Isicwangciso sonyango se-primary myelofibrosis (PMF) sisekelwe kwi-stratification yengozi.Ngenxa yeendlela ezahlukeneyo zokubonakaliswa kweklinikhi kunye nemiba ekufuneka iqwalaselwe kwizigulane ze-PMF, izicwangciso zonyango kufuneka zithathele ingqalelo isifo somguli kunye neemfuno zeklinikhi.Unyango lokuqala kunye ne-ruxolitinib (i-Jakavi / Jakafi) kwizigulane ezine-spleen enkulu zibonise ukunciphisa okukhulu kwe-spleen kwaye yayizimeleyo kwimeko yokuguqulwa komqhubi.Ubukhulu obukhulu bokuncipha kwe-spleen bucebisa ukuxilongwa okungcono.Kwizigulane ezinomngcipheko ophantsi ongenayo isifo esibalulekileyo seklinikhi, zinokujongwa okanye zingenwe kulingo lwezonyango, kunye novavanyo oluphindaphindiweyo rhoqo kwiinyanga ezi-3-6.Ruxolitinib(i-Jakavi / Jakafi) unyango lweziyobisi lunokuqaliswa kwizigulane eziphantsi okanye eziphakathi kwengozi-1 ezibonisa i-splenomegaly kunye / okanye isifo seklinikhi, ngokwemigaqo yonyango ye-NCCN.
Kwimingcipheko ephakathi-2 okanye izigulane ezinomngcipheko ophezulu, i-allogeneic HSCT ikhethwayo.Ukuba uqhaqho-fakelo alufumaneki, i-ruxolitinib (i-Jakavi/Jakafi) iyacetyiswa njengokhetho lonyango lokuqala okanye ukungena kulingo lwezonyango.I-Ruxolitinib (i-Jakavi/Jakafi) kuphela iyeza elivunyiweyo ngoku kwihlabathi jikelele elijolise kwi-JAK / STAT esebenzayo kakhulu, i-pathogenesis ye-MF.Izifundo ezimbini ezipapashwe kwiNew England Journal kunye neJenali yeLeukemia & Lymphoma icebisa ukuba i-ruxolitinib (i-Jakavi / Jakafi) inokunciphisa kakhulu isifo kunye nokuphucula umgangatho wobomi kwizigulane ezine-PMF.Kwimingcipheko ephakathi-i-2 kunye nezigulane ezinobungozi obukhulu be-MF, i-ruxolitinib (i-Jakavi / Jakafi) yakwazi ukunciphisa i-spleen, ukuphucula izifo, ukuphucula ubomi, kunye nokuphucula i-pathology yamathambo, ukuhlangabezana neenjongo eziphambili zokulawula izifo.
I-PMF ineziganeko zonyaka ezinokwenzeka ze-0.5-1.5 / 100,000 kwaye ine-prognosis embi kakhulu yazo zonke ii-MPNs.I-PMF ibonakaliswe yi-myelofibrosis kunye ne-extramedullary hematopoiesis.Kwi-PMF, i-fibroblasts yomongo wethambo ayithathwa kwii-clones ezingaqhelekanga.Phantse isinye kwisithathu sezigulane ezine-PMF azinazo iimpawu ngexesha lokuxilongwa.Izikhalazo ziquka ukudinwa okubalulekileyo, i-anemia, ukungakhululeki kwesisu, urhudo ngenxa yokuhlutha kwangoko okanye i-splenomegaly, ukopha, ukuncipha komzimba, kunye ne-peripheral edema.Ruxolitinib(I-Jakavi / Jakafi) ivunyiwe ngo-Agasti 2012 yonyango lwe-myelofibrosis ephakathi okanye ephezulu, kubandakanywa ne-primary myelofibrosis.Ichiza okwangoku lifumaneka kumazwe angaphezu kwama-50 kwihlabathi jikelele.