Myelofibrosis (MF) yog hu ua myelofibrosis.Nws kuj yog ib yam kab mob tsawg heev.Thiab qhov ua rau ntawm nws pathogenesis tsis paub.Cov tsos mob tshwm sim yog cov qe ntshav liab me me thiab cov menyuam yaus granulocytic anemia nrog ntau cov kua muag poob cov qe ntshav liab.Cov pob txha pob txha aspiration feem ntau qhia tau hais tias qhuav aspiration, thiab tus po yog feem ntau markedly loj nrog ntau qib ntawm osteosclerosis.
Primary myelofibrosis (PMF) yog clonal myeloproliferative disorder (MPD) ntawm hematopoietic qia hlwb.Kev kho mob ntawm thawj myelofibrosis feem ntau txhawb nqa, suav nrog kev hloov ntshav.Hydroxyurea tuaj yeem muab rau thrombocytosis.Kev pheej hmoo tsawg, cov neeg mob asymptomatic tuaj yeem pom yam tsis muaj kev kho mob.
Ob qhov kev tshawb fawb randomized theem III (STUDY1 thiab 2) tau ua rau cov neeg mob uas muaj MF (primary MF, post-geniculocytosis MF, lossis post-primary thrombocythemia MF).Hauv ob qho tib si kev tshawb fawb, cov neeg mob cuv npe muaj palpable splenomegaly tsawg kawg yog 5 cm hauv qab lub tawb tawb thiab nyob nruab nrab (2 prognostic yam) los yog muaj kev pheej hmoo siab (3 los yog ntau dua prognostic yam) raws li International Working Group pom zoo (IWG).
Thawj koob tshuaj ntawm ruxolitinib yog raws li kev suav platelet.15 mg ob zaug ib hnub rau cov neeg mob platelet suav ntawm 100 thiab 200 x 10^9 / L thiab 20 mg ob zaug ib hnub rau cov neeg mob platelet suav ntau dua 200 x 10^9 / L.
Cov koob tshuaj ntawm tus kheej tau muab raws li kev zam thiab kev ua tau zoo rau cov neeg mob platelet suav ntawm 100 thiab 125 x 10^9 / L, nrog rau qhov siab tshaj plaws ntawm 20 mg ob zaug ib hnub;rau cov neeg mob platelet suav ntawm 75 thiab 100 x 10^9 / L, 10 mg ob zaug ib hnub;thiab rau cov neeg mob platelet suav ntawm 50 thiab tsawg dua lossis sib npaug li 75 x 10^9 / L, 2 zaug hauv ib hnub ntawm 5mg txhua zaus.
Ruxolitinibyog qhov ncauj JAK1 thiab JAK2 tyrosine kinase inhibitor tau pom zoo nyob rau hauv European Union thaum Lub Yim Hli 2012 rau kev kho mob ntawm nruab nrab lossis muaj kev pheej hmoo siab myelofibrosis, suav nrog thawj myelofibrosis, post-geniculocytosis myelofibrosis thiab post-primary thrombocythemia myelofibrosis.Tam sim no, ruxolitinib Jakavi tau pom zoo nyob hauv ntau dua 50 lub teb chaws thoob ntiaj teb, suav nrog European Union, Canada thiab ntau lub tebchaws Esxias, Latin thiab South American.