Ua kapa ʻia ʻo Myelofibrosis (MF) he myelofibrosis. He mai laha loa ia. A ʻaʻole ʻike ʻia ke kumu o kona pathogenesis. ʻO nā hōʻike maʻamau o ke koko ʻulaʻula ʻōpiopio a me ka anemia granulocytic juvenile me ka helu kiʻekiʻe o ka waimaka ʻulaʻula ʻulaʻula. Hōʻike pinepine ʻia ka manaʻo maloʻo o ka iwi, a ua hoʻonui nui ʻia ka spleen me nā pae like ʻole o ka osteosclerosis.
ʻO ka myelofibrosis mua (PMF) kahi maʻi myeloproliferative clonal (MPD) o nā pūnaewele hematopoietic stem. ʻO ka mālama ʻana i ka myelofibrosis mua ke kākoʻo nui, me ka hoʻokaʻawale koko. Hiki ke hāʻawi ʻia ka Hydroxyurea no ka thrombocytosis. Hiki ke nānā ʻia nā maʻi haʻahaʻa haʻahaʻa, asymptomatic me ka ʻole o ka mālama ʻana.
Ua hana ʻia ʻelua mau haʻawina ʻo III (STUDY1 a me 2) i nā maʻi me MF (MF mua, post-geniculocytosis MF, a i ʻole thrombocythemia MF post-primary). Ma nā haʻawina ʻelua, ua loaʻa i nā maʻi i kākau inoa ʻia ka splenomegaly palpable ma kahi o 5 knm ma lalo o ka iwi iwi ʻaoʻao a ma ke ʻano haʻahaʻa (2 prognostic factor) a i ʻole ke kūlana kiʻekiʻe (3 a ʻoi aku ka prognostic factor) e like me ka International Working Group consensus criteria (IWG).
Hoʻokumu ʻia ka helu mua o ka ruxolitinib ma ka helu platelet. 15 mg ʻelua i kēlā me kēia lā no nā mea maʻi me ka helu platelet ma waena o 100 a me 200 x 10^9/L a me 20 mg ʻelua i kēlā me kēia lā no nā mea maʻi me ka helu platelet ʻoi aku ma mua o 200 x 10^9/L.
Hāʻawi ʻia nā ʻāpana pākahi e like me ka tolerability a me ka pono no nā maʻi me ka helu platelet ma waena o 100 a me 125 x 10^9/L, me ka nui o ka nui o 20 mg ʻelua i kēlā me kēia lā; no nā poʻe maʻi me ka helu platelet ma waena o 75 a me 100 x 10^9/L, 10 mg ʻelua i kēlā me kēia lā; a no nā poʻe maʻi me ka helu platelet ma waena o 50 a emi iho a i ʻole like me 75 x 10^9/L, 2 manawa i kēlā me kēia lā ma 5mg i kēlā me kēia manawa.
ʻO RuxolitinibHe mea hoʻopaneʻe waha JAK1 a me JAK2 tyrosine kinase i ʻāpono ʻia ma ka European Union ma ʻAukake 2012 no ka mālama ʻana i ka myelofibrosis waena a i ʻole kiʻekiʻe, me ka myelofibrosis mua, post-geniculocytosis myelofibrosis a me post-primary thrombocythemia myelofibrosis. I kēia manawa, ua ʻae ʻia ʻo ruxolitinib Jakavi ma mua o 50 mau ʻāina ma ka honua holoʻokoʻa, me ka European Union, Canada a me kekahi mau ʻāina ʻĀsia, Latin a me ʻAmelika Hema.