Iyini iMyelofibrosis Eyinhloko?

-Delile

• Izimpawu eziyinhloko ze-myelofibrosis
• Izigaba eziyinhloko ze-myelofibrosis
• Yini ebangela i-myelofibrosis eyinhloko?
• Izici zobungozi ze-myelofibrosis eyinhloko
• Izinketho zokuqala zokwelashwa kwe-myelofibrosis
• Imithi yokwelapha izimpawu
• Ama-JAK inhibitors
• Ukufakelwa kwesitokisi
• I-Chemotherapy kanye nemisebe
• Ukumpontshelwa igazi
• Ukuhlinzwa
• Ukuhlolwa kwamanje kwemitholampilo
• Izinguquko zendlela yokuphila
• Umbono
• Ukudla okudlela endlini

I-myelofibrosis eyinhloko (i-MF) umdlavuza ongajwayelekile odala izicubu ezibomvu, ezaziwa nge-fibrosis, emnkantsheni wethambo. Lokhu kuvimbela umnkantsha wakho ekukhiqizeni inani elijwayelekile lamaseli egazi.

I-MF eyinhloko uhlobo lomdlavuza wegazi. Ingenye yezinhlobo ezintathu zama-myeloproliferative neoplasms (MPN) ezenzeka lapho amaseli ehlukana kaningi noma engafi kaningi ngendlela efanele. Amanye ama-MPN afaka i-polycythemia vera ne-thrombocythemia ebalulekile.

Odokotela babheka izinto eziningana zokuxilonga iMF eyinhloko. Ungathola ukuhlolwa kwegazi kanye nomnkantsha wamathambo ukuthola i-MF.

Izimpawu eziyinhloko ze-myelofibrosis

Ungahle ungazitholi izimpawu iminyaka eminingi. Izimpawu zivame ukuqala ukuvela kancane kancane ngemuva kokulimala emnkantsheni wethambo bese ziqala ukuphazamisa ukukhiqizwa kwamaseli egazi.

Izimpawu eziyinhloko ze-myelofibrosis zingabandakanya:

• ukukhathala
• ukuphelelwa umoya
• isikhumba esiphaphathekile
• imfiva
• ukutheleleka okuvamile
• ukulinyazwa okulula
• ukujuluka ebusuku
• ukulahlekelwa isifiso sokudla
• ukuncipha kwesisindo okungaqondakali
• izinsini ezophayo
• ukuphuma kwamakhala kaningi
• ukugcwala noma ubuhlungu esiswini ohlangothini lwesobunxele (kubangelwa ubende obukhulisiwe)
• izinkinga ngokusebenza kwesibindi
• ukulunywa
• ubuhlungu obuhlangene noma bethambo
• ukuvuvukala

Abantu abane-MF ngokuvamile banezinga eliphansi kakhulu lamaseli abomvu egazi. Kungenzeka futhi babe nesibalo samaseli amhlophe egazi esiphakeme kakhulu noma esiphansi kakhulu. Udokotela wakho angathola kuphela lokhu okungahambi kahle ngesikhathi sokuhlolwa okujwayelekile kulandela ukubalwa kwegazi okujwayelekile.

Izigaba eziyinhloko ze-myelofibrosis

Ngokungafani nezinye izinhlobo zomdlavuza, i-MF eyinhloko ayinazo izigaba ezichazwe ngokucacile. Udokotela wakho esikhundleni salokho angasebenzisa iDynamic International Prognostic Scoring System (DIPSS) ukukuhlukanisa ngeqembu eliphansi, eliphakathi, noma elinobungozi obukhulu.

Bazobheka ukuthi ngabe wena:

• babe nezinga le-hemoglobin elingaphansi kwama-gramu ayi-10 kudesilitha ngayinye
• unesibalo samaseli amhlophe egazi esingaphezu kuka-25 × 10⁹ ilitha ngalinye
• baneminyaka engaphezu kwengama-65 ubudala
• babe namaseli wokuqhuma ajikeleza alingana noma angaphansi kwephesenti elilodwa
• ukuzwa izimpawu ezifana nokukhathala, ukujuluka ebusuku, umkhuhlane, nokuncipha

Ubhekwa njengengozi ephansi uma kungekho kulokhu okungenhla okusebenza kuwe. Ukuhlangabezana neyodwa noma ezimbili zalezi zindlela kukubeka eqenjini eliphakathi kwengozi. Ukuhlangabezana nalezi zindlela ezintathu noma ngaphezulu kukubeka eqenjini elisengozini enkulu.

Yini ebangela i-myelofibrosis eyinhloko?

Abaphenyi abaqondi kahle ukuthi yini ebangela i-MF. Imvamisa akulutholwa ngofuzo. Lokho kusho ukuthi awukwazi ukuthola lesi sifo kubazali bakho futhi awukwazi ukusidlulisela ezinganeni zakho, yize i-MF ijwayele ukugijima emindenini. Olunye ucwaningo luphakamisa ukuthi kungabangelwa ukushintshwa kwezakhi zofuzo okuthinta izindlela zokubonisa amaseli.

yabantu abane-MF banokuguqulwa kwezakhi zofuzo okwaziwa njenge-janus-associated kinase 2 (JAK2) ethinta amangqamuzana egazi. I- JAK2 ukuguquka komzimba kudala inkinga yokuthi umnkantsha ukhiqiza kanjani amaseli abomvu egazi.

Amaseli we-stem egazi angajwayelekile emnkantsheni wethambo akha amangqamuzana egazi avuthiwe aziphindaphinda ngokushesha futhi athathe umongo wethambo. Ukwakheka kwamaseli egazi kubanga isibazi nokuvuvukala okuthinta ikhono lomnkantsha wethambo lokudala amaseli egazi ajwayelekile. Lokhu kuvame ukuphumela kumaseli abomvu egazi angaphansi kwavamile namaseli amhlophe amaningi kakhulu.

Abaphenyi baxhumanise iMF nezinye izakhi zofuzo. Cishe amaphesenti ama-5 kuye kwayishumi abantu abane-MF bane- I-MPL ukuguquka kwezakhi zofuzo. Cishe amaphesenti angama-23.5 anokuguqulwa kwezakhi zofuzo okubizwa ngokuthi i-calreticulin (CALR).

Izici zobungozi ze-myelofibrosis eyinhloko

I-MF eyinhloko ayivamile kakhulu. Kwenzeka cishe ku-1.5 kuphela kubantu abayi-100,000 e-United States. Lesi sifo singathinta abesilisa nabesifazane.

Izici ezimbalwa zingakhuphula ubungozi bomuntu bokuthola i-MF eyinhloko, kufaka phakathi:

• engaphezu kweminyaka engama-60 ubudala
• ukuvezwa kwemishanguzo efana ne-benzene ne-toluene
• ukuvezwa kwemisebe ye-ionizing
• ukuba ne- JAK2 ukuguquka kwezakhi zofuzo

Izinketho zokuqala zokwelashwa kwe-myelofibrosis

Uma ungenazo izimpawu ze-MF, udokotela wakho kungenzeka angakufaki kunoma yikuphi ukwelashwa kunalokho akubheke ngokucophelela ngokuhlolwa okujwayelekile. Lapho nje izimpawu seziqalile, ukwelashwa kuhlose ukuphatha izimpawu nokuthuthukisa ikhwalithi yempilo yakho.

Izinketho zokwelapha eziyinhloko ze-myelofibrosis zifaka phakathi imithi, i-chemotherapy, i-radiation, ukufakelwa kwamaseli we-stem, ukumpontshelwa igazi nokuhlinzwa.

Imithi yokwelapha izimpawu

Imithi eminingana ingasiza ekwelapheni izimpawu ezinjengokukhathala nokuqina kwegazi.

Udokotela wakho angancoma i-aspirin yedosi ephansi noma i-hydroxyurea ukunciphisa ingozi ye-venous thrombosis (DVT) ejulile.

Imithi yokwelapha isibalo segazi elibomvu eliphansi (i-anemia) exhunywe ne-MF ifaka:

• ukwelashwa kwe-androgen
• ama-steroids, njenge-prednisone
• thalidomide (Thalomid)
• lenalidomide (Revlimid)
• ama-erythropoiesis stimulating agents (ama-ESA)

Ama-JAK inhibitors

Ama-JAK inhibitors alapha izimpawu ze-MF ngokuvimba umsebenzi we JAK2 isakhi sofuzo kanye ne-protein ye-JAK1. I-Ruxolitinib (Jakafi) ne-fedratinib (i-Inrebic) yimithi emibili evunyelwe yi-Food and Drug Administration (i-FDA) yokwelapha ubungozi obuphakathi noma ubungozi obukhulu be-MF. Amanye ama-inhibitors amaningi e-JAK njengamanje ahlolwa ezivivinyweni zokwelashwa.

I-Ruxolitinib ikhonjisiwe ukuthi inciphisa ukukhuliswa kobende futhi yehlise izimpawu eziningana ezihlobene ne-MF, njengokungaphatheki kahle esiswini, ubuhlungu bamathambo, nokunwaya. Iphinde inciphise amazinga ama-cytokines ahlasela okuvuvukala egazini. Lokhu kungasiza ekunciphiseni izimpawu ze-MF kufaka phakathi ukukhathala, umkhuhlane, ukujuluka ebusuku, nokuncipha.

I-Fedratinib ivame ukunikezwa lapho i-ruxolitinib ingasebenzi. I-inhibitor ekhethiwe kakhulu ye-JAK2. Kunengozi encane yokulimala kobuchopho okungathi sína futhi okungaba yingozi okwaziwa njenge-encephalopathy.

Ukufakelwa kwesitokisi

I-allogeneic stem cell transplant (ASCT) iyona kuphela ikhambi langempela le-MF. Eyaziwa nangokuthi ukufakelwa umnkantsha wethambo, kubandakanya ukufaka ukumnika kwamaseli ama-stem kumnikeli onempilo. Lawa maseli we-stem anempilo angena esikhundleni samangqamuzana angasebenzi kahle.

Inqubo inengozi enkulu yemiphumela emibi esongela impilo. Uzohlolwa ngokucophelela ngaphambi kokufaniswa nomnikeli. I-ASCT ivame ukucatshangelwa kuphela kubantu abanobungozi obuphakathi noma abanobungozi obukhulu abangaphansi kweminyaka engama-70.

I-Chemotherapy kanye nemisebe

Izidakamizwa ze-Chemotherapy kufaka phakathi i-hydroxyurea zingasiza ekunciphiseni ubende obukhulisiwe obuxhumene neMF. Ukwelashwa ngemisebe kwesinye isikhathi kuyasetshenziswa lapho ama-JAK inhibitors kanye ne-chemotherapy enganele ukunciphisa usayizi wobende.

Ukumpontshelwa igazi

Ukumpontshelwa igazi kwamangqamuzana abomvu egazi angasetshenziswa ukukhulisa isibalo samaseli abomvu nokwelapha i-anemia.

Ukuhlinzwa

Uma i-spleen ekhulisiwe ibangela izimpawu ezinzima, udokotela wakho ngezinye izikhathi angancoma ukususwa kokuhlinzwa kwepeni. Le nqubo yaziwa njenge-splenectomy.

Ukuhlolwa kwamanje kwemitholampilo

Izidakamizwa eziningi okwamanje zisaphenywa zokwelapha i-myelofibrosis eyinhloko. Lokhu kufaka phakathi ezinye izidakamizwa eziningi ezivimbela i-JAK2.

I-MPN Research Foundation igcina uhlu lwezivivinyo zokwelashwa ze-MF. Ezinye zalezi zivivinyo seziqalile ukuhlolwa. Abanye njengamanje baqasha iziguli. Isinqumo sokujoyina isivivinyo somtholampilo kufanele senziwe ngokucophelela nodokotela wakho nomndeni wakho.

Izidakamizwa zidlula ezigabeni ezine zokuhlolwa kwemitholampilo ngaphambi kokuthola imvume yi-FDA. Yimithi embalwa kuphela emisha njengamanje esesigabeni sesi-III sezilingo zomtholampilo, kufaka phakathi i-pacritinib ne-momelotinib.

Izivivinyo zomtholampilo weSigaba I no-II ziphakamisa ukuthi i-everolimus (RAD001) ingasiza ekwehliseni izimpawu nosayizi wobende kubantu abane-MF. Lesi sidakamizwa sivimbela indlela kumaseli akhiqiza igazi angaholela ekukhuleni kweseli okungavamile ku-MF.

Izinguquko zendlela yokuphila

Ungazizwa ucindezelekile ngokomoya ngemuva kokuthola ukuxilongwa okuyinhloko kwe-MF, noma ngabe awunazo izimpawu. Kubalulekile ukucela ukwesekwa kumndeni nabangane.

Ukuhlangana nomhlengikazi noma usonhlalakahle kungakunika ulwazi oluningi mayelana nokuthi ukuxilongwa komdlavuza kungayithinta kanjani impilo yakho. Ungase futhi ufune kudokotela wakho mayelana nokusebenza nochwepheshe wezempilo yengqondo onamalayisense.

Olunye ushintsho lwendlela yokuphila lungakusiza ukuphatha ukucindezeleka. Ukuzindla, i-yoga, ukuhamba kwemvelo, noma ngisho nokulalela umculo kungasiza ekuthuthukiseni imizwa yakho nenhlalakahle jikelele.

Umbono

I-MF eyinhloko kungenzeka ingazibangeli izimpawu ezinyathelweni zayo zokuqala futhi ingalawulwa ngezindlela ezahlukahlukene zokwelapha. Ukubikezela umbono nokusinda kwe-MF kungaba nzima. Lesi sifo asiqhubeki isikhathi eside kwabanye abantu.

Izilinganiso zokusinda zihluka kuye ngokuthi umuntu useqenjini eliphansi, eliphakathi, noma elisengozini enkulu. Olunye ucwaningo lubonisa ukuthi labo abaseqenjini elinobungozi obuncane banamazinga afanayo okusinda eminyakeni emihlanu yokuqala ngemuva kokuxilongwa njengabantu abaningi, lapho amazinga okusinda eqala ukwehla. Abantu abaseqenjini elisengozini enkulu basinda kwaze kwaba yiminyaka engu-7.

I-MF ingaholela ezinkingeni ezinkulu ngokuhamba kwesikhathi. I-MF eyinhloko idlulela kumdlavuza wegazi obucayi futhi onzima ukwelashwa owaziwa ngokuthi yi-acute myeloid leukemia (AML) kumaphesenti angaba ngu-15 kuya kwangama-20 amacala.

Iningi lezindlela zokwelashwa kwe-MF eyinhloko ligxila ekuphatheni izinkinga ezixhunyaniswe ne-MF. Lokhu kufaka phakathi i-anemia, ubende obukhulisiwe, izinkinga zokunqanda igazi, ukuba namaseli egazi amhlophe amaningi noma ama-platelet, nokuba ne-platelet count ephansi. Ukwelashwa kuphatha nezimpawu ezinjengokukhathala, ukujuluka ebusuku, isikhumba esilumayo, umkhuhlane, ubuhlungu bamalunga, ne-gout.

Ukudla okudlela endlini

I-MF eyinhloko umdlavuza ongavamile othinta amangqamuzana akho egazi. Abantu abaningi ngeke babe nezimpawu ekuqaleni kuze kube yilapho umdlavuza usuthuthukile. Ukuphela kokwelapha okungabakhona kwe-MF eyinhloko ukufakelwa i-stem cell, kepha kunezinye izindlela zokwelapha ezahlukahlukene zokuphatha izimpawu nokwenza ngcono impilo yakho.