I-Myelofibrosis (MF) ibizwa ngokuthi i-myelofibrosis.Kuphinde kube yisifo esiyivelakancane kakhulu.Futhi imbangela ye-pathogenesis yayo ayaziwa.Ukubonakaliswa okujwayelekile komtholampilo amangqamuzana egazi abomvu ezingane kanye ne-juvenile granulocytic anemia enenani eliphezulu lokudilika kwamaseli abomvu egazi.Ukuphefumula komnkantsha ngokuvamile kubonisa ukulangazelela okomile, kanti ubende uvame ukukhuliswa ngokuphawulekayo ngamazinga ahlukahlukene e-osteosclerosis.
I-myelofibrosis eyinhloko (PMF) i-clonal myeloproliferative disorder (MPD) yamaseli e-hematopoietic stem.Ukwelashwa kwe-primary myelofibrosis kusekela ngokuyinhloko, kuhlanganise nokumpontshelwa igazi.IHydroxyurea inganikezwa nge-thrombocytosis.Iziguli ezisengozini ephansi, ezingenazimpawu zingabonwa ngaphandle kokwelashwa.
Izifundo ezimbili zesigaba III ezingahleliwe (STUDY1 no-2) zenziwa ezigulini ezine-MF (primary MF, post-geniculocytosis MF, noma i-post-primary thrombocythemia MF).Kuzo zombili izifundo, iziguli ezibhalisiwe zazine-splenomegaly ezwakalayo okungenani engu-5 cm ngaphansi kwezimbambo futhi zazisesilinganisweni (izici ezi-2 zokubikezela) noma ubungozi obukhulu (izici ezi-3 noma ngaphezulu zokubikezela) ngokuya nge-International Working Group consensus criteria (IWG).
Umthamo wokuqala we-ruxolitinib ususelwa ekubaleni kweplatelet.I-15 mg kabili ngosuku ezigulini ezinezibalo zeplatelet phakathi kuka-100 no-200 x 10^9/L kanye no-20 mg kabili ngosuku ezigulini ezinezibalo zeplatelet ezingaphezu kuka-200 x 10^9/L.
Imithamo yomuntu ngamunye yanikezwa ngokuvumelana nokubekezelelana nokusebenza kahle kweziguli ezinezibalo zeplatelet phakathi kuka-100 no-125 x 10 ^ 9/L, ngomthamo omkhulu we-20 mg kabili ngosuku;ezigulini ezinezibalo zeplatelet phakathi kuka-75 no-100 x 10^9/L, 10 mg kabili ngosuku;futhi ezigulini ezinezibalo zeplatelet phakathi kuka-50 nangaphansi noma ezilingana no-75 x 10^9/L, izikhathi ezi-2 nsuku zonke ngo-5mg isikhathi ngasinye.
I-Ruxolitinibiyi-JAK1 yomlomo yomlomo kanye ne-JAK2 tyrosine kinase inhibitor egunyazwe ku-European Union ngo-Agasti 2012 ukuze kwelashwe i-myelofibrosis eyingozi emaphakathi noma eyingozi, okuhlanganisa i-myelofibrosis eyinhloko, i-post-geniculocytosis myelofibrosis kanye ne-post-primary thrombocythemia myelofibrosis.Njengamanje, i-ruxolitinib Jakavi ivunyelwe emazweni angaphezu kuka-50 emhlabeni wonke, kuhlanganise ne-European Union, Canada kanye namazwe amaningana ase-Asia, Latin kanye naseNingizimu Melika.