Ukuqonda iMyelofibrosis

-Delile

• Ziyini izimpawu?
• Kubangelwa yini?
• Ingabe zikhona izinto eziyingozi?
• Kutholakala kanjani?
• Ilashwa kanjani?
• Ukwelapha i-anemia
• Ukwelapha ubende obukhulisiwe
• Ukwelapha izakhi zofuzo eziguquliwe
• Ukwelashwa kokuhlola
• Ingabe zikhona izinkinga?
• Ukuhlala ne-myelofibrosis

Yini i-myelofibrosis?

I-Myelofibrosis (MF) uhlobo lomdlavuza womnkantsha othinta amandla omzimba wakho wokukhiqiza amaseli egazi. Kuyingxenye yeqembu lezimo ezibizwa nge-myeloproliferative neoplasms (MPNs). Lezi zimo zidala ukuthi amangqamuzana omnkantsha wakho ayeke ukukhula nokusebenza ngendlela okufanele enze ngayo, okuholela kwizicubu ezibomvu.

I-MF ingaba yinhloko, okusho ukuthi iyenzeka yodwa, noma yesibili, okusho ukuthi ivela kwesinye isimo - imvamisa esithinta umnkantsha wakho. Amanye ama-MPN nawo angadlulela ku-MF. Ngenkathi abanye abantu bengahamba iminyaka bengenazo izimpawu, abanye banezimpawu eziba zimbi kakhulu ngenxa yokushwabana emnkantsheni wethambo labo.

Ziyini izimpawu?

I-Myelofibrosis ivame ukuza kancane, futhi abantu abaningi abaziboni izimpawu ekuqaleni. Kodwa-ke, njengoba iqhubeka futhi iqala ukuphazamisa ukukhiqizwa kwamangqamuzana egazi, izimpawu zayo zingafaka:

• ukukhathala
• ukuphelelwa umoya
• ukulimaza noma ukopha kalula
• ukuzwa ubuhlungu noma ukugcwala ohlangothini lwakho lwesobunxele, ngaphansi kwezimbambo zakho
• ukujuluka ebusuku
• imfiva
• ubuhlungu bamathambo
• ukulahlekelwa isifiso sokudla nokuncipha
• ukuphuma kwamakhala noma ukopha kwezinsini

Kubangelwa yini?

I-Myelofibrosis ihlotshaniswa nokuguqulwa kwezakhi zofuzo kumaseli wegazi. Kodwa-ke, abacwaningi abaqiniseki ukuthi yini ebangela lolu shintsho.

Lapho amangqamuzana aguqukile ephindaphindeka futhi ehlukana, adlulisela ukuguquka kwawo kumaseli egazi amasha. Ekugcineni, amaseli aguqukile adlula ikhono lomnkantsha wethambo lokukhiqiza amaseli egazi aphilile. Lokhu kuvame ukuholela ekutheni kube namangqamuzana abomvu egazi ambalwa kakhulu namaseli amhlophe amaningi kakhulu. Kubuye kubangele ukusikeka nokuqina kwethambo lakho lomnkantsha, okuvame ukuthamba futhi kube nesiponji.

Ingabe zikhona izinto eziyingozi?

I-Myelofibrosis ayivamile, yenzeka cishe kubantu abayi-1.5 kuphela kwabangu-100 000 e-United States. Noma kunjalo, izinto eziningana zingakhuphula ingozi yokuyithuthukisa, kufaka phakathi:

• Ubudala. Ngenkathi abantu banoma yibuphi ubudala bangaba ne-myelofibrosis, kuvame ukutholakala kulabo abaneminyaka engaphezu kwengu-50.
• Enye inkinga yegazi. Abanye abantu abane-MF bayithuthukisa njengenkinga yesinye isimo, njenge-thrombocythemia noma i-polycythemia vera.
• Ukuvezwa ngamakhemikhali. I-MF ihlotshaniswa nokuchayeka kumakhemikhali athile ezimboni, kufaka phakathi i-toluene ne-benzene.
• Ukuvezwa yimisebe. Abantu abaye bavezwa ezintweni ezinomsakazo bangaba nengozi eyengeziwe yokuthuthukisa i-MF.

Kutholakala kanjani?

I-MF ivame ukukhombisa ukubalwa kwegazi okuphelele (CBC). Abantu abane-MF bavame ukuba namazinga aphansi kakhulu amangqamuzana abomvu egazi namazinga aphezulu ngokungavamile noma aphansi amangqamuzana amhlophe egazi nama-platelets.

Ngokuya ngemiphumela yokuhlolwa kwakho kwe-CBC, udokotela wakho angenza futhi i-bone marrow biopsy. Lokhu kuhilela ukuthatha isampula elincane lomnkantsha wakho bese ulibhekisisa kakhulu ukuthola izimpawu ze-MF, ezinjengezibazi.

Ungadinga futhi i-X-ray noma i-MRI scan ukukhipha ezinye izimbangela zezimpawu zakho noma imiphumela ye-CBC.

Ilashwa kanjani?

Ukwelashwa kwe-MF kuvame ukuncika ezinhlotsheni zezimpawu onazo. Izimpawu eziningi ezivamile ze-MF zihlotshaniswa nesimo esidalwa yi-MF, njenge-anemia noma ubende obukhulisiwe.

Ukwelapha i-anemia

Uma i-MF ibangela i-anemia enamandla, ungahle udinge:

• Ukumpontshelwa igazi. Ukumpontshelwa igazi njalo kungakhuphula inani elibomvu lamaseli egazi lakho futhi kwehlise izimpawu ze-anemia, njengokukhathala nobuthakathaka.
• Ukwelashwa kweHormone. Uhlobo lokwenziwa kwe-hormone yesilisa i-androgen ingakhuthaza ukukhiqizwa kwamangqamuzana egazi abomvu kwabanye abantu.
• Ama-Corticosteroids. Lokhu kungasetshenziswa nge-androgens ukukhuthaza ukukhiqizwa kwamangqamuzana egazi abomvu noma ukunciphisa ukubhujiswa kwawo.
• Imithi kadokotela. Imithi yokuzivikela emzimbeni, efana ne-thalidomide (Thalomid), ne-lenalidomide (Revlimid), ingahle ithuthukise ukubalwa kwamaseli egazi. Zingasiza futhi ngezimpawu zesigaxa esikhulisiwe.

Ukwelapha ubende obukhulisiwe

Uma unesipele esandisiwe esihlobene ne-MF esibangela izinkinga, udokotela wakho angancoma:

• Ukwelashwa ngemisebe. Ukwelashwa ngemisebe kusebenzisa imishayo ehlosiwe ukubulala amaseli nokunciphisa usayizi wobende.
• Ukwelashwa ngamakhemikhali. Eminye imithi yokwelashwa ngamakhemikhali inganciphisa usayizi wobende obukhulisiwe.
• Ukuhlinzwa. I-splenectomy inqubo yokuhlinzwa esusa ubende lwakho. Udokotela wakho angakutusa lokhu uma ungaphenduli kahle kwezinye izindlela zokwelashwa.

Ukwelapha izakhi zofuzo eziguquliwe

Umuthi omusha obizwa nge-ruxolitinib (Jakafi) wavunywa yi-U.S. Food and Drug Administration ngo-2011 ukwelapha izimpawu ezihambisana neMF. I-Ruxolitinib ihlose ukuguqulwa okuthile kwezakhi zofuzo okungaba yimbangela ye-MF. Ku, kuboniswe ukunciphisa ubukhulu bezimpondo ezikhulisiwe, ukunciphisa izimpawu ze-MF, nokwenza ngcono ukubikezela.

Ukwelashwa kokuhlola

Abaphenyi basebenzela ukwakha izindlela ezintsha zokwelapha ze-MF. Yize eziningi zalezi zidinga ukutadisha okuqhubekayo ukuze kuqinisekiswe ukuthi ziphephile, odokotela sebeqalile ukusebenzisa izindlela ezimbili zokwelapha ezimeni ezithile:

• Ukufakelwa kwe-stem cell. Ukufakelwa kwama-stem esitokisini kunamandla okwelapha i-MF nokubuyisela ukusebenza komnkantsha wamathambo. Kodwa-ke, inqubo ingadala izinkinga ezisongela impilo, ngakho-ke imvamisa yenziwa kuphela uma kungekho okunye okusebenzayo.
• I-Interferon-alpha. I-Interferon-alfa ibambezele ukwakheka kwezicubu ezibomvu emnkantsheni wamathambo wabantu abathola ukwelashwa kusenesikhathi, kepha kudingeka olunye ucwaningo ukunquma ukuphepha kwalo okuhlala isikhathi eside.

Ingabe zikhona izinkinga?

Ngokuhamba kwesikhathi, i-myelofibrosis ingaholela ezinkingeni eziningana, kufaka phakathi:

• Ukwenyuka komfutho wegazi esibindini sakho. Ukwanda kokugeleza kwegazi kusuka kubende obukhulisiwe kungakhuphula ingcindezi emthanjeni wesibindi esibindini sakho, kubangele isimo esibizwa nge-portal hypertension. Lokhu kungabeka ingcindezi enkulu emithanjeni emincane esiswini nasesimeni somzimba, okungaholela ekopheni ngokweqile noma emthanjeni oqhekekile.
• Izimila. Amaseli egazi angakha ngama-clumps ngaphandle komnkantsha wethambo, abangele ukuthi kukhule izicubu kwezinye izindawo zomzimba wakho. Ngokuya ngokuthi lezi zilonda zitholakala kuphi, zingadala izinkinga ezahlukahlukene ezahlukahlukene, kufaka phakathi ukuquleka, ukuphuma kwegazi esiswini, noma ukucindezelwa komgogodla.
• I-leukemia enamandla. Cishe amaphesenti ayi-15 kuye kwangama-20 abantu abane-MF baqhubeka nokuthuthukisa i-acute myeloid leukemia, uhlobo lomdlavuza olunzima nolunolaka.

Ukuhlala ne-myelofibrosis

Ngenkathi i-MF ivame ukungazibangeli izimpawu ezinyathelweni zayo zokuqala, ekugcineni ingaholela ezinkingeni ezinkulu, kufaka phakathi izinhlobo ezinomdlavuza ezingaphezulu. Sebenza nodokotela wakho ukunquma indlela engcono yokwelashwa kuwe nokuthi ungaziphatha kanjani izimpawu zakho. Ukuhlala ne-MF kungakhathaza, ngakho-ke ungakuthola kuwusizo ukufuna ukwesekwa enhlanganweni efana neLeukemia neLymphoma Society noma iMyeloproliferative Neoplasm Research Foundation. Zombili lezi zinhlangano zingakusiza ekutholeni amaqembu osizo endawo, imiphakathi eku-inthanethi, kanye nezinsizakusebenza zezezimali zokwelashwa.