Ngaba uhlala uziva udiniwe kwaye udiniwe ngaphandle kwesizathu? Ngaba unemivumbo emzimbeni wakho wonke? Ngaba uziva ubunzima obuncinci okanye uzive ugcwele kwicala lasekhohlo lesisu sakho? Kusenokuba kukho isizathu esibalulekileyo emva kwezi zinto esingacingi ngaso. Leyo yimeko ebizwa ngokuba yi-myelofibrosis. Musa ukoyika xa usiva eli gama. Siza kuthetha ngokuba yintoni, kutheni isenzeka, zithini iimpawu, kunye nonyango ngendlela elula kakhulu onokuyiqonda.
Ngamafutshane, yintoni i-myelofibrosis?
Cinga ngamathambo amakhulu angaphakathi emizimbeni yethu njengeefektri zokwenza igazi. Ezi fektri sizibiza ngokuba yi-bone marrow . Kukule bone marrow apho zonke iintlobo ezintathu zeeseli zegazi ezibalulekileyo emizimbeni yethu ziveliswa khona.
1. Iiseli zegazi ezibomvu: Zithwala ioksijini emzimbeni wonke.
2. Iiseli zegazi ezimhlophe: Zilwa nezifo kunye neentsholongwane.
3. Iiplatelets: Zinceda ekuvaleni igazi kwaye ziyeke ukopha.
I-Myelofibrosis luhlobo olungaqhelekanga lomhlaza wegazi apho umongo wamathambo, umzi-mveliso wokwenza igazi, uzaliswa kancinci kancinci zizicwili zesilonda. Njengokuba ukhula luthatha kancinci kancinci intsimi echumileyo, esi sicwili sesilonda sithintela umongo wamathambo ekwenzeni iiseli zegazi ngendlela eqhelekileyo.
Ngenxa yokuba lo msebenzi ungenziwanga kakuhle, i-spleen yethu, elilungu elikwicala eliphezulu lasekhohlo lesisu, izama ukunceda kulo msebenzi. Oko kukuthi, i-spleen iqala ukwenza igazi. Kodwa loo nto ayingomsebenzi wayo ophambili. Ngenxa yoku, i-spleen iyasinda kakhulu, kwaye kancinci kancinci iqala ukuba nkulu kwaye idumbe. Izigulane ezininzi ziba neempawu zokudumba kwe-spleen.
Esi sisifo esihlala sisenzeka ubomi bonke, sihlala siqhubeka kancinci. Nangona kunjalo, ngamanye amaxesha sinokuba mandundu ngokukhawuleza, kwaye kwabanye abantu sinokuqhubeka sibe yi-acute leukemia. Yiyo loo nto ugqirha wakho eza kukujonga ngokusondeleyo emva kokuba uxilongiwe.
Ngaba kukho iintlobo eziphambili ze-myelofibrosis?
Ewe, kukho iintlobo ezimbini eziphambili zale nto.
- I-Primary Myelofibrosis: Olu lolona hlobo luxhaphakileyo. Luvela ngokungazi, ngaphandle kwempembelelo yesinye isifo.
- I-Myelofibrosis yesibini:Oku kwenzeka xa ezinye iimeko ezinxulumene negazi ziba nzima. Umzekelo, umntu one-Essential Thrombocythemia okanye iPolycythemia Vera unokufumana i-myelofibrosis ngokuhamba kwexesha. Zonke ezi zifo ziyinxalenye yeqela leemhlaza zegazi ezibizwa ngokuba yiMyeloproliferative Neoplasms (MPNs). Ngamafutshane, zonke ezi zifo zibangela ukuba umongo wethambo uvelise uhlobo olunye okanye ngaphezulu lweeseli zegazi kunokuba kufanele.
Zithini iimpawu zesi sifo? Sizazi njani?
Ixesha elininzi, esi sifo sikhula kancinci kakhulu. Ngenxa yoko, sinokuqhubeka iminyaka emininzi singabonisi zimpawu. Xa iimpawu ziqala ukubonakala, into yokuqala efika engqondweni kukudinwa kakhulu . Oku kungenxa yokunqongophala kweeseli ezibomvu zegazi emzimbeni, okukukuba yi-anemia. Kunye noku, ngenxa yokwanda kwepleen, ungaziva ubunzima, iintlungu, okanye uzive ugcwele kwinxalenye ephezulu yasekhohlo yesisu.
Makhe sibone ukuba zeziphi ezinye iimpawu ezikhoyo kwitheyibhile engezantsi.
| Uphawu | Ingcaciso elula |
|---|---|
| Intlungu yamathambo namalungu | Intlungu ivela ngaphakathi emathanjeni ngenxa yotshintsho kumongo wethambo. |
| Ukutyumza okanye ukopha okulula | Ngenxa yokuncipha kweeplatelets, nokuba sisiqhuma esincinci sinokubangela ukukrweleka nokopha. |
| Isibindi Esikhulisiweyo | Njengespleen, isibindi sinokudumba njengoko sizama ukwenza igazi. |
| Ifiva | Ukuziva ushushu ngaphandle kwesizathu. |
| Usulelo oluqhelekileyo | Izifo zisasazeka lula kuba iiseli ezimhlophe zegazi zomzimba, ezisebenza njengeeseli zokuzikhusela zomzimba, ziyancitshiswa. |
| Ukuziva ugcwele nangona emva kokutya kancinci | Ngenxa yokuba i-spleen idumbile kwaye isisu sivuvukile, nokuba emva kokutya irayisi encinci, isisu sivakala sigcwele. |
| Ulusu olurhawuzelelayo | Kunokubakho ukurhawuzelela okukhulu emzimbeni wonke. |
| Ukubila kakhulu ebusuku | Ukubila kakhulu ebusuku kangangokuba amashiti ayamanzi. |
| Ukunciphisa umzimba ngaphandle kwesizathu | Ukuba unciphisa umzimba ngaphandle kokuzama. |
Kutheni oku kusenzeka? Zithini izizathu?
Oogqirha abakayazi imbangela echanekileyo ye-primary myelofibrosis, kodwa bayiqonda kakuhle indlela eyenzeka ngayo.
Iqala ngokuguquka kwejini kwiseli ye-stem, iseli yokuqala eyenza igazi kwi-bone mongo . Iseli ene-gene enengxaki iba yiseli yomhlaza, yahlulahlula ngokukhawuleza kwaye yenza iikopi zayo. Ezi seli zomhlaza zizalisa yonke i-bone mongo.
Ezi seli zimbi azihlali nje apho. Iikhemikhali ezizikhuphayo zonakalisa umongo wethambo kwaye izicubu zesilonda ziqala ukwakheka. Oku kubizwa ngokuba yi-fibrosis.
Oogqirha bafumanise ukuba abantu abaninzi abanesifo banezakhi zofuzo ezahlukeneyo. Ezona zibalulekileyo zezi:
- UJAK2 (uJACK-ABABINI)
- I-CALR (iCal-R)
- I-MPL
Ezi nguqu zemfuza zizo ezibangela esi sifo kumongo wethambo.
Ngubani osengozini enkulu yokuhlaselwa sesi sifo?
Nangona nabani na enokuba nesi sifo, abanye abantu basengozini enkulu.
- Ngaphezulu kweminyaka engama-60 ubudala: Esi sifo sidla ngokufunyaniswa kubantu abadala.
- Ukubakho kwezinye izifo zegazi:Kwabo banezifo ezifana ne-'Essential Thrombocytosis' okanye i-'Polycythemia Vera', esithethe ngazo ngaphambili.
- Ukuvezwa kwimitha ye-ionizing: Ukuvezwa kwimitha ye-radiation ephezulu. (Oku kunqabile kakhulu).
- Ukuvezwa kwiikhemikhali ezithile: Ukuvezwa ixesha elide kwiikhemikhali ezisetyenziswa kwiifektri, ezifana ne-benzene (oku nako kunqabile kakhulu).
Ziziphi iingxaki ezinokubakho ngenxa yesi sifo?
Njengoko i-myelofibrosis iqhubeka, iingxaki ezahlukeneyo zinokwenzeka.
Ukuveliswa kweeseli zegazi okunciphileyo
Xa izicubu ezibomvu ziqokelelana kumongo wethambo, azikwazi ukwenza iiseli zegazi eziphilileyo. Oku kunokukhokelela ekunciphiseni iiseli ezibomvu zegazi, okubangela i-anemia , kunye nokuncipha kweeplatelets, okwandisa umngcipheko wokopha.
Ukwenziwa kweeseli zegazi ezingaphandle kwesibindi ezivela kumongo wethambo
Xa umongo wethambo ungakwazi ukwenza umsebenzi wawo, amalungu anjengespleen nesibindi aqala ukwenza igazi. Oku kunokubangela ukuba la malungu adumbe aze achaphazele ukusebenza kwawo.
Uxinzelelo lwegazi oluphezulu kwi-Portal Vein
Umthambo we-portal ngumthambo wegazi oyintloko othwala igazi ukusuka kwi-spleen ukuya esibindini. I-spleen ekhulisiweyo inokuthintela ukuhamba kwegazi kulo mthambo, inyuse uxinzelelo olungaphakathi kuwo. Oku kunokukhokelela ekuphumeni kwegazi okukhulu.
Ukujika ube yi-leukemia ebukhali
Kwabanye abaguli, i-myelofibrosis ekugqibeleni inokukhula ibe luhlobo lwe-leukemia olubi kakhulu nolusasazeka ngokukhawuleza olubizwa ngokuba yi-Acute Myeloid Leukemia (AML).
Ugqirha uluchonga njani ngokuchanekileyo olu sifo?
Emva kokumamela iimpawu zakho nokukuxilonga, ugqirha uya kuyalela iimvavanyo ezininzi ukuqinisekisa ukuba unesifo.
1. Uvavanyo lweGazi: Olu vavanyo lujonga ukuba ingaba inani leeseli zakho zegazi (ezibomvu, ezimhlophe, iiplatelets) ziqhelekile okanye aziqhelekanga. Lujonga nokuba imilo yeeseli zegazi ayiqhelekanga na.
2. Uvavanyo lweMifanekiso: I -CT scan okanye i-ultrasound scan ingenziwa ukuze kubonwe ukuba i-spleen okanye isibindi sakho sikhulu na. I -MRI scan inokunceda ekuboneni ukuba kukho naziphi na izibazi kumongo wethambo.
3. I-Bone Marrow Biopsy: Olu lolona vavanyo lubalulekileyo lokuqinisekisa esi sifo. Kule meko, kuthathwa isampuli encinci yomongo wethambo, ngokuqhelekileyo kwithambo lesinqe okanye kwelinye ithambo elikhulu, phantsi kwe-anesthetic ekhaphukhaphu. Ithunyelwa kwilebhu ukuze kuchongwe ukuba zingakanani na izicwili ezikwisilonda ezikhoyo, nokuba kukho iiseli zomhlaza, kwaye nokuba kukho naluphi na utshintsho lwemfuza olufana ne-'JAK2' esithethe ngayo ngaphambili.
Ngokusekelwe kwiziphumo zezi mvavanyo, ugqirha wakho uya kugqiba ukuba isifo sakho sikwinqanaba lokuqala (prefibrotic) okanye sikwinqanaba eliphezulu (overt). Baza kuvavanya nenqanaba lomngcipheko wakho (eliphantsi, eliphakathi, okanye elinomngcipheko ophezulu) ngokusekwe kwiimpawu zakho kunye nohlobo lotshintsho lwemfuza. Unyango lucwangcisiwe ngokusekwe kweli nqanaba lomngcipheko.
Zithini iindlela zonyango zoku?
Indlela yonyango ixhomekeke kwinqanaba lomngcipheko wesifo sakho, ubukhali beempawu zakho, ubudala bakho, kunye nempilo yakho iyonke.
Eyona nto ibalulekileyo kukuba umntu onobungozi obuphantsi kwaye ongenazo iimpawu usenokunganikwa amayeza ekuqaleni. Endaweni yoko, ugqirha uza kuhlala ekujonga (ukulinda ngononophelo).
Kukho iindlela ezahlukeneyo zonyango zeemeko ezinobungozi obuphakathi kunye nezo zinobungozi obuphezulu . Injongo ephambili yonyango kukulawula iimpawu kunye nokuthintela isifo ukuba singabi mandundu.
| Iimpawu/imeko enyangwayo | Iindlela zonyango ezisetyenzisiweyo |
|---|---|
| Ngeempawu eziqhelekileyo kunye ne-spleen ekhulisiweyo | Amayeza onyango ajoliswe kuwo abizwa ngokuba yi-JAK inhibitors. Imizekelo: I-Ruxolitinib (Jakafi®), i-Fedratinib (Inrebic®), i-Pacritinib (Vonjo®), i-Momelotinib (Ojjaara®). La mayeza anciphisa ubukhulu be-spleen kwaye alawula iimpawu ezifana nokudinwa kunye nokubila ebusuku. |
| I-Anemia | - Amayeza anceda ekuveliseni iiseli ezibomvu zegazi (umz. i-Erythropoietin) - Amanye amayeza (Androgens, Immunomodulators, Corticosteroids) - Utofelo-gazi ukuba kuyimfuneko |
| Kwispleen ekhulisiweyo (ezinye iindlela) | - Amayeza e-chemotherapy - Unyango lwemitha kwi-spleen - Olu alusetyenziswa rhoqo. - I-Splenectomy - Le yinkqubo engaqhelekanga kakhulu kwaye inobungozi. |
Ukufakelwa kweeStem Cell
Olu lolona nyango lunokunyanga ngokupheleleyo i-myelofibrosis. Nangona kunjalo, luyinkqubo enobungozi kakhulu eneziphumo ebezingalindelekanga ezibi. Ngenxa yoko, ayifanelekanga kubo bonke abaguli. Ngokwesiqhelo, oogqirha bacebisa olu nyango kubantu abasengozini enkulu, ulutsha kunye nabantu abangenazo ezinye izifo ezibi.
Oku kuquka ukutshabalalisa ngokupheleleyo umongo wethambo wesigulana onesifo kunye nokunika isigulana ii-stem cells ezifunyenwe kumnikeli ophilileyo.
Umyalezo Wokuya Ekhaya
- I-Myelofibrosis ngumhlaza wegazi obangela amanxeba nokukhula komongo wethambo. Nangona oku kuyoyikisa, musa ukoyika.
- Ukuba uneempawu ezifana nokudinwa okungathethekiyo, ubunzima kwicala lasekhohlo lesisu sakho, okanye ukukrweleka okulula, musa ukuzityeshela kwaye ubone ugqirha ngoko nangoko.
- Uvavanyo lwegazi kunye ne-bone marrow biopsy zibalulekile ukuze kufunyanwe isifo esichanekileyo.
- Nokuba uyalufuna okanye awuludingi unyango, kwaye loluphi uhlobo lonyango olufanelekileyo, kuxhomekeke kwinqanaba lomngcipheko wesifo sakho kunye neempawu zakho.
- Thetha nogqirha wakho ngokukhululekileyo. Buza zonke izinto ozixhalabileyo, amathandabuzo, kunye nemibuzo yakho. Qaphela iziphumo ebezingalindelekanga zonyango.
- Njengoko isayensi yezonyango iqhubeka, unyango olutsha lwesi sifo luhlala luvela. Ngoko ke, jongana nonyango unethemba kwaye unethemba.
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