Iingxaki zeMyelofibrosis kunye neendlela zokuNciphisa umngcipheko wakho

Umxholo

• Ipeni eyandisiweyo
• Amathumba (ukukhula okungadibaniyo nomhlaza) kwamanye amalungu omzimba wakho
• Uxinzelelo lwePortal
• Ubalo lweplatelet ephantsi
• Intlungu kunye nentlungu edibeneyo
• Igawuthi
• Anemia kakhulu
• I-leukemia ye-myeloid efanelekileyo (AML)
• Ukunyanga iingxaki ze-MF
• Ukunciphisa umngcipheko weengxaki ze-MF
• Ukuthatha

I-Myelofibrosis (MF) luhlobo olungapheliyo lomhlaza wegazi apho izicwili ezibomvu kumongo wethambo zinciphisa ukuveliswa kweeseli zegazi ezisempilweni. Ukunqongophala kweeseli zegazi kubangela uninzi lweempawu kunye neengxaki ze-MF, ezinje ngokudinwa, ukutyumka ngokulula, umkhuhlane, kunye nethambo okanye iintlungu ezidibeneyo.

Abantu abaninzi abafumani naziphi na iimpawu kumanqanaba okuqala esi sifo. Njengoko isifo siqhubeka, iimpawu kunye neengxaki eziboshwe kwiiseli zegazi ezingaqhelekanga ziqala ukuvela.

Kubalulekile ukuba usebenze nogqirha wakho ukuze unyange iMF, ngakumbi ngokukhawuleza xa uqala ukufumana iimpawu. Unyango lunokunceda ukunciphisa umngcipheko weengxaki kunye nokwandisa ukusinda.

Nanku ukujonga ngokuthe ngqo kwiingxaki ezinokubakho zeMF kunye nendlela onokuwunciphisa ngayo umngcipheko.

Ipeni eyandisiweyo

I-spleen yakho inceda ukulwa nokusuleleka kunye nokucoca iiseli zegazi ezindala okanye ezonakalisiweyo. Ikwagcina iiseli ezibomvu zegazi kunye neeplatelets ezinceda ukujiya kwegazi lakho.

Xa une-MF, umongo wethambo lakho awunako ukwenza iiseli zegazi ezaneleyo ngenxa yokuqhekeka. Iiseli zegazi ekugqibeleni ziveliswa ngaphandle komongo wethambo kwamanye amalungu omzimba wakho, njengepeni lakho.

Oku kubizwa ngokuba yi-extramedullary hematopoiesis. Udakada ngamanye amaxesha luba lukhulu ngokungaqhelekanga njengoko lusebenza nzima ukwenza ezi seli.

I-spleen eyandisiweyo (splenomegaly) inokubangela iimpawu ezingathandekiyo. Ingabangela iintlungu zesisu xa ityhalela phezulu kwamanye amalungu kwaye ikwenze uzive ugcwele nangona ungakhange utye kakhulu.

Amathumba (ukukhula okungadibaniyo nomhlaza) kwamanye amalungu omzimba wakho

Xa iiseli zegazi ziveliswa ngaphandle komongo wethambo, amathumba angenomdla wokukhulisa iiseli zegazi ngamanye amaxesha enza kwezinye iindawo zomzimba.

Ezi tumors zinokubangela ukopha ngaphakathi kwenkqubo yakho yamathumbu. Oku kunokukwenza ukuba ukhohlele okanye uchithe igazi. Izidumba zinokucinezela intambo yakho yomqolo okanye zibangele ukuxhuzula.

Uxinzelelo lwePortal

Igazi ligeleza ukusuka kudakada liye esibindini lityhutyha umthambo. Ukunyuka kwegazi okwandileyo kwipeni eyandisiweyo kwi-MF kubangela uxinzelelo lwegazi oluphezulu kwi-portal vein.

Ukonyuka koxinzelelo lwegazi ngamanye amaxesha kunyanzela igazi elingaphezulu kwesisu kunye nesisu. Oku kunokuqhekeza imithambo encinci kwaye kubangele ukopha. Malunga nabantu abane-MF banamava kule ngxaki.

Ubalo lweplatelet ephantsi

Iiplatelets egazini zinceda igazi lakho ukuba ligutyungeke emva kokulimala. Inani leplatelet linokuwela ngaphantsi kwesiqhelo njengoko iMF iqhubeka. Inani eliphantsi leeplatelets laziwa ngokuba yi-thrombocytopenia.

Ngaphandle kweeplatelets ezaneleyo, igazi lakho alinakugquma ngokufanelekileyo. Oku kunokukwenza ukuba wophe ngokulula.

Intlungu kunye nentlungu edibeneyo

I-MF inokuluqinisa umongo wethambo lakho. Inokubangela nokudumba kwizicubu ezinxulumene namathambo. Oku kukhokelela kwithambo kunye nentlungu edibeneyo.

Igawuthi

I-MF ibangela ukuba umzimba uvelise i-uric acid engaphezulu kwesiqhelo. Ukuba i-uric acid iyacacisa, ngamanye amaxesha ihlala kumalungu. Oku kubizwa ngokuba yi-gout. Igawuthi inokubangela ukudumba nokuqaqanjelwa kwamalungu.

Anemia kakhulu

Inani elibomvu leeseli ezibomvu elibizwa ngokuba yi-anemia luphawu oluqhelekileyo lwe-MF. Ngamanye amaxesha i-anemia iba qatha kwaye ibangele ukudinwa, ukubetha, kunye nezinye iimpawu.

I-leukemia ye-myeloid efanelekileyo (AML)

Phantse iipesenti ezili-15 ukuya kuma-20 zabantu, i-MF iqhubela phambili kwifom yomhlaza eyaziwa ngokuba yi-acute myeloid leukemia (AML). I-AML ngumhlaza ohamba ngokukhawuleza wegazi kunye nomongo wethambo.

Ukunyanga iingxaki ze-MF

Ugqirha wakho unokuyalela unyango ngeendlela ezahlukeneyo ukulungisa iingxaki ze-MF. Oku kubandakanya:

• I-JAK inhibitors, kubandakanya i-ruxolitinib (Jakafi) kunye ne-fedratinib (Inrebic)
• iziyobisi ezisebenza ngokuchasene nomzimba, ezifana ne-thalidomide (Thalomid), lenalidomide (Revlimid), ii-interferon, kunye ne-pomalidomide (Pomalyst)
• ii-corticosteroids, ezinje nge-prednisone
• ukususwa kotyando (splenectomy)
• Unyango lwe-androgen
• iziyobisi ngamachiza, njengehydroxyurea

Ukunciphisa umngcipheko weengxaki ze-MF

Kubalulekile ukusebenzisana nogqirha wakho ukulawula i-MF. Ukubeka iliso rhoqo kubalulekile ekunciphiseni umngcipheko weengxaki ze-MF. Ugqirha wakho unokucela ukuba uze kubalo lwegazi kunye novavanyo lomzimba kube kanye okanye kabini ngonyaka okanye rhoqo kanye ngeveki.

Ukuba okwangoku awunazimpawu kunye ne-MF yomngcipheko ophantsi, abukho ubungqina bokuba uya kuxhamla kungenelelo lwangaphambili. Ugqirha wakho unokulinda ukuqala unyango de imeko yakho iqhubeke.

Ukuba unempawu okanye uphakathi okanye unobungozi obukhulu kwi-MF, ugqirha wakho unokumisela unyango.

I-JAK inhibitors i-ruxolitinib kunye ne-fedratinib ijolise kwindlela engaqhelekanga yomqondiso obangelwe kukutshintsha kwemfuza kwe-MF. La machiza abonakaliswe ukunciphisa ubukhulu ubungakanani besilenda kunye nokujongana nezinye iimpawu zokuphelelwa ngamandla kubandakanya iintlungu zethambo kunye nokudibana. Uphando banokuwunciphisa kakhulu umngcipheko weengxaki kunye nokwandisa ukusinda.

Ukufakelwa komongo wethambo kuphela kwonyango olunokuthi lunyange iMF. Kubandakanya ukufumana ukumiliselwa kweeseli zeziqu ezivela kumnikeli esempilweni, othatha indawo yeeseli ezinesiphene ezibangela iimpawu ze-MF.

Le nkqubo inemingcipheko ebalulekileyo kunye neyobungozi engozini yobomi. Ihlala icetyiswa kuphela kubantu abancinci ngaphandle kwezinye iimeko zempilo esele zikho.

Unyango olutsha lwe-MF luhlala luphuhliswa. Zama ukuhlala usexesheni ngophando lwamva nje kwi-MF, kwaye ubuze ugqirha wakho malunga nokuba kufanelekile na ukuba ubhalise kuvavanyo lwezonyango.

Ukuthatha

I-Myelofibrosis ngumhlaza onqabileyo apho ukonakala kugcina umongo wethambo lakho ekuveliseni iiseli zegazi ezoneleyo. Ukuba une-MF ephakathi okanye enobungozi obukhulu, unyango oluninzi lunokujongana neempawu, ukunciphisa umngcipheko wobunzima, kunye nokwandisa ukusinda.

Izilingo ezininzi eziqhubekayo ziyaqhubeka nokuphonononga unyango olutsha. Hlala unxibelelana nogqirha wakho kwaye uxoxe ukuba loluphi unyango olulungele wena.