Myelofibrosis (MF) e bitsoa myelofibrosis. Hape ke lefu le sa tloaelehang haholo. 'Me sesosa sa pathogenesis ea eona ha e tsejoe. Matšoao a tloaelehileng a kliniki ke lisele tse khubelu tsa mali tsa bana le bana ba granulocytic anemia e nang le palo e phahameng ea lisele tse khubelu tsa mali. Hangata 'mele oa masapo o bontša takatso e omileng,' me hangata spleen e atolosoa ka mefuta e sa tšoaneng ea lefu la ho fokola ha masapo.
Primary myelofibrosis (PMF) ke lefu la clonal myeloproliferative (MPD) la lisele tsa stem tsa hematopoietic. Kalafo ea myelofibrosis e ka sehloohong e tšehetsa haholo-holo, ho akarelletsa le tšelo ea mali. Hydroxyurea e ka fanoa bakeng sa thrombocytosis. Bakuli ba kotsing e tlase, ba se nang matšoao ba ka bonoa ntle le kalafo.
Lithuto tse peli tsa mekhahlelo ea boraro (STUDY1 le 2) li ile tsa etsoa ho bakuli ba nang le MF (primary MF, post-geniculocytosis MF, kapa post-primary thrombocythemia MF). Lithutong tsena ka bobeli, bakuli ba ngolisitsoeng ba ne ba e-na le splenomegaly e hlakileng bonyane 5 cm ka tlas'a likhopo 'me ba ne ba le boemong bo itekanetseng (2 lintlha tsa prognostic) kapa kotsi e kholo (3 kapa ho feta lintlha tsa prognostic) ho latela International Working Group consensus criteria (IWG).
Tekanyetso ea pele ea ruxolitinib e ipapisitse le lipalo tsa platelet. 15 mg habeli ka letsatsi bakeng sa bakuli ba nang le palo ea liplatelete pakeng tsa 100 le 200 x 10 ^ 9/L le 20 mg habeli ka letsatsi bakeng sa bakuli ba nang le lipalo tse fetang 200 x 10 ^ 9/L.
Litekanyetso tsa motho ka mong li ile tsa fanoa ho latela mamello le katleho ho bakuli ba nang le lipalo tsa platelet pakeng tsa 100 le 125 x 10 ^ 9 / L, ka tekanyo e kholo ea 20 mg habeli ka letsatsi; bakeng sa bakuli ba nang le lipalo tsa platelet pakeng tsa 75 le 100 x 10 ^ 9 / L, 10 mg habeli ka letsatsi; le bakeng sa bakuli ba nang le lipalo tsa platelet pakeng tsa 50 le ka tlase ho kapa ho lekana le 75 x 10 ^ 9 / L, makhetlo a 2 ka letsatsi ho 5mg nako le nako.
Ruxolitinibke molomo oa JAK1 le JAK2 tyrosine kinase inhibitor e amohelehang ho European Union ka August 2012 bakeng sa phekolo ea myelofibrosis e ka hare kapa e kotsi haholo, ho akarelletsa le myelofibrosis ea mantlha, post-geniculocytosis myelofibrosis le post-primary thrombocythemia myelofibrosis. Hajoale, ruxolitinib Jakavi e amoheloa linaheng tse fetang 50 lefatšeng ka bophara, ho kenyeletsoa European Union, Canada le linaha tse 'maloa tsa Asia, Latin le Amerika Boroa.