Ingabe uhlala uzizwa ukhathele futhi ukhathele ngaphandle kwesizathu? Ingabe unezibazi emzimbeni wakho wonke? Ingabe uzizwa usinda kancane noma uzizwa ugcwele ohlangothini lwesobunxele lwesisu sakho? Kungase kube nesizathu esibucayi ngemuva kwalezi zinto esingazicabangi. Leso yisimo esibizwa ngokuthi i-myelofibrosis. Ungesabi uma uzwa leli gama. Sizoxoxa ngokuthi liyini, kungani lenzeka, yiziphi izimpawu, nokuthi yiziphi izindlela zokwelapha ngendlela elula kakhulu ongayiqonda.
Kalula nje, iyini i-myelofibrosis?
Cabanga ngamathambo amakhulu angaphakathi emizimbeni yethu njengezimboni zokwenza igazi. Lezi zimboni sizibiza ngokuthi umnkantsha wamathambo . Kungaphakathi kwalo mnkantsha lapho kukhiqizwa khona zonke izinhlobo ezintathu zamangqamuzana egazi abalulekile emizimbeni yethu.
1. Amaseli Egazi Abomvu: Athwala umoya-mpilo emzimbeni wonke.
2. Amaseli Amhlophe Egazi: Alwa nezifo namagciwane.
3. Ama-Platelet: Akha amahlule egazi futhi ayeke ukopha.
I-Myelofibrosis uhlobo olungavamile lomdlavuza wegazi lapho umongo wamathambo, imboni eyenza igazi, ugcwala kancane kancane izicubu zesilonda. Njengoba nje ukhula luthatha kancane kancane insimu evundile, lesi silonda sivimbela umongo wamathambo ekwenzeni amaseli egazi ngendlela evamile.
Ngenxa yokuthi lo msebenzi awenziwa kahle, i-spleen yethu, okuyilungu elingaphezulu kwesobunxele sesisu, izama ukusiza ngalo msebenzi. Okusho ukuthi, i-spleen iqala ukwenza igazi. Kodwa lowo akuwona umsebenzi wayo oyinhloko. Ngenxa yalokhu, i-spleen iyagcwala ngokweqile, futhi kancane kancane iqala ukuba nkulu futhi ivuvuke. Iziguli eziningi ziba nezimpawu ngalokhu kuvuvukala kwe-spleen.
Lesi isifo esivame ukuba yisifo sokuphila konke, esivame ukuthuthuka kancane kakhulu. Kodwa-ke, ngezinye izikhathi singaba sibi kakhulu ngokushesha, kanti kwabanye abantu singathuthuka sibe yi-acute leukemia. Yingakho udokotela wakho ezokuqapha eduze ngemva kokuxilongwa.
Ingabe zikhona izinhlobo eziyinhloko ze-myelofibrosis?
Yebo, kunezinhlobo ezimbili eziyinhloko zalokhu.
- I-Myelofibrosis Eyinhloko: Lolu uhlobo oluvame kakhulu. Luvela ngokuzenzakalelayo, ngaphandle kwethonya lanoma yisiphi esinye isifo.
- I-Myelofibrosis Yesibili:Lokhu kwenzeka lapho ezinye izimo ezihlobene negazi ziba zimbi kakhulu. Isibonelo, umuntu one-Essential Thrombocythemia noma i-Polycythemia Vera angaba ne-myelofibrosis ngokuhamba kwesikhathi. Zonke lezi zifo zingeqembu lomdlavuza wegazi elibizwa ngokuthi i-Myeloproliferative Neoplasms (MPNs). Kalula nje, zonke lezi zifo zibangela ukuthi umongo wethambo ukhiqize uhlobo olulodwa noma ngaphezulu lwamaseli egazi kunalokho okufanele.
Ziyini izimpawu zalesi sifo? Sazi kanjani?
Esikhathini esiningi, lesi sifo sikhula kancane kakhulu. Ngakho-ke, singaqhubeka iminyaka eminingi ngaphandle kokubonisa izimpawu. Lapho izimpawu ziqala ukubonakala, into yokuqala efika engqondweni ukukhathala okukhulu . Lokhu kungenxa yokuntuleka kwamangqamuzana abomvu egazi emzimbeni, okuyi-anemia. Kanye nalokhu, ngenxa yokwanda kwe-spleen, ungase uzizwe usinda, ubuhlungu, noma umuzwa wokugcwala engxenyeni ephezulu kwesobunxele yesisu.
Ake sibone ukuthi yiziphi ezinye izimpawu ezikuleli thebula elingezansi.
| Isibonakaliso | Incazelo elula |
|---|---|
| Ubuhlungu bamathambo namalunga | Ubuhlungu buvela ngaphakathi kwamathambo ngenxa yezinguquko emnkantsheni wethambo. |
| Ukuphuka noma ukuphuma kwegazi okulula | Ngenxa yokwehla kwama-platelet, ngisho nokuqunjelwa okuncane kungabangela ukuklwebheka nokopha. |
| Isibindi Esikhulisiwe | Njenge-spleen, isibindi singavuvukala njengoba sizama ukwenza igazi. |
| Imfiva | Ukuzizwa ushisa ngaphandle kwesizathu. |
| Ukutheleleka okuvamile | Izifo zisakazeka kalula ngoba amangqamuzana amhlophe egazi omzimba, asebenza njengamaseli okuzivikela omzimba, ayancishiswa. |
| Ukuzizwa usuthi ngisho nangemva kokudla kancane | Ngenxa yokuthi udengezi luvuvukele futhi isisu sivuvukile, ngisho nangemva kokudla ilayisi elincane, isisu sizizwa sigcwele. |
| Isikhumba esilumayo | Kungase kube nokulunywa okukhulu emzimbeni wonke. |
| Ukujuluka ngokweqile ebusuku | Ukujuluka kakhulu ebusuku kangangokuthi amashidi ayamanzi. |
| Ukwehlisa isisindo ngaphandle kwesizathu | Uma wehlisa isisindo ngaphandle kokuzama. |
Kungani lokhu kwenzeka? Yiziphi izizathu?
Odokotela abakayazi imbangela eqondile ye-myelofibrosis eyinhloko, kodwa banokuqonda okuhle kokuthi kwenzeka kanjani.
Kuqala ngokuguquka kwezakhi zofuzo esiseleni se-stem, iseli lokuqala elakha igazi emnkantsheni wethambo . Iseli elinezakhi zofuzo ezinephutha liba iseli lomdlavuza, lihlukana ngokushesha futhi lenze amakhophi alo. Lawa maseli omdlavuza agcwalisa wonke umnkantshe wethambo.
Lawa maseli amabi awahlali nje lapho. Amakhemikhali awakhiphayo alimaza umongo wamathambo futhi izicubu zesilonda ziqala ukwakheka. Lokhu kubizwa ngokuthi i-fibrosis.
Odokotela bathole ukuthi abantu abaningi abanalesi sifo banezinguquko eziningana ezivamile ezakhiweni zabo zofuzo. Okubaluleke kakhulu kuzo yilezi:
- I-JAK2 (uJack-Two)
- I-CALR (Cal-R)
- I-MPL
Lezi zinguquko zofuzo yizona ezibangela lesi sifo emnkantsheni wethambo.
Ubani osengozini enkulu yokuthola lesi sifo?
Nakuba noma ubani engaba nalesi sifo, abanye abantu basengozini enkulu.
- Abantu abangaphezu kweminyaka engama-60 ubudala: Lesi sifo sivame ukutholakala kubantu abadala.
- Ukuba khona kwezinye izifo zegazi:Kulabo abanezifo ezifana ne-'Essential Thrombocytosis' noma i-'Polycythemia Vera', esikhulume ngazo ngaphambilini.
- Ukuchayeka emisebeni e-ionizing: Ukuchayeka emithamo ephezulu yemisebe. (Lokhu akuvamile kakhulu).
- Ukuchayeka kumakhemikhali athile: Ukuchayeka isikhathi eside kumakhemikhali asetshenziswa emafektri, njenge-benzene (lokhu nakho akuvamile kakhulu).
Yiziphi izinkinga ezingaba khona ngenxa yalesi sifo?
Njengoba i-myelofibrosis iqhubeka, kungaba nezinkinga ezahlukahlukene.
Ukwehla kokukhiqizwa kwamaseli egazi
Uma izicubu zesilonda ziqoqana emnkantsheni wethambo, azikwazi ukwenza amangqamuzana egazi aphilile. Lokhu kungaholela ekunciphiseni kwamangqamuzana abomvu egazi, okubangela i-anemia , kanye nokwehla kwama-platelet, okwandisa ingozi yokopha.
Ukwakheka kwamaseli egazi angaphandle kwesibindi avela emnkantsheni wethambo
Uma umongo wethambo ungakwazi ukwenza umsebenzi wawo, izitho ezifana nobende nesibindi ziqala ukwenza igazi. Lokhu kungabangela ukuthi lezo zitho zivuvuke futhi kuthinte ukusebenza kwazo.
Umfutho Wegazi Ophakeme ku-Portal Vein
Umthambo we-portal uwumthambo wegazi oyinhloko othwala igazi lisuka ebhodweni liye esibindini. Ubhodwe okhulisiwe ungavimba ukugeleza kwegazi kulo mthambo, kwandise ingcindezi engaphakathi kwawo. Lokhu kungaholela ekuphumeni kwegazi okukhulu.
Ukuphenduka kube yi-leukemia ebukhali
Kwabanye iziguli, i-myelofibrosis ingagcina ikhule ibe uhlobo lwe-leukemia olubi kakhulu nolusakazeka ngokushesha olubizwa ngokuthi i-Acute Myeloid Leukemia (AML).
Udokotela usithola kanjani lesi sifo ngokunembile?
Ngemva kokulalela izimpawu zakho nokukuhlola, udokotela uzoyala ukuhlolwa okuningana ukuqinisekisa ukuxilongwa.
1. Ukuhlolwa Kwegazi: Lokhu kuhlola ukuthi inani lamaseli egazi lakho (obomvu, omhlophe, ama-platelet) lijwayelekile noma alijwayelekile. Kuhlola nokuthi ukuma kwamaseli egazi akujwayelekile yini.
2. Ukuhlolwa Kwezithombe: Kungenziwa i -CT scan noma i-ultrasound scan ukuze kubonwe ukuthi ngabe ubende noma isibindi sakho sikhulile yini. I -MRI scan ingasiza futhi ekuboneni ukuthi kukhona yini izibazi emnkantsheni wethambo.
3. I-Bone Marrow Biopsy: Lolu vivinyo olubaluleke kakhulu lokuqinisekisa lesi sifo. Kulokhu, kuthathwa isampula encane yomnkantsha, ngokuvamile ethanjeni lesinqe noma kwelinye ithambo elikhulu, ngaphansi komuthi wokubulala izinzwa okhanyayo. Uthunyelwa elabhorethri ukuze kutholakale ukuthi zingakanani izibazi ezikhona, ukuthi ngabe kukhona yini amangqamuzana omdlavuza, nokuthi ngabe kukhona yini izinguquko zofuzo ezifana ne-`JAK2` esikhulume ngayo ngaphambili.
Ngokusekelwe emiphumeleni yalezi zivivinyo, udokotela wakho uzonquma ukuthi isifo sakho sisesigabeni sokuqala (prefibrotic) noma sisesigabeni esithuthukile (obala). Bazohlola nezinga lakho lobungozi (eliphansi, eliphakathi, noma eliyingozi kakhulu) ngokusekelwe ezimpawini zakho kanye nohlobo lokuguqulwa kwezakhi zofuzo. Ukwelashwa kuhlelwa ngokusekelwe kuleli zinga lobungozi.
Yiziphi izindlela zokwelapha lokhu?
Indlela yokwelashwa incike ezingeni lengozi yesifo sakho, ubukhali bezimpawu zakho, ubudala bakho, kanye nempilo yakho iyonke.
Into ebalulekile ukuthi umuntu osengozini encane futhi ongenazo izimpawu angase anganikwa noma yimiphi imithi ekuqaleni. Kunalokho, udokotela uzohlala ekuqapha (Ukulinda Ngokuqaphela).
Kunezindlela eziningana zokwelapha izimo eziyingozi eziphakathi nendawo kanye neziyingozi kakhulu . Umgomo oyinhloko wokwelapha ukulawula izimpawu nokuvimbela lesi sifo ukuthi singabi sibi kakhulu.
| Izimpawu/isimo eselashwayo | Izindlela zokwelapha ezisetshenziswayo |
|---|---|
| Ngezimpawu ezijwayelekile kanye nobende okhuphukile | Imithi yokwelapha eqondiwe ebizwa ngokuthi i-JAK inhibitors. Izibonelo: I-Ruxolitinib (Jakafi®), i-Fedratinib (Inrebic®), i-Pacritinib (Vonjo®), i-Momelotinib (Ojjaara®). Lezi zinciphisa usayizi we-spleen futhi zilawula izimpawu ezifana nokukhathala nokujuluka ebusuku. |
| Nge-Anemia | - Imithi esiza ekukhiqizeni amaseli abomvu egazi (isib. i-Erythropoietin) - Eminye imithi (i-Androgens, i-Immunomodulators, i-Corticosteroids) - Ukumpontshelwa igazi uma kudingeka |
| Ngobende olukhulisiwe (ezinye izindlela) | - Imithi yokwelapha ngamakhemikhali - Ukwelashwa ngemisebe kubende - Lokhu akuvamile ukusetshenziswa. - Ukususwa kwe-splenectomy - Lena inqubo engavamile kakhulu futhi eyingozi. |
Ukufakelwa Kweseli Lesiqu
Lena ukuphela kwendlela yokwelapha engayelapha ngokuphelele i-myelofibrosis. Kodwa-ke, inqubo eyingozi kakhulu enemiphumela emibi emibi kakhulu. Ngakho-ke, ayifaneleki kubo bonke iziguli. Ngokuvamile, odokotela batusa lokhu kwelashwa kubantu abasengozini enkulu, intsha kanye nabantu abangenazo ezinye izifo ezimbi kakhulu.
Lokhu kuhilela ukubhubhisa ngokuphelele umongo wethambo wesiguli onesifo nokunikeza isiguli amaseli okuqala atholwe kumnikeli onempilo.
Umlayezo Wokuya Nawe Ekhaya
- I-Myelofibrosis umdlavuza wegazi obangela izibazi nokukhula komnkantsha. Nakuba lokhu kuyesabisa, ungesabi.
- Uma unezimpawu ezifana nokukhathala okukhulu okungachazeki, ukusinda ohlangothini lwesobunxele lwesisu sakho, noma ukuphuka kalula, ungazinaki futhi ubone udokotela ngokushesha.
- Ukuhlolwa kwegazi kanye ne-bone marrow biopsy kubalulekile ukuze kutholakale ukuxilongwa okunembile.
- Ukuthi udinga ukwelashwa noma cha, nokuthi hlobo luni lokwelashwa olufanele, kuncike ezingeni lengozi yesifo sakho kanye nezimpawu zakho.
- Khuluma nodokotela wakho ngokukhululekile. Buza konke ukwesaba kwakho, ukungabaza, nemibuzo. Qaphela imiphumela emibi yokwelashwa.
- Njengoba isayensi yezokwelapha ithuthuka, izindlela ezintsha zokwelapha lesi sifo ziyavela njalo. Ngakho-ke, bhekana nokwelashwa ngethemba kanye nokuhle.
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