I-Myelofibrosis (MF) ibizwa ngokuba yi-myelofibrosis.Kwakhona sisifo esinqabileyo kakhulu.Kwaye unobangela we-pathogenesis yayo awaziwa.Iimpawu zeklinikhi eziqhelekileyo ziseligazi elibomvu lolutsha kunye ne-granulocytic anemia yolutsha enenani eliphezulu leeseli ezibomvu zegazi.I-Bone marrow aspiration idla ngokubonisa i-aspiration eyomileyo, kwaye i-spleen ihlala yandiswa ngokuphawulekayo ngamaqondo ahlukeneyo e-osteosclerosis.
I-Primary myelofibrosis (PMF) yi-clonal myeloproliferative disorder (MPD) yeeseli ze-hematopoietic stem.Unyango lwe-primary myelofibrosis luxhasa ngokuyintloko, kubandakanywa utofelo-gazi.I-Hydroxyurea inokunikwa i-thrombocytosis.Umngcipheko ophantsi, izigulane ezingenazimpawu zingabonwa ngaphandle kwonyango.
Izifundo ezimbini zesigaba se-III (STUDY1 kunye ne-2) zenziwa kwizigulane ezine-MF (i-MF esisiseko, i-post-geniculocytosis MF, okanye i-post-primary thrombocythemia MF).Kuzo zombini izifundo, izigulane ezibhalisiweyo zine-splenomegaly ecacileyo ubuncinane i-5 cm ngaphantsi kwe-rib cage kwaye yayiphakathi (i-2 prognostic factor) okanye umngcipheko ophezulu (i-3 okanye i-prognostic factor more) ngokwe-International Working Group consensus criteria (IWG).
I-dose yokuqala ye-ruxolitinib isekelwe kwizibalo zeplatelet.I-15 mg kabini imihla ngemihla kwizigulane ezine-platelet counts phakathi kwe-100 kunye ne-200 x 10 ^ 9 / L kunye ne-20 mg kabini imihla ngemihla kwizigulane ezine-platelet counts ezingaphezu kwe-200 x 10 ^ 9 / L.
Amanani omntu ngamnye anikwe ngokunyamezela kunye nokusebenza kakuhle kwizigulane ezineeplatelet counts phakathi kwe-100 kunye ne-125 x 10 ^ 9 / L, kunye ne-dose ephezulu ye-20 mg kabini imihla ngemihla;kwizigulane ezine-platelet counts phakathi kwe-75 kunye ne-100 x 10 ^ 9 / L, i-10 mg kabini imihla ngemihla;kunye nezigulane ezine-platelet counts phakathi kwe-50 kwaye ngaphantsi okanye ilingana ne-75 x 10 ^ 9 / L, amaxesha e-2 imihla ngemihla kwi-5mg ngexesha ngalinye.
Ruxolitinibi-JAK1 yomlomo kunye ne-JAK2 i-tyrosine kinase inhibitor evunyiweyo kwi-European Union ngo-Agasti 2012 yonyango lwe-myelofibrosis ephakathi okanye ephezulu, kubandakanywa i-myelofibrosis yokuqala, i-post-geniculocytosis myelofibrosis kunye ne-post-primary thrombocythemia myelofibrosis.Okwangoku, i-ruxolitinib Jakavi ivunyiwe kumazwe angaphezu kwama-50 emhlabeni jikelele, kuquka i-European Union, iKhanada kunye namazwe amaninzi ase-Asia, eLatini kunye naseMzantsi Melika.