Ese umwongo wawe w'amagufwa utanga n'uturemangingo tw'imitsi? Reka twige ku birebana na myelofibrosis!

Wigeze wumva indwara yitwa myelofibrosis? Mu by’ukuri ni ubwoko budasanzwe bwa kanseri y’amaraso. Muri make, ni igihe umwongo w’amagufwa , ari wo gice cyoroshye kandi gifite imiyoboro y’amagufwa yawe, usimburwa n’inkovu z’imitsi . Ni ubwoko kandi bwa leukemia y’igihe kirekire, kandi iri mu itsinda ryitwa myeloproliferative disorders. Indwara za myeloproliferative ni iyo uturemangingo twinshi tw’amaraso tuvumburwa mu mwongo w’amagufwa, aho uturemangingo tw’amaraso dukorerwa.

Myelofibrose ni iki mu by'ukuri?

Reka turebe ibi mu buryo burambuye. Umwongo w'amagufwa yawe urimo uturemangingo tw'amaraso dukora amaraso. Utwo turemangingo tw'amaraso tuzahinduka uturemangingo tw'amaraso dutukura, uturemangingo tw'amaraso twera, na plaquettes. Ariko ku muntu ufite myelofibrosis, impinduka ziba mu bigize imiterere y'uturemangingo, cyangwa ADN, muri imwe muri utwo turemangingo tw'amaraso. Ibi bituma uturemangingo tuba uturemangingo tw'indwara za kanseri. Utwo turemangingo tw'amaraso dufite inenge turagabanyuka tugashyira iyo mpinduka ku turemangingo dushya twose dukora.

Uko igihe kigenda gihita, utwo turemangingo tudasanzwe twa kanseri twiyongera cyane. Tumwe muri utwo turemangingo dutera ububyimbirwe mu musokoro w'amagufwa. Uku kubyimbirwa niko gutuma utwo turemangingo twavuzwe mbere twirema. Rero, kubera iyo nkovu n'uturemangingo twa kanseri twinshi, umwokoro w'amagufwa utakaza ubushobozi bwo gukora utwo turemangingo tw'amaraso tuzima.

Ni ubuhe bwoko bw'ingenzi bwa myelofibrose?

Hari ubwoko bubiri bw'ingenzi bwa myelofibrose.

• Myelofibrose y'ibanze: Ubu ni ubwoko bw'indwara ibaho mu buryo butunguranye, nta sano ifitanye n'izindi ndwara.
• Myelofibrose ya kabiri: Ibi biterwa n'indi ndwara y'amaraso. Urugero, ishobora guterwa n'indwara nka thrombocytosis y'ibanze cyangwa polycythemia vera. Ubu bwoko bwa kabiri bugizwe n'abantu bari hagati ya 10% na 20% by'abarwayi basanzwe.

Iyi ndwara yitwa Myelofibrosis ikunze kugaragara gute?

Myelofibrosis mu by’ukuri ni indwara idakunze kugaragara cyane . Urugero, muri Leta Zunze Ubumwe za Amerika, bivugwa ko iyi ndwara igaragara ku bantu bagera kuri 1.5 mu 100.000 buri mwaka. Ishobora kwibasira umuntu uwo ari we wese, hatitawe ku myaka ye. Nta tandukaniro ry’imyaka rihari, ariko birashoboka cyane ko igaragara ku bantu barengeje imyaka 50. Iyo myelofibrosis igaragaye mu bana bato, akenshi ifatwa mbere y’imyaka 3.

Ni izihe ngaruka myelofibrosis ishobora kugira ku mubiri wawe?

Iyo uturemangingo tw’amaraso tukozwe mu buryo budasanzwe muri ubu buryo, hashobora kubaho ingorane zitandukanye. Reka turebere hamwe ibyo ari byo:

• Anemia: Iyi ni indwara aho uturemangingo dutukura tudahagije. Nkuko mubizi, uturemangingo dutukura ni two dutwara umwuka mu mubiri wose. Rero iyo uturemangingo dutukura tudahagije, ingirabuzimafatizo z'umubiri ntizibona umwuka uhagije. Ibi bishobora gutera ibimenyetso nko kunanirwa, gucika intege, no kubura umwuka .

• Thrombocytopenia: Iyi ni indwara aho platelets ziba nke cyane. Platelets ni zo zifasha kubumba amaraso iyo habayeho imvune. Rero iyo umubare wa platelets zawe uri hasi, umubiri wawe ushobora kuva amaraso menshi no gukomereka .

• Splenomegali: Udusimba twawe tugenzura umubare w’uturemangingo tw’amaraso kandi tugakuramo uturemangingo dutukura twangiritse mu mubiri wawe. Rero iyo ukoze uturemangingo twinshi tw’amaraso tudasanzwe, uturemangingo twawe tugomba gukora cyane. Dushobora kwiyongera. Iyo uturemangingo twawe twagutse, ushobora kumva wuzuye cyangwa utameze neza mu gice cyo hejuru cy’ibumoso cy’inda yawe .

• Hematopoiesis yo hanze ya medullary: Iyi ni iterambere ry'uturemangingo dukora amaraso hanze y'umusemburo w'amagufwa , mu bindi bice by'umubiri. Utu turemangingo dushobora kwirema ahantu nko mu bihaha, mu nzira y'igifu, mu ruti rw'umugongo, mu bwonko, cyangwa mu duce tw'imitsi. Utu turemangingo dushobora kwishyira hamwe tugakora ibibyimba , bishobora kwinjira mu zindi ngingo cyangwa bikabangamira imikorere yabyo.

• Umuvuduko ukabije w'amaraso mu muyoboro w'amaraso: Iki ni ukwiyongera k'umuvuduko w'amaraso mu mitsi itwara amaraso ava mu ruhu rwawe akayajyana mu mwijima. Ibi bishobora guterwa cyane no kwangirika kw'imitsi bitewe n'indwara yo mu nda iterwa n'amaraso yavuye hanze y'umuyoboro w'amaraso yavuzwe haruguru.

Ikindi kintu ni uko ku bantu bagera kuri 12% bafite myelofibrose y’ibanze, iyi ndwara ishobora kuvamo kanseri y’amaraso ikomeye cyane yitwa acute myeloid leukemia .

Ni iki gitera myelofibrose?

Abahanga mu bya siyansi ntibaramenya neza icyatera myelofibrose. Ariko basanze bifitanye isano n'impinduka muri ADN y'uturemangingo tumwe na tumwe . Hari ubwoko bwa poroteyine yitwa Janus-associated kinases (JAKs) . Izi poroteyine za JAK zigenzura ikorwa ry'uturemangingo tw'amaraso mu musokoro w'amagufwa, zikohereza ibimenyetso ku turemangingo kugira ngo twitandukanye kandi dukure. Iyo izi poroteyine za JAK zikoze cyane, uturemangingo tw'amaraso twinshi cyane cyangwa duke cyane dushobora gukorwa.

Hagati ya 60% na 65% by'abantu bafite myelofibrosis bafite impinduka mu ngirabuzimafatizo ya JAK2 . Abandi 5% kugeza kuri 10%Hari impinduka mu ngirabuzimafatizo ya myeloproliferative leukemia (MPL). Nanone, impinduka yitwa Calreticulin (CALR) igaragara hagati ya 20% na 25% by'abarwayi ba myelofibrosis.

Ni nde uri mu kaga gakomeye kuri ibi?

Ushobora kuba ufite ibyago byinshi byo kurwara myelofibrose kubera impamvu zikurikira:

• Niba ufite imyaka irenga 50 .
• Niba ufite indwara y'amaraso yitwa primary thrombocytosis cyangwa polycythemia vera.
• Niba warahuye n'imirasire ya iyonizing cyangwa ibinyabutabire bya peteroli nka benzene na toluene.

Ni ibihe bimenyetso bya myelofibrose?

Myelofibrosis ni indwara igenda ikura buhoro buhoro , bityo ushobora kumara imyaka myinshi udafite ibimenyetso. Hafi kimwe cya gatatu cy'abarwayi nta bimenyetso bafite mu ntangiriro.

Iyo ibimenyetso bigaragaye, ibikunze kugaragara ni umunaniro ukabije (uterwa no kubura amaraso) no kwiyongera k'umwobo w'amaraso . Ibimenyetso bishobora kandi kuba birimo:

• Akazi gakomeye
• Umuriro
• Kuryarya
• Uruhu rwerurutse
• Kugabanya ibiro
• Kubira ibyuya nijoro
• Ububabare bw'amagufwa cyangwa ingingo
• Kwandura indwara zikunze kugaragara
• Urwagashya cyangwa umwijima byagutse
• Amaraso adasobanutse neza
• Kuva amaraso cyangwa gukomereka bidasanzwe
• Kwaguka kw'imitsi yo mu gifu no mu muhogo (iyi mitsi ishobora guturika no kuva amaraso).

Ni gute wasuzuma iyi ndwara?

Umuganga, cyane cyane inzobere mu ndwara za kanseri, azagusuzuma umubiri, akubaze amateka y'ubuzima bwawe, kandi akubaze ibimenyetso by'ubu. Azasuzuma kandi niba urwagashya rwagutse n'ibimenyetso by'amaraso make.

Hanyuma, hakorwa ibizamini bitandukanye kugira ngo hamenyekane izindi ndwara no kwemeza niba ufite myelofibrosis.

Gupima amaraso

• Umubare wuzuye w'amaraso (CBC): Ibi bipima umubare w'ubwoko butandukanye bw'uturemangingo mu maraso yawe. Niba uturemangingo dutukura turi hasi ugereranije n'ibisanzwe, cyangwa uturemangingo tw'umweru n'udupira tw'amaraso tudafite ireme, bishobora kuba ikimenyetso cya myelofibrosis.
• Gupima amaraso mu maraso (PBS): Ibi bigenzura ingano, imiterere, n'ibindi biranga uturemangingo tw'amaraso yawe kugira ngo harebwe ko nta kibazo kirimo. Iyo hari uturemangingo tugaragara nk'utudasanzwe hamwe n'umubare munini w'uturemangingo tw'amaraso tudakuze, bishobora kuba ikimenyetso cya myelofibrosis.
• Ibipimo bya shimi y'amaraso:Ibi bizamini bipima ingano y'ibintu bitandukanye bisohoka mu maraso biva mu ngingo zawe. Ibi bishobora kukwereka uburyo urugingo rukora neza. Urugero rwinshi rw'ibintu nka aside urique, bilirubin, na lactic dehydrogenase mu maraso nabyo bishobora kuba ikimenyetso cya myelofibrosis.

Ibipimo by'umusemburo w'amagufwa

• Bisipo y'umusemburo w'amagufwa: Ibi bikubiyemo gufata icyitegererezo gito cy'umusemburo w'amagufwa yawe no kuwusuzuma hifashishijwe mikorosikopi. Uturemangingo turasuzumwa kugira ngo hamenyekane niba ufite myelofibrosis.
• Gushaka umwobo w'amagufwa: Ibi bikubiyemo gukura amazi mu mwobo w'amagufwa no kuyapima kugira ngo arebe ibimenyetso bya myelofibrosis.

Hashobora kuba hakenewe ibindi bizamini

Hashobora gukenerwa ibindi bipimo kugira ngo hemezwe ko ufite indwara:

• Isesengura ry’impinduka mu turemangingo: Muganga wawe azapima uturemangingo tw’amaraso n’utwo mu magufwa kugira ngo arebe niba hari impinduka mu turemangingo tw’amaraso zijyanye na myelofibrosis, nka JAK2, CALR, na MPL. Ubuvuzi bumwe na bumwe bwibanda ku turemangingo twa kanseri dufite impinduka mu turemangingo twa JAK2.
• Uburyo bwo gufata amashusho: Muganga wawe ashobora gukora ultrasound kugira ngo arebe niba urwagashya rwagutse. Ashobora kandi gukora MRI kugira ngo arebe niba inkovu ziri mu musokoro w'amagufwa. Iyi nkovu ishobora kuba ikimenyetso cya myelofibrosis.

Myelofibrose ivurwa ite?

Niba udafite ibimenyetso, ushobora kudakenera kuvurwa. Ariko, nubwo waba udakeneye kuvurwa byihuse, muganga wawe azakomeza gukurikirana ikibazo cyawe.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), na Vonjo® (pacritinib) ni imiti yemewe n'Ikigo gishinzwe ibiribwa n'imiti muri Amerika (FDA) . Ikoreshwa mu kuvura myelofibrosis iri hagati y’ibice bigera kuri bibiri. Iyi miti yose uko ari itatu ikora nk'imiti ibuza JAK . Ni ukuvuga ko igabanya Janus-associated kinase (JAK) signaling ikora cyane. Kubera iyo mpamvu, iyi miti ifasha kugabanya ibimenyetso bijyana na myelofibrosis, nko kongera urwagashya, kubira ibyuya nijoro, kuribwa, kugabanya ibiro, n'umuriro.

Ku bantu benshi, intego y'ingenzi yo kuvura ni ukuvura indwara zifitanye isano na myelofibrosis, nko kubura amaraso no kwaguka k'umwobo.

Uburyo bwo kuvura indwara yo kubura amaraso

Hariho uburyo bwo kuvura indwara yo kubura amaraso nko:

• Androgens: Gufata androgens nka danazol bishobora kongera umusaruro w'uturemangingo dutukura mu maraso.
• Udukingirizo tw’umubiri: Iyi miti yongera ubushobozi bw’ubudahangarwa bw’umubiri wawe bwo kurwanya uturemangingo twa kanseri, bityo ikagabanya ibimenyetso. Imiti nka interferon, thalidomide (Thalomid®), na lenalidomide (Revlimid®) iri muri uru rwego. Ishobora kandi gutangwa hamwe na glucocorticoids nka prednisone.
• Imiti ivura indwara ya chimiotherapie: Imwe mu miti ivura indwara ya chimiotherapie ishobora kugabanya ibimenyetso biterwa no kwiyongera kw'uturemangingo tw'amaraso no kwiyongera k'umwobo. Hydroxyurea na cladribine ni imiti nk'iyo.
• Guterwa amaraso: Iyo ufite ikibazo gikomeye cyo kubura amaraso, guterwa amaraso buri gihe bishobora kongera umubare w'uturemangingo dutukura tw'amaraso yawe.

Uburyo bwo kuvura splenomegaly

Imiti igabanya ubukana bwa JAK, imiti igabanya ubukana bw'umubiri, n'imiti ya chimiotherapie ikoreshwa mu kuvura ubwandu bw'agashishwa. Mu bihe bikomeye, ushobora gukenera kubagwa agashishwa kawe (splenectomy) cyangwa se ugahabwa imiti ivura ubwandu hakoreshejwe imirasire.

Ubuvuzi bushingiye ku mirasire bushobora kandi kuba ngombwa mu kuvura indwara yitwa hematopoiesis yo hanze y'umuyoboro w'amagufwa, aho uturemangingo tw'amaraso dukura hanze y'umusokoro w'amagufwa.

Ese myelofibrosis ishobora gukira burundu?

Guteranya uturemangingo tw’amaraso two mu bwoko bwa Allogeneic hematopoietic selplantation (HCT) bishobora kuba ari bwo buryo bwonyine bwo gukiza iyi ndwara. Ariko, ubu buryo ni uburyo buteje akaga kandi ntibukwiriye buri wese.

Muri ubu buryo, uturemangingo tw’amaraso tudasanzwe dusimburwa n’uturemangingo tw’umuntu muzima utanga amaraso. Mbere yo guterwa uturemangingo, uzahabwa imiti igabanya ubukana cyangwa imiti ivura imirasire kugira ngo wice uturemangingo twarwaye. Hanyuma, uturemangingo dushya tw’utanga amaraso dushobora gufata imirimo y’umubiri wawe.

Ubuvuzi bwa HCT bufite ibyago byinshi byo kugira ibibazo . Kubwibyo, bukwiriye itsinda ry’abantu runaka gusa. Ingaruka zo kugira ibibazo ziraruta cyane ku bantu bafite izindi ndwara. Kuba ubikwiriye biterwa n'ibintu byinshi, nko imyaka yawe, uburemere bw'ibimenyetso byawe, ndetse n'ubushobozi bwo kugira icyo ugeraho mu buvuzi.

Ese igihe cyo kubaho kimeze gite iyo umuntu arwaye iyi ndwara?

Myelofibrosis ni kanseri ikomeye cyane . Igipimo cy’aba barwayi cyo kubaho ubusanzwe ni imyaka itandatu. Igipimo cy’abazima bivuze ko bamwe babaho imyaka itarenze itandatu, mu gihe umubare nk’uwo ubaho imyaka irenga itandatu. Ibi si bimwe kuri buri wese, biratandukanye bitewe n’umuntu ku giti cye.

Hari ibintu byinshi bigena igihe indwara yawe izakwirakwira:

• imyaka yawe.
• Ibimenyetso byawe.
• Umubare w'uturemangingo tw'amaraso yawe.
• Ubukana bw'inkovu mu musokoro w'amagufwa.
• Niba hari impinduka mu miterere y’uturemangingo (JAK2, CALR, MPL) cyangwa ntazo.

Igikuru ni ukuganira na muganga wawe ku buryo bworoshye ku bijyanye n'uko indwara yawe izagutwara ubuzima bwawe.

Niyitaho nte? (Kwiyitaho)

Baza muganga wawe niba ushobora kungukirwa n'ubuvuzi bwo kugabanya ububabare . Amatsinda y'ubuvuzi bwo kugabanya ububabare ashobora kuba arimo abaganga, abaforomo, abakozi b'imibereho myiza, n'abandi bahanga. Bashobora kuguha ibikoresho n'ubufasha ukeneye kugira ngo ubane n'iyi ndwara. Uretse ubuvuzi uhabwa n'umuganga wawe wa kanseri, aba bantu bashobora kuba isoko ikomeye y'ubufasha. Abahanga mu by'ubuvuzi bwo kugabanya ububabare bashobora kugufasha kunoza ubuzima bwawe nk'umuntu ubana na kanseri.

Amaherezo, ibintu by'ingenzi ugomba kwibuka

Myelofibrosis ni ubwoko budasanzwe bwa kanseri y'amaraso aho umwotsi wo mu igufwa wawe usimburwa n'uturemangingo tw'imitsi. Iyi ni indwara ikomeye isaba gukurikiranwa n'abaganga buri gihe cyangwa kuvurwa. Bitewe n'uko indwara yawe imeze, ushobora kutagira ibimenyetso mu gihe cy'imyaka myinshi. Mu bindi bihe, ibimenyetso bishobora kwiyongera vuba bigatuma bigorana gukora imirimo ya buri munsi. Ariko, hari uburyo bwo kuvura bufasha mu kugenzura ibimenyetso bibangamira ubuzima bwawe bwa buri munsi.

Hagati aho, baza muganga wawe ku byerekeye uburyo bwo kugufasha guhangana n'izi mpinduka. Ubuvuzi bw'indwara zibabaza umubiri n'amatsinda y'ubufasha ni amahitamo meza cyane mu gihe umenyera ubuzima bwa kanseri.

Wibuke ko utari wenyine. Abaganga, umuryango n'inshuti bose barahari kugira ngo bagufashe. Ntutinye kuvugana na muganga wawe ku bibazo byose ushobora kuba ufite.

Myelofibrosis , kanseri y'amaraso, umwongo w'amagufwa, kubura amaraso, kwaguka k'umwobo w'imyakura, imiti igabanya ubukana bwa JAK, kanseri y'amaraso