero ekenza ane shanduko yeJAK2.
• Maitiro ekutora mifananidzo: Chiremba wako anogona kuita ultrasound kuti aone kana chirebvu chakura. Vanogonawo kuita MRI kuti vaone kana paine mavanga mumongo wemapfupa. Mavanga aya anogona kuva chiratidzo che myelofibrosis.

Myelofibrosis inorapwa sei?

Kana usiri kuratidza zviratidzo, ungasada kurapwa. Zvisinei, kunyangwe kana usingade kurapwa nekukasika, chiremba wako acharamba achitarisa mamiriro ako ezvinhu.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), uye Vonjo® (pacritinib) mishonga inotenderwa neU.S. Food and Drug Administration (FDA) . Inoshandiswa kurapa myelofibrosis ine njodzi iri pakati nepakati kusvika kune ine njodzi huru. Mishonga yese mitatu iyi inoshanda seJAK inhibitors . Kureva kuti, inoderedza kushanda zvakanyanya kweJanus-associated kinase (JAK). Nekuda kweizvozvo, mishonga iyi inobatsira kuderedza zviratidzo zvine chekuita nemyelofibrosis, zvakaita sekuwedzera kwepleen, kudikitira husiku, kuvaviwa, kudzikira uremu, uye fivha.

Kune vazhinji, chinangwa chikuru chekurapa ndechekugadzirisa zvirwere zvine chekuita ne myelofibrosis, zvakaita seanemia uye kukura kwepleen.

Kurapa kweAnemia

Kune nzira dzekurapa anemia dzakadai se:

• Androgens: Kutora androgens dzakadai sedanazol kunogona kuwedzera kugadzirwa kwemasero matsvuku eropa.
• Mishonga inogadzirisa muviri: Mishonga iyi inowedzera kugona kwemasoja emuviri wako kurwisa masero egomarara, nokudaro ichideredza zviratidzo. Mishonga yakaita se interferon, thalidomide (Thalomid®), uye lenalidomide (Revlimid®) iri muboka iri. Inogonawo kupihwa pamwe chete ne glucocorticoids yakaita se prednisone.
• Mishonga yekurapa nemakemikari: Mimwe mishonga yekurapa nemakemikari inogona kuderedza zviratidzo zvinokonzerwa nekuwedzera kwemasero eropa uye kukura kwepleen. Hydroxyurea necladribine mishonga yakadaro.
• Kuwedzerwa ropa: Kana uine anemia yakanyanya, kuwedzerwa ropa nguva dzose kunogona kuwedzera huwandu hwemasero matsvuku eropa rako.

Kurapa kweSplenomegaly

Mishonga inodzivirira chirwere cheJAK, mishonga inogadzirisa masero emuviri, uye mishonga yechemotherapy inoshandiswa kudzora chiropa chakakura. Kana zvanyanya kuipa, ungangoda kubviswa chiropa chako nekuvhiyiwa (splenectomy) kana kurapwa nemwaranzi.

Kurapa nemwaranzi kunogonawo kudiwa kurapa chirwere chinonzi extramedullary hematopoiesis, umo masero eropa anokura kunze kwemongo wemapfupa.

Myelofibrosis inogona kurapwa zvachose here?

Kuisa masero eropa anokonzera allogeneic (HCT) ndiyo chete nzira yekurapa chirwere ichi. Zvisinei, iyi inzira ine njodzi uye haina kukodzera munhu wese.

Mukuita uku, masero ako eropa asina kujairika anotsiviwa nemasero anobva kune munhu ane hutano hwakanaka. Usati waisirwa masero, uchapihwa chemotherapy kana radiation therapy yekuuraya masero ako ane chirwere. Zvadaro, masero matsva anobva kune anokupa anogona kutora mabasa emuviri wako.

Kurapwa neHCT kune njodzi huru yematambudziko . Saka, kwakakodzera chete boka revanhu vakasarudzwa. Njodzi yematambudziko yakanyanya kukwira kune vanhu vane mamwe matambudziko ehutano. Kana wakakodzera izvi zvinoenderana nezvinhu zvakawanda, zvakaita sezera rako, kuoma kwezviratidzo zvako, uye mukana wekubudirira kwekurapwa.

Upenyu hunotarisirwa sei kana munhu achirarama nechirwere ichi?

Myelofibrosis igomarara rakakomba zvikuru . Avhareji yekurarama kwevarwere ava inowanzova makore matanhatu. Avhareji yekurarama zvinoreva kuti vamwe vanhu vanorarama makore asingasviki matanhatu, nepo nhamba yakafanana ichirarama kwemakore anopfuura matanhatu. Izvi hazvina kufanana kune munhu wese, zvinosiyana kubva pamunhu kusvika pamunhu.

Pane zvinhu zvakawanda zvinosarudza kufambira mberi kwechirwere chako:

• zera rako.
• Zviratidzo zvako.
• Kuwanda kwemasero eropa rako.
• Kuoma kwemavanga mumwongo wemapfupa.
• Kana paine kuchinja kwemajini (JAK2, CALR, MPL) kana kwete.

Chinhu chinonyanya kukosha ndechekutaura pachena nachiremba wako nezvekuti kuongororwa kwako kuchakanganisa sei hupenyu hwako.

Ndinozvitarisira sei? (Kuzvitarisira)

Bvunza chiremba wako kana uchigona kubatsirwa nekurapwa kwevarwere vanorwara . Mapoka ekurapa varwere vanorwara anogona kusanganisira vanachiremba, vanamukoti, vashandi vemagariro evanhu, nevamwe nyanzvi. Vanogona kukupa zvekushandisa nerutsigiro rwaunoda kuti urarame nechirwere ichi. Pamusoro pekurapwa kwaunowana kubva kuna chiremba wako wekenza, vanhu ava vanogona kukubatsira zvikuru. Nyanzvi dzekurapa varwere vanorwara dzinogona kukubatsira kuvandudza hupenyu hwako semunhu anorarama nekenza.

Chekupedzisira, zvinhu zvinonyanya kukosha kuti urangarire

Myelofibrosis igomarara reropa risingawanzoitiki umo muro wemapfupa ako unotsiviwa nenyama ine fibrous. Iri igomarara rakakomba rinoda kutariswa nachiremba nguva dzose uye/kana kurapwa. Zvichienderana negomarara rako, ungasave nezviratidzo kwemakore. Mune zvimwe zviitiko, zviratidzo zvinogona kukurumidza kukura uye zvichiita kuti zviome kuita mabasa ezuva nezuva. Zvisinei, kune marapirwo aripo ekubatsira kudzora zviratidzo zvinokanganisa hupenyu hwako hwezuva nezuva.

Panguva ino, bvunza chiremba wako nezvezvinhu zvinogona kukubatsira kugadzirisa shanduko idzi. Kutarisirwa kwevarwere vane kenza uye mapoka ekutsigirana inzira dzinobatsira zvikuru paunenge uchijaira hupenyu hwako nekenza.

Yeuka kuti hausi wega. Vanachiremba, mhuri, neshamwari varipo kuti vakubatsire. Usatya kutaura nachiremba wako nezvemibvunzo yaungave nayo.

Myelofibrosis , kenza yeropa, muro wemapfupa, kupererwa neropa, kukura kwepleen, zvinodzivisa JAK, leukemia