Myelofibrosis

ʻO Myelofibrosis kahi maʻi o ka iwi iwi kahi e kuapo ʻia ka momona e ka fibrous scar tissue.

ʻO ka iwi iwi ka mea momona, momona ma loko o kou mau iwi. ʻO nā hunaola nā hunaola ʻole i loko o ka iwi iwi e hoʻomohala i kāu mau hunaola āpau. Hana ʻia kou koko i:

• Nā koko ʻulaʻula (e halihali i ka oxygen i kāu mauʻaʻaʻa)
• Nā keʻokeʻo koko keʻokeʻo (kahi e hakakā ai i ka maʻi)
• Nā Platelet (ka mea e kōkua i kou koko koko)

Ke wili ʻia ka iwi iwi, ʻaʻole hiki ke lawa i nā huna koko. ʻO ka anemia, nā pilikia kahe ʻana o ke kahe, a me kahi kiʻekiʻe o ka maʻi no nā maʻi.

ʻO ka hopena, hoʻāʻo ke akepaʻa a me ka iwi e hana i kekahi o kēia mau huna koko. ʻO kēia ke kumu e pehu ai kēia mau meaola.

ʻO ke kumu o ka myelofibrosis ʻike pinepine ʻole ʻia. ʻAʻohe mea ʻike pilikia e ʻike ʻia. Ke kū ʻia, ulu pinepine ia i ka poʻe ma mua o 50 mau makahiki. Hoʻohui like ʻia nā wahine a me nā kāne. Ua hoʻonui ʻia kahi hanana o kēia kūlana i nā Iudaio Ashkenazi.

ʻO nā maʻi ʻaʻa Koko a me nā iwi pū, e like me ka myelodysplastic syndrome, leukemia, a me ka lymphoma, e hōʻeha paha i ka iwi o ka iwi. Kapa ʻia kēia myelofibrosis lua.

Pākuʻi ʻia paha nā ʻōuli i kekahi o kēia mau mea:

• ʻO ka piha o ka ʻōpū, ʻeha, a i ʻole piha i ka manaʻo ma mua o ka pau ʻana o kahi pāʻina (ma muli o ka māhuahua hoʻonui)
• ʻEha iwi
• Kahe koko maʻalahi, ʻōpala
• Ka luhi
• Hoʻonui i ka likelika o ka loaʻa ʻana i kahi maʻi
• ʻIli pale
• Ka hanu ʻana me ka hoʻoikaika kino
• Kaumaha emi
• Hoʻoulu ka pō
• Hewa papa haʻahaʻa
• Ake akea
• Mū maloo
• ʻIli ʻili

E hana ka mea mālama ola i kahi hoʻokolohua kino a nīnau e pili ana i nā ʻōuli.

ʻO nā hoʻāʻo e hana ʻia ana paha:

• Helu piha ke koko (CBC) me ka hamo koko e nānā i nā ʻano kikoʻī o ke koko
• Ke ana ʻana i ka nāʻaʻa (ka pae enzyme LDH)
• Ke hoʻāʻo genetic
• ʻO ka biopsy iwi ʻōiwi e kuhi i ke ʻano a nānā i nā maʻi ʻōiwi iwi

Hiki i ka iwi o ka iwi a me ke koʻokoʻo kele ke hoʻomaikaʻi i nā ʻōuli, a hoʻōla paha i ka maʻi. Manaʻo pinepine ʻia kēia hana no ka poʻe ʻōpio.

Pili paha kekahi hana i nā hana ʻē aʻe:

• Hoʻopili ke koko a me nā lāʻau e hoʻoponopono i ka anemia
• ʻO ka uila a me ka chemotherapy
• Nā lāʻau i māka ʻia
• ʻO ka hemo ʻana o ka spleen (splenectomy) inā hoʻā ka ʻōhū i nā ʻōuli, a i ʻole e kōkua i ka anemia

Ke kaumaha nei ka maʻi, kū mālie ka iwi o ka iwi. ʻO ka helu platelet haʻahaʻa e hiki ai i ke kahe maʻalahi. Eʻoi aku ka maikaʻi o ka pehu Spleen me ka anemia.

ʻO ke ola ʻana o ka poʻe me myelofibrosis mua he 5 mau makahiki. Akā ke ola nei kekahi poʻe i mau kekeke.

Komo pū nā pilikia:

• Hoʻonui i ka leukemia myelogenous koʻikoʻi
• Nā maʻi ʻino
• Kahena
• Huʻi koko
• Hoka kōpaʻa

E hana i kahi manawa me kāu mea hāʻawi inā loaʻa iā ʻoe nā ʻōuli o kēia maʻi. E ʻimi koke i ka mālama olakino no ke kahe ʻole ʻole o ka kaohi, ka liʻiliʻi o ka hanu, a me ka jaundice (ʻili melemele a me nā keʻokeʻo o nā maka) e ʻino loa ana.

Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Myelofibrosis kumu; Myelofibrosis kekona; ʻO ka iwi iwi - myelofibrosis

ʻO Gotlib J. Polycythemia vera, thrombositosis pono, a me myelofibrosis kumu. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 157.

Lōʻihi NM, Kavanagh EC. Myelofibrosis. I: Pope TL, Bloem HL, Beltran J, Morrison WB, Wilson DJ, eds. Kiʻi Musculoskeletal. ʻElua ed. Piladelapia, PA: Elsevier Saunders; 2015: chap 76.

Mascarenhas J, Najfeld V, Kremyanskaya M, Keyzner A, Salama ME, Hoffman R. ʻOihana myelofibrosis. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 70.