Ua kapa ʻia ʻo Myelofibrosis (MF) he myelofibrosis.He mai laha loa.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 kulu ʻulaʻula.Hōʻike pinepine ʻia ka manaʻo maloʻo o ka iwi, a ua hoʻonui pinepine 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 ʻia, me ke kahe koko.Hiki ke hā'awi 'ia 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 ka liʻiliʻi 5 knm ma lalo o ka iwi iwi ʻaoʻao a ma kahi haʻahaʻa (2 prognostic factor) a i ʻole ke kiʻekiʻe kiʻekiʻe (3 a ʻoi aku ka prognostic factor) e like me ka International Working Group consensus criteria (IWG).
Hoʻokumu ʻia ka nui mua o ka ruxolitinib i ka helu platelet.15 mg ʻelua manawa 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 nā 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 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 mau manawa i kēlā me kēia lā ma 5mg i kēlā me kēia manawa.
ʻO RuxolitinibʻO ia ka JAK1 a me ka JAK2 tyrosine kinase inhibitor waha i ʻāpono ʻia i ka European Union i 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 ʻAsia, Latin a me ʻAmelika Hema.