Ukuqonda iMyelofibrosis

Umxholo

• Ziintoni iimpawu?
• Ibangelwa yintoni?
• Ngaba zikhona izinto ezinobungozi?
• Kufunyaniswa njani?
• Inyangwa njani?
• Ukunyanga i-anemia
• Ukunyanga udakada owandisiweyo
• Ukunyanga iigene eziguqukileyo
• Unyango lovavanyo
• Ngaba zikhona iingxaki?
• Ukuhlala ne-myelofibrosis

Yintoni i-myelofibrosis?

I-Myelofibrosis (MF) luhlobo lomhlaza womongo wethambo ochaphazela ukukwazi komzimba wakho ukuvelisa iiseli zegazi. Inxalenye yeqela leemeko ezibizwa ngokuba zii-myeloproliferative neoplasms (MPNs). Ezi meko zibangela ukuba iiseli zomongo wethambo ziyeke ukukhula kunye nokusebenza ngendlela ebekufanele ukuba ziphume ngayo, zikhokelela kwizicwili ezibomvu.

I-MF ingaba yiprayimari, oku kuthetha ukuba iyenzeka yodwa, okanye yesibini, oko kuthetha ukuba kubangelwa yenye imeko-ihlala ichaphazela umongo wethambo. Ezinye ii-MPN zinokuqhubela phambili ziye kwi-MF. Ngelixa abanye abantu banokuhamba iminyaka bengenazo iimpawu, abanye baneempawu eziba mandundu ngenxa yokunqaba kumongo wabo wethambo.

Ziintoni iimpawu?

I-Myelofibrosis ithande ukuza kancinci, kwaye abantu abaninzi abaziqapheli iimpawu ekuqaleni. Nangona kunjalo, njengoko iqhubeka kwaye iqala ukuphazamisa ukuveliswa kweeseli zegazi, iimpawu zayo zinokubandakanya:

• ukudinwa
• ukuqhawukelwa ngumphefumlo
• ukugruzuka okanye ukopha ngokulula
• ukuziva iintlungu okanye ukugcwala kwicala lasekhohlo, ngaphantsi kweembambo zakho
• ukubila ebusuku
• ifiva
• iintlungu zethambo
• ukuphelelwa ngumdla wokutya kunye nokwehla kobunzima
• ukuphuma kweempumlo okanye iintsini ezophayo

Ibangelwa yintoni?

I-Myelofibrosis inxulumene nokutshintsha kwemfuza kwiiseli zegazi. Nangona kunjalo, abaphandi abaqinisekanga ukuba yintoni ebangela olu tshintsho.

Xa iiseli eziguqulweyo ziphindaphindeka zize zahlule, zidlulisela utshintsho kwiiseli ezintsha zegazi. Ekugqibeleni, iiseli eziguqulweyo zigqitha kumnkantsha wethambo wokuvelisa iiseli eziphilileyo zegazi. Oku kuhlala kukhokelela kwiiseli ezibomvu ezimbalwa kakhulu kunye neeseli ezimhlophe zegazi. Ikwabangela ukonakala kunye nokuqaqanjelwa komongo wethambo lakho, elihlala lithambile kwaye lifana nesiponji.

Ngaba zikhona izinto ezinobungozi?

I-Myelofibrosis inqabile, ivela kuphela malunga ne-1.5 kubo bonke abantu abayi-100,000 e-United States. Nangona kunjalo, izinto ezininzi zinokunyusa umngcipheko wokuziphuhlisa, kubandakanya:

• Ubudala. Ngelixa abantu bayo nayiphi na iminyaka banokuba ne-myelofibrosis, ihlala ifunyaniswa kwabo baneminyaka engaphezulu kwama-50.
• Esinye isifo segazi. Abanye abantu abane-MF bayiphuhlisa njengengxaki yenye imeko, enjenge-thrombocythemia okanye i-polycythemia vera.
• Ukubonakaliswa kwimichiza. I-MF inxulunyaniswa nokuvezwa kweekhemikhali ezithile zorhwebo, kubandakanya i-toluene kunye ne-benzene.
• Ukubonakaliswa kwimitha. Abantu abaye bavezwa kwizixhobo ezinemitha ye-radio banokuba nomngcipheko okhulayo we-MF.

Kufunyaniswa njani?

I-MF ihlala ibonisa ubalo lwegazi olupheleleyo (CBC). Abantu abane-MF bathambekele ekubeni namanqanaba asezantsi kakhulu eeseli ezibomvu kunye namazinga aphezulu ngokungaqhelekanga okanye asezantsi eeseli ezimhlophe zegazi kunye neeplatelets.

Ngokusekwe kwiziphumo zovavanyo lwakho lwe-CBC, ugqirha wakho unokwenza kwakhona umongo wethambo. Oku kubandakanya ukuthatha isampulu encinci yomongo wethambo lakho kwaye ujonge ngakumbi ngakumbi kwiimpawu ze-MF, ezinje ngokuqhekeka.

Unokufuna kwakhona i-X-ray okanye iMRI ukuskena ukulawula naziphi na ezinye izizathu ezinokubangela iimpawu zakho okanye iziphumo zeCBC.

Inyangwa njani?

Unyango lwe-MF luhlala luxhomekeke kwiindidi zeempawu onazo. Uninzi lweempawu eziqhelekileyo ze-MF zinxulunyaniswa nemeko ebangelwa yi-MF, enje nge-anemia okanye i-spleen eyandisiweyo.

Ukunyanga i-anemia

Ukuba i-MF ibangela i-anemia enkulu, unokufuna:

• Utofelo-gazi. Ukufakwa kwegazi rhoqo kunokunyusa ukubala kweeseli ezibomvu kunye nokunciphisa iimpawu ze-anemia, ezinje ngokudinwa kunye nokuba buthathaka.
• Unyango lweHormone. Inguqulelo yokwenziwa kwehomoni eyindoda kunye ne-androgen inokukhuthaza ukuveliswa kweeseli ebomvu kwigazi kwabanye abantu.
• ICorticosteroids. Ezi zinokuthi zisetyenziswe kunye ne-androgens ukukhuthaza ukuveliswa kweeseli ezibomvu zegazi okanye ukunciphisa ukutshatyalaliswa kwabo.
• Amayeza amiselweyo. Unyango lwe-Immunomodulatory, njenge-thalidomide (Thalomid), kunye ne-lenalidomide (Revlimid), inokuphucula ukubalwa kweeseli zegazi. Banokukunceda kunye neempawu zepeni eyandisiweyo.

Ukunyanga udakada owandisiweyo

Ukuba unesipeliti esandisiweyo esinxulumene neMF esibangela iingxaki, ugqirha unokucebisa:

• Unyango ngemitha. Unyango lwe-radiation lusebenzisa imiqadi ekujoliswe kuyo ukubulala iiseli kunye nokunciphisa ubungakanani bepeni.
• Unyango ngamayeza. Ezinye iziyobisi ze-chemotherapy zinokunciphisa ubungakanani bepeni yakho eyandisiweyo.
• Ugqirha. I-splenectomy yinkqubo yotyando esusa udakada. Ugqirha wakho unokucebisa oku ukuba awuphenduli kakuhle kwezinye iindlela zonyango.

Ukunyanga iigene eziguqukileyo

Iyeza elitsha elibizwa ngokuba yi-ruxolitinib (Jakafi) lavunywa yi-U.S. YoLawulo lwezoKutya kunye noLawulo lweziyobisi ngo-2011 ukunyanga iimpawu ezinxulumene ne-MF. I-Ruxolitinib ijolise kutshintsho oluthile olunokuba sisizathu se-MF. Ku, kwaboniswa ukunciphisa ubungakanani bezipeni ezandisiweyo, ukunciphisa iimpawu ze-MF, kunye nokuphucula ukuxela kwangaphambili.

Unyango lovavanyo

Abaphandi basebenzela ekuphuhliseni unyango olutsha lwe-MF. Ngelixa uninzi lwezi zidinga ukuqhubekeka nokufunda ukuze uqiniseke ukuba zikhuselekile, oogqirha baqalisile ukusebenzisa iindlela ezimbini zonyango kwezinye iimeko:

• Ukufakelwa kweseli. Ukufakelwa kweeseli zesitina kunakho ukunyanga i-MF kunye nokubuyisela ukusebenza komongo wethambo. Nangona kunjalo, le nkqubo inokubangela iingxaki ezisongela ubomi, ngoko ke yenziwa kuphela xa kungekho nto iyenye isebenza.
• I-interferon-alpha. I-Interferon-alfa ikulibazisile ukwenziwa kwethishu ebomvu kumongo wethambo wabantu abafumana unyango kwangoko, kodwa uphando olongezelelekileyo luyafuneka ukumisela ukhuseleko lwexesha elide.

Ngaba zikhona iingxaki?

Ixesha elingaphezulu, i-myelofibrosis inokukhokelela kwiingxaki ezininzi, kubandakanya:

• Ukunyusa uxinzelelo lwegazi kwisibindi sakho. Ukuhamba kwegazi okwandileyo ukusuka kwipeni eyandisiweyo kunokuphakamisa uxinzelelo kwi-portal vein kwisibindi sakho, kubangele imeko ebizwa ngokuba yi-portal hypertension. Oku kunokubeka uxinzelelo oluninzi kwiimvumba ezincinci esiswini sakho kunye nesisu, oko kunokukhokelela ekuphumeni kakhulu kwegazi okanye umthambo ophukileyo.
• Amathumba. Iiseli zegazi zingenza ii-clumps ezingaphandle komongo wethambo, zibangele ukukhula kwezidumbu kwezinye iindawo zomzimba wakho. Kuxhomekeke apho zikhona ezi tumors, zinokubangela iingxaki ezahlukeneyo, kubandakanya ukubanjwa, ukopha kwithumbu lesisu, okanye uxinzelelo lomqolo.
• Umhlaza wegazi. Phantse iipesenti ezili-15 ukuya kuma-20 zabantu abane-MF bayaqhubeka nokuphuhlisa i-leukemia ye-myeloid, uhlobo olubi nolunobukrakra lomhlaza.

Ukuhlala ne-myelofibrosis

Ngelixa i-MF ihlala ingazibonakalisi iimpawu kumanqanaba ayo okuqala, ekugqibeleni inokubangela iingxaki ezinzulu, kubandakanya iintlobo zomhlaza ezinobundlongondlongo. Sebenza nogqirha wakho ukumisela elona khondo lilungileyo lonyango kunye nokuba ungazilawula njani iimpawu zakho. Ukuhlala ne-MF kunokuba noxinzelelo, ke unokukufumanisa kuluncedo ukufuna inkxaso kumbutho ofana neLeukemia kunye neLymphoma Society okanye iMyeloproliferative Neoplasm Research Foundation. Yomibini le mibutho inokukunceda ufumane amaqela enkxaso yasekhaya, uluntu lwase-intanethi, kunye nezixhobo zemali kunyango.