Njira yochiritsira ya primary myelofibrosis (PMF) imachokera ku chiopsezo cha stratification. Chifukwa cha mitundu yosiyanasiyana ya mawonetseredwe azachipatala ndi nkhani zomwe ziyenera kuchitidwa kwa odwala a PMF, njira zothandizira chithandizo ziyenera kuganizira za matenda a wodwalayo komanso zosowa zachipatala. Chithandizo choyambirira cha ruxolitinib (Jakavi / Jakafi) mwa odwala omwe ali ndi ndulu yayikulu adawonetsa kuchepa kwakukulu kwa ndulu ndipo anali wodziyimira pawokha pakusintha kwa dalaivala. Kuchuluka kwa kuchepa kwa ndulu kumapereka chidziwitso chabwinoko. Odwala omwe ali pachiwopsezo chochepa omwe alibe matenda oopsa, amatha kuwonedwa kapena kulowetsedwa m'mayesero azachipatala, ndikuwunikanso kubwereza miyezi 3-6 iliyonse.Ruxolitinib(Jakavi / Jakafi) mankhwala ochizira mankhwala amatha kuyambika kwa odwala omwe ali ndi chiopsezo chochepa kapena chapakati-1 omwe amapereka splenomegaly ndi / kapena matenda opatsirana, malinga ndi malangizo a mankhwala a NCCN.
Kwa odwala omwe ali pachiwopsezo chapakati-2 kapena omwe ali pachiwopsezo chachikulu, allogeneic HSCT ndiyokondedwa. Ngati kulowetsedwa kulibe, ruxolitinib (Jakavi/Jakafi) akulimbikitsidwa ngati njira yoyamba yothandizira mankhwala kapena kulowa m'mayesero achipatala. Ruxolitinib (Jakavi/Jakafi) ndiye mankhwala okhawo ovomerezeka padziko lonse lapansi omwe amalimbana ndi njira ya JAK/STAT yowonjezereka, pathogenesis ya MF. Maphunziro awiri omwe adasindikizidwa mu New England Journal ndi Journal of Leukemia & Lymphoma amasonyeza kuti ruxolitinib (Jakavi / Jakafi) ikhoza kuchepetsa kwambiri matendawa ndikusintha moyo wa odwala omwe ali ndi PMF. Odwala omwe ali ndi chiopsezo chapakati-2 ndi MF, ruxolitinib (Jakavi / Jakafi) adatha kuchepetsa ndulu, kupititsa patsogolo matenda, kupititsa patsogolo moyo, ndi kupititsa patsogolo matenda a mafupa, kukwaniritsa zolinga zazikulu za matenda.
PMF ili ndi mwayi wapachaka wa 0.5-1.5 / 100,000 ndipo ili ndi chidziwitso choyipa kwambiri cha MPNs zonse. PMF imadziwika ndi myelofibrosis ndi extramedullary hematopoiesis. Mu PMF, ma fibroblasts a mafupa samatengedwa kuchokera ku ma clones osadziwika. Pafupifupi gawo limodzi mwa magawo atatu a odwala omwe ali ndi PMF alibe zizindikiro panthawi yomwe amadwala. Zodandaula zimaphatikizapo kutopa kwakukulu, kuchepa kwa magazi m'thupi, kusapeza bwino m'mimba, kutsekula m'mimba chifukwa cha kukhuta koyambirira kapena splenomegaly, magazi, kuchepa thupi, ndi edema yotumphukira.Ruxolitinib(Jakavi/Jakafi) idavomerezedwa mu Ogasiti 2012 pochiza myelofibrosis yapakatikati kapena yayikulu, kuphatikiza primary myelofibrosis. Mankhwalawa akupezeka m'maiko opitilira 50 padziko lonse lapansi.