Isu lokwelapha le-primary myelofibrosis (PMF) lisekelwe ekuhlukaniseni ubungozi.Ngenxa yokuhlukahluka kokubonakaliswa komtholampilo kanye nezinkinga okufanele kubhekwane nazo ezigulini ze-PMF, amasu okwelapha kudingeka acabangele isifo sesiguli kanye nezidingo zomtholampilo.Ukwelashwa kokuqala nge-ruxolitinib (i-Jakavi/Jakafi) ezigulini ezinobende olukhulu kubonise ukuncipha okukhulu kwe-spleen futhi kwakuzimele esimweni sokushintsha umshayeli.Ukuncipha okukhulu kobende kuphakamisa ukubikezelwa okungcono.Ezigulini ezisengozini encane engenaso isifo esibalulekile emtholampilo, zingabhekwa noma zifakwe ezivivinyweni zomtholampilo, ngokuhlolwa okuphindaphindiwe njalo ezinyangeni ezi-3-6.I-RuxolitinibUkwelashwa kwezidakamizwa (i-Jakavi/Jakafi) kungaqalwa ezigulini ezisengozini ephansi noma ephakathi-1 ezethula nge-splenomegaly kanye/noma isifo somtholampilo, ngokusho kwemihlahlandlela yokwelapha ye-NCCN.
Ezigulini ezisengozini emaphakathi-2 noma ezisengozini enkulu, i-allogeneic HSCT iyakhethwa.Uma ukufakelwa kabusha kungatholakali, i-ruxolitinib (i-Jakavi/Jakafi) iyanconywa njengendlela yokwelashwa yomugqa wokuqala noma ukungena ezinhlolweni zokwelashwa.I-Ruxolitinib (i-Jakavi/Jakafi) ukuphela kwesidakamizwa esivunyelwe okwamanje emhlabeni wonke esiqondise endleleni esebenza ngokweqile ye-JAK/STAT, i-pathogenesis ye-MF.Izifundo ezimbili ezishicilelwe ku-New England Journal kanye ne-Journal of Leukemia & Lymphoma ziphakamisa ukuthi i-ruxolitinib (i-Jakavi/Jakafi) ingase inciphise kakhulu lesi sifo futhi ithuthukise izinga lokuphila ezigulini ezine-PMF.Ezigulini ezinobungozi obuphakathi kwe-2 kanye ne-MF, i-ruxolitinib (i-Jakavi / Jakafi) yakwazi ukunciphisa i-spleen, ukuthuthukisa izifo, ukuthuthukisa ukusinda, nokuthuthukisa i-pathology yomnkantsha, ukuhlangabezana nemigomo eyinhloko yokuphathwa kwezifo.
I-PMF inethuba lezehlakalo lonyaka elingu-0.5-1.5/100,000 futhi linesibikezelo esibi kakhulu sawo wonke ama-MPN.I-PMF ibonakala nge-myelofibrosis kanye ne-extramedullary hematopoiesis.Ku-PMF, i-bone marrow fibroblasts ayitholakali kuma-clones angavamile.Cishe ingxenye eyodwa kwezintathu yeziguli ezine-PMF azinazo izimpawu ngesikhathi sokuxilongwa.Izikhalazo zihlanganisa ukukhathala okukhulu, i-anemia, ukungakhululeki kwesisu, isifo sohudo ngenxa yokusutha ngaphambi kwesikhathi noma i-splenomegaly, ukopha, ukuncipha kwesisindo, kanye ne-peripheral edema.I-Ruxolitinib(i-Jakavi/Jakafi) yagunyazwa ngo-Agasti 2012 ukuze zelashwe i-myelofibrosis esengozini emaphakathi noma ephezulu, okuhlanganisa ne-primary myelofibrosis.Njengamanje umuthi utholakala emazweni angaphezu kuka-50 emhlabeni jikelele.