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 ezinobungozi obuphantsi obungenasifo esibalulekileyo seklinikhi, zinokujongwa okanye zingenwe kulingo lwezonyango, kunye novavanyo oluphindaphindiweyo rhoqo kwiinyanga ezi-3-6.I-Ruxolitinib(i-Jakavi / Jakafi) unyango lweziyobisi lunokuqaliswa kwizigulane eziphantsi okanye eziphakathi kwe-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 kwechiza elivunyiweyo kwihlabathi jikelele elijolise kwi-JAK / STAT esebenzayo kakhulu, i-pathogenesis ye-MF. Izifundo ezimbini ezipapashwe kwiNew England Journal kunye neJournal yeLeukemia & Lymphoma zibonisa ukuba i-ruxolitinib (i-Jakavi / Jakafi) inokunciphisa kakhulu isifo kunye nokuphucula umgangatho wobomi kwizigulane ezine-PMF. Kwimingcipheko ephakathi-i-2 kunye nezigulane ze-MF ezinobungozi, i-ruxolitinib (i-Jakavi / Jakafi) yakwazi ukunciphisa i-spleen, ukuphucula izifo, ukuphucula ukusinda, kunye nokuphucula i-pathology ye-bone marrow, 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-MPN. I-PMF ibonakaliswe yi-myelofibrosis kunye ne-extramedullary hematopoiesis. Kwi-PMF, i-fibroblasts yomongo wethambo ayiveli 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, ukwehla kobunzima, kunye ne-peripheral edema.I-Ruxolitinib(i-Jakavi / Jakafi) yavunywa ngo-Agasti 2012 yonyango lwe-myelofibrosis ephakathi okanye ephezulu, kuquka i-primary myelofibrosis. Ichiza okwangoku lifumaneka kumazwe angaphezu kwama-50 kwihlabathi jikelele.