ʻO ka hoʻolālā lapaʻau no ka myelofibrosis mua (PMF) e pili ana i ka stratification pilikia. Ma muli o ke ʻano o nā hōʻike lapaʻau a me nā pilikia e hoʻoponopono ʻia i nā 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 mea maʻi me kahi spleen nui i hōʻike i ka hōʻemi nui o ka spleen a kūʻokoʻa i ke kūlana hoʻololi o ka mea hoʻokele. ʻO ka nui o ka hōʻemi ʻana i ka spleen e hōʻike ana i kahi prognosis maikaʻi aʻe. I nā poʻe maʻi 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 poʻe maʻi waena-pilikia-2 a i ʻole nā ​​​​mea maʻi kiʻekiʻe, makemake ʻia ka allogeneic HSCT. Inā ʻaʻole hiki ke hoʻololi ʻia, 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 ʻāpono 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 ke kiʻekiʻe-pilikia MF maʻi, ruxolitinib (Jakavi / Jakafi) hiki ke emi i ka spleen, hoʻomaikaʻi i ka maʻi, hoʻomaikaʻi i ke ola, a hoʻomaikaʻi i ka iwi o ka iwi pathology, e hālāwai me nā pahuhopu mua o ka hoʻoponopono maʻi.
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 fibroblasts o ka 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 poho 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.