Ingabe umongo wakho wethambo ukhiqiza izicubu ezine-fibrous? Ake sifunde nge-myelofibrosis!

Wake wezwa ngesifo esibizwa ngokuthi i-myelofibrosis? Empeleni uhlobo olungavamile kakhulu lomdlavuza wegazi. Kalula nje, yilapho umongo wamathambo , okuyingxenye ethambile nethambile yamathambo akho, ushintshwa khona yizicubu zesibazi esine-fibrous . Futhi uhlobo lwe-leukemia yesikhathi eside, futhi luyilungu leqembu elibizwa ngokuthi izifo ze-myeloproliferative. Izifo ze-myeloproliferative zenzeka lapho kukhiqizwa amangqamuzana egazi amaningi kakhulu emnothweni wamathambo, lapho kwakhiwa khona amangqamuzana egazi.

Iyini ngempela i-myelofibrosis?

Ake sibheke lokhu ngokuningiliziwe. Umnkantsha wakho wethambo uqukethe amaseli okuqala akha igazi. Lawa maseli okuqala kamuva akhula abe amaseli abomvu egazi, amaseli amhlophe egazi, nama-platelet. Kodwa kumuntu one-myelofibrosis, kwenzeka uguquko ezintweni zofuzo, noma i-DNA, kwelinye lala maseli okuqala. Lokhu kubangela ukuthi iseli libe yiseli lomdlavuza elinesici. Leli seli elinesici libe selihlukana futhi lidlulisele uguquko kuwo wonke amaseli amasha eliwenzayo.

Ngokuhamba kwesikhathi, la maseli omdlavuza angajwayelekile aqoqana ngobuningi. Amanye ala maseli abangela ukuvuvukala emnkantsheni wamathambo. Lokhu kuvuvukala yikho okubangela ukwakheka kwezicubu zesibazi engikhulume ngazo ekuqaleni. Ngakho-ke, ngenxa yalesi zicubu zesibazi kanye namangqamuzana omdlavuza angaphezulu, umnkantshe ulahlekelwa ikhono lawo lokwenza amaseli egazi anempilo.

Yiziphi izinhlobo eziyinhloko ze-myelofibrosis?

Kunezinhlobo ezimbili eziyinhloko ze-myelofibrosis.

• I-myelofibrosis eyinhloko: Lolu uhlobo oluvela ngokuzenzekelayo, ngaphandle kokuxhumana nanoma yisiphi esinye isifo.
• I-myelofibrosis yesibili: Lokhu kubangelwa esinye isifo segazi. Isibonelo, kungabangelwa izifo ezifana ne-primary thrombocytosis noma i-polycythemia vera. Lolu hlobo lwesibili luhlanganisa phakathi kuka-10% no-20% weziguli ezixilongiwe.

Sivame kangakanani lesi sifo esibizwa ngokuthi i-Myelofibrosis?

I-Myelofibrosis empeleni iyisimo esingavamile kakhulu . Isibonelo, e-United States, kubikwa ukuthi lesi sifo sitholakala kubantu abangaba ngu-1.5 kwabangu-100,000 ngonyaka. Singathinta noma ubani, kungakhathaliseki ubudala. Akukho mehluko weminyaka, kodwa kungenzeka kakhulu ukuthi sitholakale kubantu abangaphezu kweminyaka engu-50. Uma i-myelofibrosis ikhula ezinganeni ezincane, ivame ukutholwa ingakafiki eminyakeni engu-3.

Yimiphi imiphumela engaba nayo i-myelofibrosis emzimbeni wakho?

Uma amangqamuzana egazi ekhiqizwa ngendlela engavamile ngale ndlela, kungavela izinkinga ezahlukahlukene. Ake sibheke ukuthi ziyini:

• I-anemia: Lesi yisimo lapho kungekho khona amangqamuzana abomvu egazi anele. Njengoba wazi, amangqamuzana abomvu egazi yiwo athwala umoya-mpilo emzimbeni wonke. Ngakho-ke uma kungekho amangqamuzana abomvu anele, izicubu zomzimba azitholi umoya-mpilo owanele. Lokhu kungabangela izimpawu ezifana nokukhathala, ubuthakathaka, kanye nokuphelelwa umoya .

• I-Thrombocytopenia: Lesi yisimo lapho ama-platelet ambalwa kakhulu. Ama-platelet yiwo asiza ukujiya kwegazi uma kukhona ukulimala. Ngakho-ke uma inani lama-platelet akho liphansi, umzimba wakho ungaba sengozini enkulu yokopha nokuklwebheka .

• I-splenomegaly: I-spleen yakho ilawula ukubalwa kwamangqamuzana egazi futhi isuse amangqamuzana abomvu egazi alimele emzimbeni wakho. Ngakho-ke uma wenza amangqamuzana egazi amaningi angajwayelekile, i-spleen yakho kufanele isebenze kanzima. Ingakhula. Uma i-spleen yakho ikhula, ungase uzizwe ugcwele noma ungakhululekile engxenyeni ephezulu kwesobunxele yesisu sakho .

• I-Extramedullary hematopoiesis: Lokhu ukuthuthuka kwamaseli akha igazi ngaphandle komnkantsha , kwezinye izingxenye zomzimba. Lawa maseli angakheka ezindaweni ezifana namaphaphu, umgudu wokugaya ukudla, umgogodla, ubuchopho, noma ama-lymph node. Lawa maseli angahlangana ndawonye ukuze akhe amathumba , angahlasela ezinye izitho noma aphazamise ukusebenza kwawo.

• Umfutho wegazi ophezulu we-Portal: Lokhu ukwanda komfutho wegazi emthanjeni othwala igazi lisuka ebhentshini lakho liye esibindini sakho. Lokhu kungenzeka kakhulu ukuthi kubangelwa ukulimala kwemithambo yegazi ngenxa ye-hematopoiesis engaphandle kwe-medullary okukhulunywe ngayo ekuqaleni.

Enye into ukuthi cishe kubantu abayi-12% abane-myelofibrosis eyinhloko, lesi simo singakhula sibe uhlobo olubi kakhulu lomdlavuza wegazi olubizwa ngokuthi i-acute myeloid leukemia .

Yini ebangela i-myelofibrosis?

Ososayensi abakakazi kahle ukuthi yini ebangela i-myelofibrosis. Kodwa bathole ukuthi ihlotshaniswa nezinguquko ku-DNA yezakhi zofuzo ezithile . Uhlobo lweprotheni olubizwa ngokuthi i-Janus-associated kinases (JAKs) luhilelekile. Lawa maprotheni e-JAK alawula ukukhiqizwa kwamangqamuzana egazi emnkantsheni wethambo, athumela izimpawu kumaseli ukuze ahlukane futhi akhule. Uma la maprotheni e-JAK eba namandla ngokweqile, kungakhiqizwa amangqamuzana egazi amaningi kakhulu noma ambalwa kakhulu.

Phakathi kuka-60% no-65% wabantu abane-myelofibrosis banoguquko ku-JAK2 gene . Abanye abangu-5% kuya ku-10%Kukhona uguquko ku-myeloproliferative leukemia (MPL) gene. Futhi, uguquko olubizwa ngokuthi iCalreticulin (CALR) lubonakala phakathi kuka-20% no-25% weziguli ezine-myelofibrosis.

Ubani osengozini enkulu yalokhu?

Ungase ube sengozini enkulu yokuthuthukisa i-myelofibrosis ngenxa yezizathu ezilandelayo:

• Uma uneminyaka engaphezu kwengu-50 ubudala .
• Uma unesifo segazi esibizwa ngokuthi i-primary thrombocytosis noma i-polycythemia vera.
• Uma uke wachayeka emisebeni ye-ionizing noma amakhemikhali e-petrochemical njenge-benzene ne-toluene.

Ziyini izimpawu ze-myelofibrosis?

I-Myelofibrosis yisifo esiqhubeka kancane kancane , ngakho-ke ungase ungabi nazimpawu iminyaka eminingi. Cishe ingxenye yesithathu yeziguli ayinazo izimpawu ezigabeni zokuqala.

Uma kuvela izimpawu, ezivame kakhulu ukukhathala okukhulu (okubangelwa yi-anemia) kanye nokwanda kwe-spleen . Izimpawu zingase zihlanganise:

• Umsebenzi onzima
• Imfiva
• Ukuluma
• Isikhumba esikhanyayo
• Ukwehlisa isisindo
• Ukujuluka ebusuku
• Ubuhlungu bamathambo noma amalunga
• Ukutheleleka okuvamile
• Ubende noma isibindi esikhulu
• Amahlule egazi angachazeki
• Ukopha noma ukuklwebheka okungavamile
• Ukwanda kwemithambo esiswini kanye nasemphinjeni (le mithambo ingaqhuma futhi yophe igazi).

Ungasithola kanjani lesi sifo?

Udokotela, ikakhulukazi udokotela wezifo zomdlavuza, uzokuhlola ngokomzimba, abuze ngomlando wakho wezokwelapha, futhi abuze ngezimpawu zakho zamanje. Bazohlola futhi ukuthi une-spleen ekhuphukile kanye nezimpawu ze-anemia.

Ngemuva kwalokho, kwenziwa izivivinyo ezahlukahlukene ukuze kutholakale ezinye izimo futhi kuqinisekiswe ukuthi une-myelofibrosis.

Ukuhlolwa kwegazi

• Ukubalwa kwegazi okuphelele (i-CBC): Lokhu kulinganisa inani lezinhlobo ezahlukene zamaseli egazini lakho. Uma amaseli akho abomvu ephansi kunokuvamile, noma amaseli akho amhlophe egazi nama-platelet engavamile, kungaba uphawu lwe-myelofibrosis.
• I-Peripheral blood smear (PBS): Lokhu kuhlola usayizi, ukuma, kanye nezinye izici zamaseli akho egazi ukuze kutholakale ukukhubazeka. Uma kukhona amaseli abukeka engajwayelekile kanye nenani elikhulu lamaseli egazi angakavuthwa, kungaba uphawu lwe-myelofibrosis.
• Ukuhlolwa kwamakhemikhali egazini:Lokhu kuhlolwa kulinganisa amazinga ezinto ezahlukahlukene ezikhishwa egazini ezivela ezithweni zakho zomzimba. Lokhu kungakutshela ukuthi isitho sisebenza kahle kangakanani. Amazinga aphezulu ezinto ezifana ne-uric acid, i-bilirubin, kanye ne-lactic dehydrogenase egazini nakho kungaba uphawu lwe-myelofibrosis.

Ukuhlolwa komnkantsha wamathambo

• Ukuhlolwa komnkantsha wethambo: Lokhu kuhilela ukuthatha isampula encane yomnkantsha wakho wethambo bese uwuhlola ngaphansi kwe-microscope. Amaseli ayahlolwa ukuze kutholakale ukuthi une-myelofibrosis yini.
• Ukufuthwa komnkantsha: Lokhu kuhilela ukukhipha uketshezi emnkantsha bese kuhlolwa izimpawu ze-myelofibrosis.

Kungase kudingeke ukuhlolwa okwengeziwe

Kungadingeka ukuhlolwa okwengeziwe ukuqinisekisa ukuxilongwa kwakho:

• Ukuhlaziywa kokuguquka kwezakhi zofuzo: Udokotela wakho uzohlola amangqamuzana akho egazi nawomnkantsha ukuze athole ukuguquka kwezakhi zofuzo okuhlobene ne-myelofibrosis, njenge-JAK2, i-CALR, kanye ne-MPL. Ezinye izindlela zokwelapha zihlose amangqamuzana omdlavuza anokuguqulwa kwe-JAK2.
• Izinqubo zokuthwebula izithombe: Udokotela wakho angenza i-ultrasound ukuhlola ukuthi ngabe kukhona yini udende olukhuphukile. Angenza ne-MRI ukuhlola izicubu ezibomvu emnkantsheni. Lezi zicubu ezibomvu zingaba uphawu lwe-myelofibrosis.

Iphathwa kanjani i-myelofibrosis?

Uma ungakhombisi zimpawu, ungase ungadingi ukwelashwa. Kodwa-ke, noma ungadingi ukwelashwa okusheshayo, udokotela wakho uzoqhubeka nokuqapha isimo sakho.

I-Jakafi® (ruxolitinib), i-Inrebic® (fedratinib), kanye ne-Vonjo® (pacritinib) imithi evunyelwe yi-U.S. Food and Drug Administration (FDA) . Isetshenziselwa ukwelapha i-myelofibrosis enobungozi obuphakathi kuya kobukhulu. Yonke le mithi emithathu isebenza njengezithibi ze-JAK . Okusho ukuthi, yehlisa i-Janus-associated kinase (JAK) signaling esebenza ngokweqile. Ngenxa yalokho, le mithi isiza ekunciphiseni izimpawu ezihlobene ne-myelofibrosis, njengobende olukhuphukile, ukujuluka ebusuku, ukulunywa, ukwehla kwesisindo, kanye nomkhuhlane.

Kwabaningi, umgomo oyinhloko wokwelashwa ukuphatha izimo ezihlobene ne-myelofibrosis, njenge-anemia kanye ne-spleen ekhulisiwe.

Ukwelashwa kwe-Anemia

Kunezindlela zokwelapha i-anemia ezifana nalezi:

• Ama-Androgen: Ukuthatha ama-androgens njenge-danazol kungandisa ukukhiqizwa kwamaseli abomvu egazi.
• Ama-immunomodulators: Le mithi yandisa ikhono lomzimba wakho lokulwa namaseli omdlavuza, ngaleyo ndlela inciphise izimpawu. Imithi efana ne-interferon, i-thalidomide (Thalomid®), kanye ne-lenalidomide (Revlimid®) ingeyalolu hlobo. Inganikezwa futhi ngokuhlanganiswa nama-glucocorticoids njenge-prednisone.
• Imithi yokwelapha ngamakhemikhali: Eminye imithi yokwelapha ngamakhemikhali inganciphisa izimpawu ezibangelwa ukwanda kwenani lamaseli egazi kanye nokwanda kwe-spleen. I-Hydroxyurea kanye ne-cladribine yimithi enjalo.
• Ukumpontshelwa igazi: Uma une-anemia enkulu, ukumpontshelwa igazi njalo kungandisa inani lamaseli akho abomvu egazi.

Ukwelashwa kwe-Splenomegaly

Ama-JAK inhibitors, ama-immunomodulators, kanye nemithi ye-chemotherapy kusetshenziselwa ukuphatha i-spleen ekhulisiwe. Ezimweni ezimbi kakhulu, kungadingeka ukuthi i-spleen yakho isuswe ngokuhlinzwa (i-spleenectomy) noma uthole ukwelashwa ngemisebe ku-spleen.

Ukwelashwa ngemisebe kungadingeka futhi ekwelapheni isimo esibizwa ngokuthi i-extramedullary hematopoiesis, lapho amangqamuzana egazi ekhula khona ngaphandle komnkantsha.

Ingabe i-myelofibrosis ingalapheka ngokuphelele?

Ukufakelwa kwamangqamuzana e-hematopoietic allogeneic (HCT) kungaba yindlela kuphela yokwelapha lesi sifo. Kodwa-ke, le inqubo eyingozi futhi ayifanele wonke umuntu.

Kule nqubo, amaseli akho egazi angajwayelekile athathelwa indawo ngamaseli avela kumnikeli onempilo. Ngaphambi kokufakelwa, uzonikezwa ukwelashwa ngamakhemikhali noma ngemisebe ukuze kubulawe amaseli akho agulayo. Ngemuva kwalokho, amaseli amasha avela kumnikeli angathatha imisebenzi yomzimba wakho.

Ukwelashwa kwe-HCT kunengozi enkulu yezinkinga . Ngakho-ke, kufanelekela kuphela iqembu elithile labantu. Ingozi yezinkinga iphakeme nakakhulu kubantu abanezinye izimo zezokwelapha. Ukuthi uyakufanelekela yini lokhu kuncike ezintweni eziningi, njengobudala bakho, ubukhali bezimpawu zakho, kanye nethuba lokuphumelela kokwelashwa.

Ingakanani isikhathi sokuphila uma uphila nalesi sifo?

I-Myelofibrosis ingumdlavuza onzima kakhulu . Izinga lokusinda eliphakathi lalaba ziguli livame ukuba yiminyaka eyisithupha. Izinga lokusinda eliphakathi lisho ukuthi abanye abantu baphila iminyaka engaphansi kweyisithupha, kanti inani elifanayo liphila iminyaka engaphezu kweyisithupha. Lokhu akufani kuwo wonke umuntu, kuyahlukahluka kuye ngomuntu nomuntu.

Kunezici eziningana ezinquma ukubikezela kwesifo sakho:

• iminyaka yakho.
• Izimpawu zakho.
• Inani lamaseli egazi lakho.
• Ubunzima bokuqhekeka kwamanxeba emnkantsheni.
• Kungakhathaliseki ukuthi kukhona yini izinguquko zofuzo (JAK2, CALR, MPL) noma cha.

Into ebaluleke kakhulu ukukhuluma ngokukhululekile nodokotela wakho mayelana nokuthi ukuxilongwa kwakho kuzothinta kanjani impilo yakho.

Ngizinakekela kanjani? (Ukuzinakekela)

Buza udokotela wakho ukuthi ungazuza yini ngokunakekelwa kwe-palliative . Amaqembu okunakekelwa kwe-palliative angafaka odokotela, abahlengikazi, izisebenzi zezenhlalakahle, kanye nabanye ochwepheshe. Bangakunikeza izinsiza kanye nokusekelwa okudingayo ukuze uphile nalesi sifo. Ngaphezu kokwelashwa okuthola kudokotela wakho we-oncologist, laba bantu bangaba umthombo omuhle wokusekela. Ochwepheshe bokunakekelwa kwe-palliative bangakusiza ukuthuthukisa ikhwalithi yempilo yakho njengomuntu ophila nomdlavuza.

Okokugcina, izinto ezibaluleke kakhulu okufanele uzikhumbule

I-Myelofibrosis uhlobo olungavamile lomdlavuza wegazi lapho umongo wakho wethambo ushintshwa khona yizicubu ezine-fibrous. Lesi yisimo esibi esidinga ukubhekwa okuqhubekayo kwezokwelapha kanye/noma ukwelashwa. Kuye ngesimo sakho, ungase ungabi nazimpawu iminyaka eminingi. Kwezinye izimo, izimpawu zingathuthuka ngokushesha futhi zenze kube nzima ukwenza imisebenzi yansuku zonke. Kodwa-ke, kunezindlela zokwelapha ezitholakalayo zokusiza ukulawula izimpawu eziphazamisa impilo yakho yansuku zonke.

Okwamanje, buza udokotela wakho ngezinsiza ezingakusiza ukubhekana nalezi zinguquko. Ukunakekelwa kwe-palliative kanye namaqembu okusekela kuyizinketho eziwusizo kakhulu njengoba ujwayela impilo nomdlavuza.

Khumbula, awuwedwa. Odokotela, umndeni, nabangani bonke bakhona ukukusiza. Ungesabi ukukhuluma nodokotela wakho nganoma yimiphi imibuzo ongase ube nayo.

I- Myelofibrosis, umdlavuza wegazi, umongo wamathambo, i-anemia, ukukhulisa udende, izithibi ze-JAK, i-leukemia