Myelofibrosis Idiopathic

I-Myelofibrosis uhlobo olungavamile lomdlavuza wamathambo oluphazamisa ukukhiqizwa okujwayelekile kwesigxobo segazi emzimbeni wakho.

I-Myelofibrosis ibangela ukuba kube khona amabala amaningi amathambo, okubangela ukungabikho okukhulu kwegazi okungabangela ubuthakathaka nokuqeda amandla. Ukuba khona kwamabala amathambo kungakwenza futhi ube nenani elincane lamaseli okuhlanganisa igazi abizwa ngama-platelets, okwandisa ingozi yokopha. I-Myelofibrosis ihlala ibangela ukukhula kwespleen.

I-Myelofibrosis kubhekwa njengolukhemiya oluqhubekayo—umdlavuza oqeda izicubu zokwakha igazi emzimbeni. I-Myelofibrosis iyingxenye yeqembu lezimpawu ezibizwa ngokuthi yi-myeloproliferative disorders.

I-Myelofibrosis ingenzeka yodwa (i-primary myelofibrosis) noma ingathuthukiswa kwenye inkinga yamathambo (i-secondary myelofibrosis).

Abanye abantu abane-myelofibrosis abanazo izimpawu futhi bangadingi ukwelashwa ngokushesha. Abanye abanezimpawu ezinzima zomdlavuza bangadinga ukwelashwa okukhulu ngokushesha. Ukwelashwa kwe-myelofibrosis, okuqondene nokululaza izimpawu, kungabandakanya izinketho eziningi.

I-Myelofibrosis ivame ukukhula kancane. Emazingeni ayo okuqala kakhulu, abantu abaningi abanawo ama-sign noma izimpawu.

Njengoba ukuphazamiseka kokukhiqizwa kwamaseli egazi okujwayelekile kuya kwanda, ama-sign nezimpawu zingafaka:

• Ukuzizwa ukhathele, ubuthakathaka noma ukuphelelwa umoya, ngokuvamile ngenxa yokushoda kwegazi
• Ubuhlungu noma ukuqina ngaphansi kwamaphaphu ngakwesobunxele, ngenxa yespleen eyongeziwe
• Ukulunywa kalula
• Ukopha kalula
• Ukukhishwa okukhulu kwezinyembezi ngesikhathi sokulala (ukukhishwa kwezinyembezi ebusuku)
• Ukuvuvukala
• Ubuhlungu lwamathambo

Yenza i-aphoyintimenti nodokotela wakho uma unezimpawu nezincazelo eziqhubekayo ezikukhathazayo.

I-Myelofibrosis ivela lapho amaseli esiqalo emathanjeni oluhlobo oluthile oluthola izinguquko (ukuguqulwa) ku-DNA yawo. Amaseli esiqalo anamandla okubuyela futhi ahlukaniswe amaseli athile amaningi akha igazi lakho—amaseli egazi ebomvu, amaseli egazi amhlophe nama-platelets.

Akucaci ukuthi yini ebangela ukuguqulwa kwe-genetic kumaseli esiqalo emathanjeni oluhlobo oluthile.

Njengoba amaseli esiqalo egazi aguquliwe ebuya futhi ehlukaniswa, adlulisela ukuguqulwa kwawo kumaseli amasha. Njengoba kunamaseli amaningi aguquliwe adalwa, aqala ukuba nemiphumela emibi ekukhiqizweni kwegazi.

Umphumela wokugcina ngokuvamile ukushoda kwamaseli egazi ebomvu—okubangela ukugula kwegazi okuphawuleka ku-myelofibrosis—nokukhulu kwamaseli egazi amhlophe nama-platelets ahlukahlukene. Kubantu abane-myelofibrosis, ubuchopho obuvamile bethambo liba nezikhali.

Kutholwe izinguquko ezithile ze-gene kubantu abane-myelofibrosis. Eyona nto ivamile yinguquko ye-gene ye-Janus kinase 2 (JAK2). Ezinye iinguquko ezingavamile zihlanganisa i-CALR ne-MPL. Abanye abantu abane-myelofibrosis abanazo iinguquko ze-gene ezingabonakali. Ukubona ukuthi lezi nguquko ze-gene zihambisana ne-myelofibrosis yakho kusiza ekuqondeni isimo sakho nokuphathwa kwakho.

Nakuba imbangela ye-myelofibrosis ngokuvamile ingaziwa, izinto ezithile zaziwa ukwanda okuthile ingozi yakho:

• Ubudala. I-Myelofibrosis ingathinta noma ubani, kodwa ivame kakhulu ukuxilongwa kubantu abadala kuno-50.
• Esinye isifo se-blood cell. Ingxenye encane yabantu abane-myelofibrosis bathuthukisa le simo njengoba kuyingxenye ye-essential thrombocythemia noma i-polycythemia vera.
• Ukuvezwa ezintweni ezithile zekhemikhali. I-Myelofibrosis ihlanganiswe nokuvezwa ekhemikhali zezimboni ezifana ne-toluene ne-benzene.
• Ukuvezwa ku-radiation. Abantu ababekwe emazingeni aphezulu kakhulu e-radiation banengozi enkulu ye-myelofibrosis.

Izinkinga ezingase zibe khona ngenxa ye-myelofibrosis zihlanganisa:

• Ubuhlungu. I-spleen enkulu kakhulu ingabangela ubuhlungu esiswini kanye nobuhlungu emhlane.
• Izinkinga zokopha. Njengoba isifo siqhubeka, inani le-platelet yakho liya ehla ngaphansi kokujwayelekile (thrombocytopenia) futhi umsebenzi we-platelet uya onakala. Inani elinganele le-platelets lingabangela ukopha okulula — inkinga oqonde ukuxoxa ngayo nodokotela wakho uma ucabanga nganoma iyiphi uhlobo lokuhlinzwa.
• I-leukemia ebukhali. Abanye abantu abane-myelofibrosis ekugcineni bathuthukisa i-acute myelogenous leukemia, uhlobo lomdlavuza wegazi nengqondo yamathambo oluqhubeka ngokushesha.

Ekubunjweni komgogodla wamathambo, ochwepheshe bezempilo basebenzisa isibopho esincinyane ukuze bakhiphe inani elincane le-marrow yamathambo elikhulu. Ngokuvamile kuthathwa endaweni esemva kwethambo le-hip, elibizwa nangokuthi i-pelvis. I-biopsy yomgogodla wamathambo ivame ukwenziwa ngesikhathi esifanayo. Le nqubo yesibili ikhipha ingxenye encane yethambo lethambo kanye ne-marrow evaliwe.

Izivivinyo nezinqubo ezisetshenziswa ukuthola i-myelofibrosis zihlanganisa:

• Izivivinyo zegazi. Ku-myelofibrosis, ukubalwa kwegazi okuphelele kubonisa amazinga aphansi kakhulu amaseli abomvu egazi, uphawu lwe-anemia oluvamile kubantu abane-myelofibrosis. Ukubalwa kwamaseli amhlophe egazi nama-platelet kuvame ukungavamile, futhi. Ngokuvamile, amazinga amaseli amhlophe egazi aphakeme kunokujwayelekile, nakuba kubantu abathile angase abe ngokujwayelekile noma aphansi kunokujwayelekile. Ukubalwa kwama-platelet kungaba phezulu noma ngaphansi kunokujwayelekile.
• Izivivinyo zobumfanekiso. Izivivinyo zobumfanekiso, ezifana ne-X-rays ne-MRI, zingasetshenziswa ukuqoqa ulwazi oluthe xaxa mayelana ne-myelofibrosis yakho.
• Ukuhlola amaseli e-kensi ngenxa ye-gene mutations. Elebhoratri, odokotela bazohlola amaseli egazi noma amaseli e-marrow yamathambo ngenxa ye-gene mutations, ezifana ne-JAK2, i-CALR ne-MPL. Udokotela wakho usebenzisa ulwazi oluvela kulezi zihloko ukuze anqume i-prognosis yakho kanye nezinketho zakho zokwelashwa.

Ukuhlolwa komgogodla wamathambo. I-biopsy yomgogodla wamathambo kanye nokubunjwa kungaqinisekisa ukuxilongwa kwe-myelofibrosis.

Ekubunjweni komgogodla wamathambo, isibopho sisetshenziswa ukudweba isampula yethambo lethambo kanye ne-marrow evaliwe evela ethambo lakho le-hip. Phakathi nenqubo efanayo, olunye uhlobo lwesibopho lungasetshenziswa ukuhoxa isampula yengxenye elikhulu le-marrow yamathambo yakho. Izampula zifundwa e-laboratory ukuze kunqunywe inani kanye nohlobo lwamaseli atholakala.

Umgomo wokwelapha abantu abaningi abane-myelofibrosis ukunikeza impumuzo emazingeni nasezimpawu zesifo. Kwabanye, ukutshalwa kwe-marrow yamathambo kungase kunikeze ithuba lokuphulukiswa, kodwa le ndlela yokwelapha iyanzima emzimbeni futhi ingase ingabi yilona khetho kubantu abaningi.

Ukuze kunqunywe ukuthi yiziphi izindlela zokwelapha i-myelofibrosis ezinethuba elikhulu lokukusiza, udokotela wakho angase asebenzise isikali esisodwa noma ngaphezulu ukuhlola isimo sakho. Lezi zikali zibheka izici eziningi zomdlavuza wakho nempilo yakho yonke ukuze unikezwe isigaba sobungozi esibonisa ukubhebhetheka kwesifo.

i-Myelofibrosis eyingozi encane ingase ingadingi ukwelashwa okusheshayo, kuyilapho abantu abane-myelofibrosis eyingozi enkulu bangase bacabangele ukwelashwa okunamandla, njengokutshalwa kwe-marrow yamathambo. Nge-myelofibrosis eyingozi ephakathi, ukwelashwa kuvame ukuqondiswa ekuphatheni izimpawu.

Ukwelashwa kwe-myelofibrosis kungase kungadingeki uma ungenawo izimpawu. Ungase ungadingi ukwelashwa ngokushesha uma ungenayo i-spleen enkulu futhi ungenayo i-anemia noma i-anemia yakho ilukhuni kakhulu. Kunokwelashwa, udokotela wakho cishe uzolandelela impilo yakho eduze ngokuhlola okuvamile nokukhetha, ebuka noma yiziphi izimpawu zokukhula kwesifo. Abanye abantu bahlala bengenawo izimpawu iminyaka eminingi.

Uma i-myelofibrosis ibangele i-anemia ebuhlungu, ungase ucabangele ukwelashwa, njengokuthi:

• Ukuthuthukiswa kwegazi. Uma une-anemia ebuhlungu, ukuthuthukiswa kwegazi okuphindaphindiwe kungandisa inani lamaseli abomvu egazi futhi kuphaphe izimpawu ze-anemia, njengokukhathala nobuthakathaka. Ngezinye izikhathi, imithi ingasiza ekuthuthukiseni i-anemia.
• Ukwelashwa kwe-Androgen. Ukuthatha inguqulo ye-hormone yabesilisa ye-androgen kungase kukhuthaze ukukhiqizwa kwamaseli abomvu egazi futhi kungase kuthuthukise i-anemia ebuhlungu kwabanye abantu. Ukwelashwa kwe-Androgen kunengozi, kufaka phakathi ukonakala kwesibindi nemiphumela yokwenza abesifazane babe ngamadoda.
• I-Thalidomide nezinye izidakamizwa ezihlobene. I-Thalidomide (Thalomid) nezinye izidakamizwa ezihlobene i-lenalidomide (Revlimid) zingasiza ekuthuthukiseni inani lamaseli egazi futhi zingase zikhiphe i-spleen enkulu. Lezi zidakamizwa zingase zihlanganiswe nezinye izidakamizwa ze-steroid. I-Thalidomide nezinye izidakamizwa ezihlobene zinezinhlobo zeziphumo ezingcolile zokuzalwa futhi zidinga izinyathelo ezikhethekile.

Uma i-spleen enkulu ibangele izinkinga, udokotela wakho angase akukhuthaze ukwelashwa. Izindlela zakho zingafaka:

• Ukwelashwa okuqondiswe ngqo kwesidakamizwa. Ukwelashwa okuqondiswe ngqo kwesidakamizwa kugxila ezintweni ezingavamile ezikhona ngaphakathi kwamaseli omdlavuza. Ukwelashwa okuqondiswe ngqo kwe-myelofibrosis kugxila kumaseli ane-mutation ye-JAK2 gene. Lezi zindlela zokwelapha zisetshenziswa ukunciphisa izimpawu ze-spleen enkulu.
• I-Chemotherapy. I-Chemotherapy isebenzisa imithi enamandla ukubulala amaseli omdlavuza. Imithi ye-Chemotherapy inganciphisa usayizi we-spleen enkulu futhi iphaphe izimpawu ezihlobene, njengokuhlungu.
• Ukususwa kwe-spleen ngokukhanda (splenectomy). Uma i-spleen yakho ikhula kangangokuba ikuhlunguza futhi iqale ukubangela izinkinga eziyingozi — futhi uma ungaphenduli ezinye izindlela zokwelapha — ungase uzuze ngokususa i-spleen yakho ngokukhanda.

Ngokungafani, kufaka phakathi ukutheleleka, ukuphuza igazi kakhulu nokwakheka kwama-blood clot okuholela e-stroke noma i-pulmonary embolism. Ngemva kwesenzo, abanye abantu bahlangabezana nokwanda kwesibindi nokukhula okungavamile kwenani le-platelet.

• Ukwelashwa ngomgogodla. Umgogodla usebenzisa imisebe enamandla, njenge-X-rays namaprotons, ukubulala amaseli omdlavuza. Ukwelashwa ngomgogodla kungasiza ekuthuthukiseni usayizi we-spleen uma ukususa ngokukhanda kungelona ikhetho.

Ukususwa kwe-spleen ngokukhanda (splenectomy). Uma i-spleen yakho ikhula kangangokuba ikuhlunguza futhi iqale ukubangela izinkinga eziyingozi — futhi uma ungaphenduli ezinye izindlela zokwelapha — ungase uzuze ngokususa i-spleen yakho ngokukhanda.

Ngokungafani, kufaka phakathi ukutheleleka, ukuphuza igazi kakhulu nokwakheka kwama-blood clot okuholela e-stroke noma i-pulmonary embolism. Ngemva kwesenzo, abanye abantu bahlangabezana nokwanda kwesibindi nokukhula okungavamile kwenani le-platelet.

Ukutshalwa kwe-marrow yamathambo, okuthiwa futhi ukutshalwa kwama-stem cell, yindlela yokushintsha i-marrow yakho yamathambo esigula usebenzisa ama-stem cell egazi anempilo. Nge-myelofibrosis, le ndlela isebenzisa ama-stem cell asuka kumnikelo (ukutshalwa kwama-stem cell okuvela kwabanye).

Lokhu kwelashwa kunethuba lokuphulukisa i-myelofibrosis, kodwa futhi kunethuba elikhulu lemiphumela emibi eyingozi ebomini, kufaka phakathi ingozi yokuthi ama-stem cell amasha azosabela ezicini ezempilo zomzimba wakho (isifo se-graft-versus-host).

Abantu abaningi abane-myelofibrosis, ngenxa yobudala, ukuzinza kwesifo noma ezinye izinkinga zempilo, abafanelekeli lokhu kwelashwa.

Ngaphambi kokutshalwa kwe-marrow yamathambo, uthola i-chemotherapy noma ukwelashwa ngomgogodla ukubhubhisa i-marrow yakho yamathambo esigula. Khona-ke uthola ukufakwa kwama-stem cell asuka kumnikelo ofanelekayo.

Ukukhathalela okuphumuzayo ukunakekelwa kwezokwelapha okukhethekile okuqondiswe ekunikezeni impumuzo ebuhlungwini nasezinye izimpawu zesifo esinzima. Ochwepheshe bokukhathalela okuphumuzayo basebenza nawe, umndeni wakho nabanye odokotela bakho ukunikeza ukwesekwa okwengeziwe okuhlanganisa ukunakekelwa kwakho okuqhubekayo. Ukukhathalela okuphumuzayo kungasetshenziswa ngenkathi uthola ezinye izindlela zokwelapha ezinamandla, njengokukhanda, i-chemotherapy noma ukwelashwa ngomgogodla.

Uma ukunakekelwa okuphumuzayo kusetshenziswa kanye nazo zonke ezinye izindlela zokwelapha ezifanele, abantu abane-cancer bangase bazizwe bekhulu futhi baphile isikhathi eside.

Ukukhathalela okuphumuzayo kunikezwa yiqembu lodokotela, oongoti nabanye ochwepheshe abaqeqeshwe ngokukhethekile. Amaqembu okunakekelwa okuphumuzayo agxile ekuthuthukiseni ikhwalithi yempilo yabantu abane-cancer nemindeni yabo. Lolu hlobo lokunakekelwa lunikezwa kanye nezindlela zokwelapha eziphilayo noma ezinye izindlela zokwelapha ongase uzithole.

Ukuphila ne-myelofibrosis kungase kuhilele ukubhekana nobuhlungu, ukungakhululeki, ukungabaza nemiphumela emibi yokwelashwa isikhathi eside. Izinyathelo ezilandelayo zingasiza ekuthuthukiseni inselele futhi zikwenze uzizwe ukhululekile futhi ulawula impilo yakho:

• Funda okwanele ngesimo sakho ukuze uzizwe ukhululekile ukwenza izinqumo. I-Myelofibrosis ayivamile. Ukuze sikusize uthole ulwazi olunembile nolokwethenjwa, cela udokotela wakho ukuba akuqondise emithonjeni efanele. Usebenzisa le mithombo, thola konke ongakwazi nge-myelofibrosis.
• Hlola izindlela zokubhekana nesifo. Uma une-myelofibrosis, ungase ubhekane nokuhlolwa kwegazi okuvamile nokubonisana nodokotela nokukhethwa kwe-marrow yamathambo okuvamile. Ngezinye izinsuku, ungase uzizwe ugula ngisho noma ungabonakali ugula. Futhi ngezinye izinsuku, ungase ugcine nje ugula ngokuba ugula.

Zama ukuthola umsebenzi osikushaya, kungaba i-yoga, ukuzivocavoca, ukuhlangana nabantu noma ukwamukela isimiso somsebenzi esiguquguqukayo. Khuluma nomxhumanisi, umhlengikazi noma umsebenzi wokuxhumana we-oncology uma udinga usizo ekubhekaneni nezinselele zomzwelo zalesi sifo.

Ungase futhi uzuze ngokujoyina iqembu lokusekela, emphakathini wakho noma kwi-inthanethi. Iqembu lokusekela labantu abanesifo esifanayo noma esifanayo, njengokuphazamiseka kwe-myeloproliferative noma olunye ubuhlungu obubalulekile, kungaba umthombo wolwazi oluwusizo, amathiphu asebenzayo nokukhuthazwa.

Hlola izindlela zokubhekana nesifo. Uma une-myelofibrosis, ungase ubhekane nokuhlolwa kwegazi okuvamile nokubonisana nodokotela nokukhethwa kwe-marrow yamathambo okuvamile. Ngezinye izinsuku, ungase uzizwe ugula ngisho noma ungabonakali ugula. Futhi ngezinye izinsuku, ungase ugcine nje ugula ngokuba ugula.

Zama ukuthola umsebenzi osikushaya, kungaba i-yoga, ukuzivocavoca, ukuhlangana nabantu noma ukwamukela isimiso somsebenzi esiguquguqukayo. Khuluma nomxhumanisi, umhlengikazi noma umsebenzi wokuxhumana we-oncology uma udinga usizo ekubhekaneni nezinselele zomzwelo zalesi sifo.