Hoʻokumu ʻia ka hoʻolālā lapaʻau no ka myelofibrosis mua (PMF) i ka stratification pilikia.Ma muli o ke ʻano o nā hōʻike lapaʻau a me nā pilikia e hoʻoponopono ʻia i nā poʻe maʻi PMF, pono nā hoʻolālā lapaʻau e noʻonoʻo i ka maʻi o ka mea maʻi a me nā pono lapaʻau.ʻO ka hoʻomaʻamaʻa mua ʻana me ruxolitinib (Jakavi/Jakafi) i nā poʻe maʻi me ka spleen nui i hōʻike i ka hoʻemi nui ʻana o ka spleen a kūʻokoʻa i ke kūlana mutation o ka mea hoʻokele.ʻO ka nui o ka hōʻemi ʻana i ka spleen e hōʻike ana i kahi prognosis maikaʻi aʻe.I nā maʻi haʻahaʻa haʻahaʻa me ka maʻi nui ʻole, hiki ke nānā ʻia a komo i loko o nā hoʻokolohua lapaʻau, me nā loiloi hou i kēlā me kēia 3-6 mahina.ʻO Ruxolitinib(Jakavi/Jakafi) hiki ke hoʻomaka i ka lāʻau lapaʻau i nā maʻi haʻahaʻa a waena paha-1 i loaʻa me ka splenomegaly a / a i ʻole ka maʻi maʻi, e like me nā alakaʻi lapaʻau NCCN.
No nā maʻi waena-pilikia-2 a i ʻole nā ​​​​mea maʻi kiʻekiʻe, makemake ʻia ka allogeneic HSCT.Inā ʻaʻole i loaʻa ka hoʻololi ʻana, ʻōlelo ʻia ʻo ruxolitinib (Jakavi/Jakafi) ma ke ʻano he koho lapaʻau mua a i ʻole e komo i nā hoʻokolohua lapaʻau.ʻO Ruxolitinib (Jakavi/Jakafi) wale nō ka lāʻau lapaʻau i ʻae ʻia i ka honua holoʻokoʻa e kuhikuhi ana i ke ala JAK / STAT overactive, ka pathogenesis o MF.ʻElua mau haʻawina i paʻi ʻia ma ka New England Journal a me ka Journal of Leukemia & Lymphoma e hōʻike ana e hiki i ka ruxolitinib (Jakavi/Jakafi) ke hōʻemi nui i ka maʻi a hoʻomaikaʻi i ka maikaʻi o ke ola i nā maʻi me PMF.Ma waena o ka pilikia-2 a me ka nui-pilikia MF maʻi, ruxolitinib (Jakavi / Jakafi) hiki ke emi i ka spleen, hoʻomaikaʻi i ka maʻi, hoʻomaikaʻi i ke ola, a me ka hoʻomaikaʻi 'ana i ka iwi o ka iwi pathology, halawai me na pahu hopu mua o ka maʻi hooponopono.
He 0.5-1.5/100,000 ka PMF i ka makahiki a loaʻa ka prognosis maikaʻi loa o nā MPN āpau.Hōʻike ʻia ka PMF e ka myelofibrosis a me extramedullary hematopoiesis.I loko o ka PMF, ʻaʻole i loaʻa mai nā fibroblast o ka iwi iwi mai nā clones maʻamau.Ma kahi o ka hapakolu o nā maʻi me ka PMF ʻaʻohe hōʻailona i ka manawa o ka maʻi.ʻO nā hoʻopiʻi e pili ana i ka luhi nui, ka anemia, ka ʻeha o ka ʻōpū, ka maʻi maʻi ma muli o ka māʻona mua a i ʻole ka splenomegaly, ke koko, ka pohō kaumaha, a me ka edema peripheral.ʻO Ruxolitinib(Jakavi/Jakafi) ua ʻae ʻia i ʻAukake 2012 no ka mālama ʻana i ka myelofibrosis waena a kiʻekiʻe paha, me ka myelofibrosis mua.Loaʻa ka lāʻau lapaʻau i kēia manawa ma mua o 50 mau ʻāina a puni ka honua.