I-Myelofibrosis iyisifo somnkantso wesifuba esibangela ukuba umnkantsha ube yi-fibrotic, noma i-scarred. Izicubu ezibomvu zifake ngaphakathi kwezicubu zomnyozi wegazi, emithanjeni engcwelengcwele yamathambo athile. Kodwa "ukukhishwa" ku-myelofibrosis, okungafani nokulimala okubangelwa ukulimala kwesikhumba sokuphulukisa. Umnkantsha we-bone ungumzila wokukhiqiza wonke amangqamuzana egazi lomzimba, ngakho-ke inqubo yokuqeda eqalayo ayiyona into enobungozi, futhi ekugcineni ingabangela izinkinga ngezibalo zeseli, izinkinga ezinkulu kanye nokunciphisa isikhathi sokuphila.
Lapho i-fibrosis ithatha, lokhu kuholela ekukhiqizeni kwegazi kwegazi nokubi kakhulu, okungajwayelekile emalini esele futhi ngezinye izikhathi kumaseli egazi ngokwabo. Ekugcineni, ku-myelofibrosis ethuthukile, lokhu kungabangela ukuba umnkantsha uhluleke ukwenza umsebenzi wayo ngokuphelele. Uma i-myelofibrosis iqhubekela phambili noma iguqukela, izinkinga ezisongela ukuphila ezifana ne-leukemia kanye nezenzakalo ezinzima zokugaya igazi kanye nemicimbi yegazi kungenzeka. Kungenzeka futhi ukuthi i-myelofibrosis iholele ekubeni ne-leukemia enamandla , umdlavuza wegazi obungozi.
I-Primary and Secondary Myelofibrosis
I-Myelofibrosis ingaba eyinhloko noma eyesibili. Uma ivela okusha, noma ithola konke ngokwayo okokuqala, ibizwa ngokuthi i-myelofibrosis eyinhloko . Uma iqala ukuhlangana nezinye izifo, ukulimala noma isimo segazi kuthiwa yi- secondary myelofibrosis. Isibonelo, i-myelofibrosis emva kwesifo segazi esibizwa ngokuthi i- polycythemia vera yindlela eyodwa ye-myelofibrosis yesibili.
Ingabe Kufana Njalo Njengomdlavuza?
Kunezinto eziningi ezifanayo nomdlavuza, kodwa ochwepheshe babhekisela kuso ngokuthi "i-neoplasm" ye-myeloproliferative . "Ukuze ugcine izinto zilula, igama le- neoplasm lingacatshangwa njengegama eliyinkimbinkimbi ngokuthi" isisu noma ukukhula "futhi izicubu noma ukukhula kungaba yinhle noma okubi. I-Myelofibrosis ayiyona into ehloniphekile, kodwa futhi ayinakho izici abantu abaye balindela lapho becabanga ngomdlavuza, noma ama-neoplasms amabi.
Lokhu kusho ukuthi kungakhathaliseki ukuthi ubheka i-myelofibrosis njengomdlavuza noma ukukhula okulimazayo kwamathambo omzimba wamathambo, okwamanje akukho ukwelashwa kwezidakamizwa okwelapha (kodwa ukufakelwa komnyoba we-bone ngomzimba kungaba yikwelapha), futhi kuyinkqubo engathuthuka ukuze ibangele ukulimaza-noma ukuhamba okuhlukile kubantu abahlukene. Izinhlobo eziphambili ze-myelofibrosis zikhawulela isikhathi sokuphila futhi zakha umthwalo wempilo obalulekile ezigulini.
Uzothola inqwaba yezingosi ezibhekene neziguli ezibhekisela ku-myelofibrosis ngokuthi "umdlavuza wegazi ongavamile." Lokhu kungase kube indlela ephumelelayo yokuxhumana nomqondo jikelele, kodwa kukhona nokunye endabeni. I-Myelofibrosis ingabangela umdlavuza wegazi, kodwa kwezinye izimo, i-myelofibrosis ingabangela umdlavuza wegazi.
Izinhlobo
Ngaphandle kwamabanga aphansi nesekondari, kunezinye izindlela zokuhlukanisa i-myelofibrosis. Enye indlela ukubhekana nezigameko zeqembu lesi sifo zibe yizigaba ezihlukahlukene zokufaka ingozi kuye ngokuthi izifo zitholaphi lapho isifo sifika kuqala. Amathuluzi amaningana ahlukene atholakalayo ukusiza odokotela banqume izinga lakho lokubeka ingozi, ukusiza ukuqondisa ukwelashwa nokulungisa ukubikezela kwakho.
Ukuvama
Ngokusho kwe -Leukemia & Lymphoma Society , i-myelofibrosis ivela kubantu abangaba ngu-1.5 kwabangu-100 000 e-United States unyaka ngamunye.
Lithinta kokubili amadoda nabesifazane futhi kuvame ukutholakala kubantu abangaphezu kweminyaka engama-60, kodwa kungenzeka noma kunini. Kulinganiselwa ukuthi abantu abangaba ngu-16 000 kuya ku-18 500 e-United States bane-myelofibrosis.
Izimbangela
Izinhlobo eziningi zamangqamuzana nezakhi zofuzo ziyaziwa ukuthi zihilelekile, noma kunjalo, imbangela eqondile yokuqeda ngokweqile imyelofibrosis akucaci ngokuphelele. Kuningi okungavamile okwakusetshenziselwa izakhi zofuzo kanye ne-chromosomal, kufaka phakathi ukuguqulwa okubizwa ngokuthi "JAK2 V617F ukuguqulwa kwamasonto," noma ngabe lokhu kuguqulwa akusho ukuthi uzokuthuthukisa imyelofibrosis eyinhloko. Isizathu sokushintsha okunjalo akungaziwa, futhi akukho ukuchayeka okuqondile noma izici eziyingozi ezingase zihlotshaniswe nemyelofibrosis eyinhloko ezimweni eziningi.
Ngaphandle kwamagciwane egazi, ezinye izifo zegazi ezingenayo umdlavuza ezibizwa ngokuthi "ama-myeloproliferative neoplasms" njenge-polycythemia vera kanye ne-thrombocythemia ebalulekile ingase iholele kumanyolo wesibindi. I-myelofibrosis yesibili noma esebenzayo ingaba khona ekuphenduleni ukulimala kwamakhemikhali noma emzimbeni, ukutheleleka noma ukulahlekelwa kwegazi emnothweni wethambo.
I-myelofibrosis eyinhloko, i-polyyelothemia-vera-imyelofibrosis ehlobene ne-polycythemia vera, kanye ne-myelofibrosis ehlobene ne-thrombocythemia ehambisana ne-thrombocythemia sonke ngezinye izikhathi ibumbana ndawonye ngokuthi "myelofibrosis," kepha ososayensi bathi kukhona okungaphezulu kokufunda nokuqonda ngokungafani phakathi kohlobo ngalunye.
Izimpawu
Iziguli eziningi azikho izimpawu ngesikhathi sokuxilongwa, kodwa izimpawu ezivamile zifaka lokhu okulandelayo:
- Ukukhathala
- Ukulahlekelwa kwesisindo
- Izithukuthuku ebusuku
- Fever
- Umzwa wokuba nokuphefumula kanzima
- Ukuphazamiseka esiswini (ngenxa yengubo ekhulisiwe)
Ukwehluleka komnyobo we-bone kungabangela izimpawu ezivela ezingeni eliphansi legazi, njengokukhathala kokungena embalwa amangqamuzana egazi obomvu kakhulu. Umthelela emaphaketheni egazi kungabangela nezinkinga zokugaya nokugaya.
Ezinye izibonakaliso, njengokugcwala kwesisu noma ukucindezelwa, kungenzeka ngenxa yalokho okwenzeka ngaphandle komnkantsha omnyama, ukuze wenze amangqamuzana egazi amasha:
- Ngokuvamile, ukukhiqizwa kwe-cell cell ukushintshela emnothweni wezintana ezinganeni noma eduze nesikhathi sokuzalwa. Ngaphambi kokuzalwa, izinsana zingakwazi ukhiqiza amangqamuzana egazi amasha ezindaweni ezifana ne-spleen, isibindi, nama-lymph nodes-lezi zindawo ezingaphandle komnkantso we-bone zibizwa ngokuthi i-extramedullary.
- Ngokuvamile kubantu abadala, indawo eyodwa kuphela yokwakheka kwamangqamuzana egazi legazi yinkompa yethambo. Ezingxenyeni ezithile zegazi zegazi kanye nokuphazamiseka kwegazi, abantu babuyela emuva ekukhiqizeni amaseli egazi lapho bekwazi khona, kulezi zindawo ezingaphandle. Ku-myelofibrosis, lokhu kuvame ukuvela ku- spleen nesibindi. Ngezinye izikhathi isilwane somuntu singaba mkhulu ngenxa ye-hematopoiesis exramedullary ku-myelofibrosis.
Izinkinga ezinkulu ze-myelofibrosis ngokuvamile zivela ekuhlulekeni komnkantso we-bone kanye ne -hematopoiesis exramedullary .
Kukhona ingozi eyengeziwe yokuguqulwa kwesifo se-myeloid leukemia (AML) esine-myelofibrosis, futhi cishe amaphesenti angu-20 abantu abane-myelofibrosis bahlakulela i-leukemia ephawulekayo.
Ukuxilongwa
Ngaphandle kolwazi udokotela wakho uthola ngokusebenzisa izimpawu zakho nokuhlola ngokomzimba, kunezivivinyo eziningana ezinikeza ulwazi olubalulekile lokuxilonga. Lokhu kufaka phakathi ukubala kwegazi, enye imisebenzi yegazi, ukuhlolwa kwezithombe zokucabanga ezifana ne-x-ray kanye ne-MRI, izivivinyo ze-bone marrow , nokuhlolwa kofuzo. Isampula segazi noma umthambo we-bone angathunyelwa ebhokisithri ukuze ifune izakhi zofuzo (njenge-JAK2, CALR, noma i-MPL mutations) okuvame ukukhona kubantu abane-myelofibrosis.
Ezinye izinto ezingabonakala njenge-myelofibrosis, kodwa azibandakanyi, zihlanganisa i-leukemia engavamile, i-myeloproliferative syndromes, i-myelomonocytic leukemia engapheli, ne-lemonemic acute leememiaid.
Ukwelapha
Njengamanje, akukho okukhethwa kwezidakamizwa okwelaphayo. Umgomo weziguli eziningi ukukhulula izimpawu, ukunciphisa ipeni ekhulisiwe nokuthuthukisa izibalo ze-cell cell. Ekhonsathini ngalezi zinhloso, umgomo obaluleke kakhulu ukunciphisa ingozi yezinkinga.
I-Myelofibrosis yisifo esinokukhetha ukwelashwa okuncane okuvunyelwe, kepha ama-agent amaningi amasha ayaphenywa futhi athuthukiswe. Ukwelashwa kuqondiswa izici zakho ezithile ezifana nobukhona bezimpawu, kanye nobungozi besimo sakho se-myelofibrosis, kanye nesikhathi sakho sempilo kanye nendawo yonke / impilo jikelele.
Kubantu abanengozi encane kakhulu futhi bengekho izimpawu, ukubonwa kuphela kungase kube kuhle. Ngezifo ezinobungozi obukhulu, ukuguqulwa kwe-stem cell kusuka kumnikeli ngokuvamile kubhekwa, kepha akuzona zonke iziguli ezifanelekile ngenxa yezingozi. Ezinye iziguli zifuna ukwelapha izidakamizwa ezivamile noma ukwelashwa kwezidakamizwa zophenyo emvivinyweni wezokwelapha.
Ngo-2011, i-Food and Drug Administration (i-FDA) ivume i-ruxolitinib (Jakafi) yokwelashwa kwe-myelofibrosis ephakathi nendawo ephezulu, kubandakanya i-myelofibrosis eyinhloko, i-post-polycythemia vera myelofibrosis, kanye ne-thrombocythemia myelofibrosis ebalulekile.
- Imvume ye-FDA isekelwe emiphumeleni yezilingo ezimbili ezilawulwe ngokungahleliwe ezigulini ezine-intermediate or risk-risk myelofibrosis eziqhathanisa i-ruxolitinib endaweni ye-placebo (Isifundo 1) noma ukutheleleka okungcono okukhona (Ucwaningo 2). Esifundweni 1, amaphesenti angu-42 weziguli eziphathwa nge-ruxolitinib, uma kuqhathaniswa namaphesenti angu-1 eziguli eziphathwa nge-placebo, zathola okungenani amaphesenti angu-35 ukunciphisa usayizi wepen emavikini angu-24. Ngesikhathi sokuvunywa, amaphesenti angama-75 weziguli ezisesifundo se-Study 1 nama-67 emaphesenti eSifundo sesi-2 abafinyelele okungenani amaphesenti angu-35 ekunciphiseni komthamo we-spleen agcine lokhu kunciphisa umthamo we-spleen.
- I-Ruxolitinib iphumelela ekunciphiseni usayizi we-spleen nokunciphisa izimpawu eziningi zeziguli. Ku-ruxolitinib yokuhlolwa kokuhlolwa komtholampilo ezigulini ezine-myelofibrosis engozini enkulu, iqembu elikhulu labahlanganyeli linokuthuthukiswa okuphawulekayo ezimpawu ezihambisana nalesi sifo: babhala idayari yansuku zonke ekutholeni izimpawu ezikhubazayo ze-myelofibrosis ezihlanganisa ukunganaki kwamathumbu, ukuzwa kwangaphambili kokugcwala, ubuhlungu ngaphansi kwezimbambo ezingakwesobunxele, ubuhlungu, ukujula ebusuku kanye nobuhlungu / amathambo.
- Ukuziphendulela kwezidakamizwa ezivame kakhulu, kubone okungenani amaphesenti angu-1 weziguli eziphathwe nge-ruxolitinib, zihlanganisa amaplatelets aphansi, i-anemia, ukulimaza, isizungu, nokusizwa ikhanda. Ukusabela kwezidakamizwa ezimbi zesibindi esiphezulu esasiphezulu kwiziguli eziphathwe nge-ruxolitinib uma kuqhathaniswa ne-placebo eSifundweni 1 sifakwe amapulethi aphansi (ahlangene namaphesenti angu-13 eziguli eziphathwa nge-ruxolitinib, uma kuqhathaniswa namaphesenti angu-1 eziguli eziphathwa nge-placebo) ne-anemia (ehlangene Amaphesenti angu-45 iziguli eziphathwa nge-ruxolitinib, uma kuqhathaniswa namaphesenti angu-19 weziguli eziphathwa nge-placebo). Imiphumela efanayo ifakiwe eSifundweni 2.
Ezinye izindlela zokwelashwa ezitholakala ukuthi ziyasebenza ekwindeni ekhulisiwe nokulawulwa kwezimpawu zihlanganisa i-chemotherapies, ukususwa kwe-spleen noma i-splenectomy, kanye nokwelashwa kwama-dose aphansi e-pleen. Ukumpontshelwa igazi kunganikezwa i-anemia, kanye neziguli ezine-anemia ezixhomeke ekumpomeni, izidakamizwa ezishukumisa umnyo-bone ezifana ne-erythropoietin, i-androgens (isibonelo, i-danazol), kanye nama-immunomodulator (isibonelo, lenalidomide) angasetshenziswa.
Isibikezelo
Ngokusekelwe ezifundweni ezedlule, amanye amaqembu abantu abatholwa nge-myelofibrosis ahlala iminyaka eminingi, kanti nakwamanye amaqembu, izikhathi zokuphila zazingaphansi kweminyaka engama-3 kuya kwengu-5 kusukela kuhlolwe. Cishe amaphesenti angama-60 weziguli ezine-myelofibrosis eziyisisekelo / idiopathic ziphila iminyaka emihlanu. Kukhona iqembu elibalulekile leziguli, kodwa, abahlala iminyaka eyishumi noma ngaphezulu.
Labo abajwayele ukwenza kahle bahlanganisa nalabo amazinga e-hemoglobin angaphezulu kuka-10 g / dL, i-platelet ibalwa ngaphezulu kuka-100x3 / L nalabo abanesibindi esincani sebindi. Usayizi wesipelingi nobulili abubonakali ukuthi kunomthelela omkhulu ekusindisweni kwezifundo, nakuba kunengqondo, ukunciphisa usayizi we-spleen kungashintsha ekuzuzeni izinzuzo kwezinye izimo.
Izwi elivela
Kuze kube manje, ukusinda kwabantu abane-myelofibrosis eyinhloko kubonakala kunezinto eziningi eziphathelene nezimpawu zabo kanye nokubonakaliswa kwesifo ekuqaleni, futhi akuthinti kakhulu noma yimuphi ukwelashwa noma ukwelashwa okulodwa; Nokho, lokhu kungumqondo wokuziphendukela kwemvelo okungashintsha njengoba isayensi ivela. Izindlela ezintsha zokwelashwa ziqhubeka zivela futhi isayensi kule ndawo isanda ngokushesha.
Imithombo:
> Myelofibrosis: Intsha entsha ye-Healthcare Professional: U-2013 Edition. I-ScholarlyEditions, uJulayi 22, 2013.
> Gangat N, Caramazza D, Vaidya R, et al. I-DIPSS-plus: Isistimu Yokubalwa Kwamaprosesa Wokuhlaziya Dynamic International (DIPSS) we-Primary Myelofibrosis Efakazela Ulwazi Lokubikezela kusuka ku-Karyotype, Platelet Count ne-Transfusion Status. J Clin Oncol. 2011; 29: 392-397.
> Greenberg PL, Attar E, uBennett JM, et al. Ama-Syndromes e-Myelodysplastic: Imihlahlandlela Yokuzivocavoca Emtholampilo ku-Oncology. I-JNCCN. 2013; 11 (7): 838-874.