He aha ka Myelofibrosis kumu?

Anter

• Nā ʻōuli myelofibrosis kumu
• Nā pae myelofibrosis kumu
• He aha ke kumu o ka myelofibrosis mua?
• Nā kumu pilikia no ka myelofibrosis kumu
• Nā koho lapaʻau myelofibrosis kumu
• Nā lāʻau e mālama ai i nā ʻōuli
• Mea kāohi iā JAK
• Mea hoʻoliʻiliʻi keleawe
• ʻO Chemotherapy a me radiation
• Hoʻopili ke koko
• ʻOki kino
• Nā hoʻokolohua hoʻokolohua o kēia manawa
• Hoʻololi ka nohona
• Outlook
• Lawe aku

ʻO ka myelofibrosis primer (MF) kahi maʻi ʻaʻai laha ʻole e kumu i ke kūkulu ʻia ʻana o nā ʻili maka, i ʻike ʻia ʻo fibrosis, i ka iwi iwi. Pale kēia i kou iwi iwi mai ka hana ʻana i kahi nui maʻamau o nā hunaola koko.

ʻO ke ʻano kumumākua MF kekahi ʻano o nā maʻi ʻaʻai koko. ʻO ia kekahi o ʻekolu mau ʻano neoplasms myeloproliferative (MPN) e hana ana i ka wā e mahele pinepine ai nā hunaola a i ʻole e make pinepine e like me ka mea e pono ai. Hoʻopili nā MPN ʻē aʻe i ka polycythemia vera a me ka thrombositosis pono.

Nānā nā kauka i kekahi mau kumuhana e hōʻike i ka MF mua. Loaʻa paha iā ʻoe kahi hoʻokolohua koko a me kahi biopsy o ka iwi iwi e kuhi iā MF.

Nā ʻōuli myelofibrosis kumu

ʻAʻole paha ʻoe e ʻike i kekahi ʻōuli no nā makahiki he nui. Hoʻomaka wale nā ​​ʻōuli e hoʻomaka mālie ma hope o ka scarring i ka iwi o ka iwi worsens a hoʻomaka e hoʻopilikia i ka hana ʻana o nā hunaola.

Hiki i nā ʻōuli myelofibrosis mua ke hoʻopili pū i:

• luhi
• pokole o ka hanu
• ʻili mae
• fiva
• nā maʻi pinepine
• paopao maʻalahi
• puhū pō
• nalo ʻai ʻole
• lilo kaumaha maopopo ʻole
• gums kahe
• kahe pinepine ka ihu
• piha a ʻeha paha i loko o ka ʻōpū ma ka ʻaoʻao hema (ma muli o kahi spleen hoʻonui ʻia)
• nā pilikia me ka hana ate
• ʻūlili
• ʻeha ami a iwi ʻeha paha
• gout

ʻO ka poʻe me MF maʻamau kahi pae haʻahaʻa o nā hunaola ʻulaʻula. Loaʻa paha iā lākou kahi helu o ke koko keʻokeʻo kiʻekiʻe loa a haʻahaʻa paha. E ʻike wale paha kāu kauka i kēia mau mea kūpono ʻole i ka manawa o ka nānā maʻamau ʻana ma hope o ka hana maʻamau o ka helu ʻana o ke koko.

Nā pae myelofibrosis kumu

ʻAʻole like me nā ʻano maʻi ʻaʻai ʻē aʻe, ʻaʻole i akaka ka wehewehe ʻia e ka MF mua. E hoʻohana paha kāu kauka i ka Dynamic International Prognostic Scoring System (DIPSS) e hoʻokaʻawale iā ʻoe i kahi hui haʻahaʻa, waena waena, a i ʻole pilikia kiʻekiʻe.

E noʻonoʻo lākou inā ʻo ʻoe:

• Loaʻa i kahi pae hemoglobin ma lalo o 10 gram i kēlā me kēia deciliter
• he helu keʻokeʻo koko keʻokeʻo i ʻoi aku ma mua o 25 × 10⁹ no lita
• ʻoi aku ma mua o 65 mau makahiki
• Loaʻa i nā pūnaewele puhi like a i ʻole emi ma mua o 1 pākēneka
• ʻike i nā ʻōuli e like me ka luhi, ka hou i ka pō, ka fiva, a me ka pohō kaumaha

Manaʻo ʻia ʻoe he haʻahaʻa haʻahaʻa inā ʻaʻole pili iā ʻoe kahi o nā mea i luna. ʻO ka hui ʻana i hoʻokahi a i ʻole ʻelua o kēia mau pae hoʻohālikelike e hoʻokau iā ʻoe i ka hui waena pilikia. ʻO ka hui ʻana i ʻekolu a ʻoi paha o kēia mau pae hoʻohālikelike e hoʻonoho iā ʻoe i ka pūʻulu kūlana kiʻekiʻe.

He aha ke kumu o ka myelofibrosis mua?

ʻAʻole maopopo ka poʻe noiʻi i nā kumu o MF. ʻAʻole hoʻoilina hoʻoilina ia. ʻO ia ka hiki ʻole iā ʻoe ke loaʻa i ka maʻi mai kou mau mākua a ʻaʻole hiki ke hoʻoili i kāu mau keiki, ʻoiai ʻo MF e holo i nā ʻohana. Hōʻike kekahi mau noiʻi e kumu ʻia paha e nā mutation gene i loaʻa e pili ana i nā ala hōʻailona o nā pūnaewele.

o ka poʻe me MF i kahi hoʻololi hoʻololi i kapa ʻia ʻo janus-pili kinase 2 (JAK2) pili i nā hunaula koko. ʻO ka JAK2 hana ka hoʻololi i kahi pilikia i ka hana ʻana o ka iwi iwi i nā ʻulaʻula o ke koko.

Hoʻokumu nā hunaola kūlohelohe ʻole i ka iwi iwi i nā hunaola makua oʻo e kope koke a lawe i ka iwi iwi. ʻO ke kūkulu ʻia ʻana o nā hunaola ke kumu o ka scarring a me ka mumū e pili ana i ka hiki ʻana o ka iwi o ka iwi e hana i nā kahe koko maʻamau. ʻO ka hopena maʻamau ka liʻiliʻi ma mua o nā hunaola koko ʻulaʻula a nui a me nā hunaola keokeo.

Ua hoʻopili ka poʻe noiʻi iā MF i nā hoʻololi kūʻē ʻē aʻe. Ma kahi o 5 a 10 pakeneka o ka poʻe me MF i loaʻa kahi MPL hoʻololi kūʻē. Ma kahi o 23.5 pākēneka he genation mutation i kapa ʻia calreticulin (CALR).

Nā kumu pilikia no ka myelofibrosis kumu

Kākaʻikahi loa ʻo Primary MF. Kū wale ia ma kahi o 1.5 wale nō i kēlā me kēia 100,000 poʻe i ʻAmelika Hui Pū ʻIa. Hiki i ka maʻi ke hoʻopili i nā kāne a me nā wahine.

Hiki i kekahi mau mea ke hoʻonui i ka makaʻu o ke kanaka i ka loaʻa ʻana o ka MF mua, e like me:

• ʻoi aku ma mua o 60 mau makahiki
• ka hōʻike ʻana i nā petrochemicals e like me benzene a me toluene
• ka hōʻike ʻana i ka radiation ionizing
• loaʻa ka JAK2 hoʻololi kūʻē

Nā koho lapaʻau myelofibrosis kumu

Inā ʻaʻohe ou hōʻailona MF, ʻaʻole kau kauka e hoʻokau iā ʻoe i nā ʻano lāʻau a ma kahi e nānā pono iā ʻoe me ka nānā maʻamau. Ke hoʻomaka koke nā ʻōuli, mālama ka mālama e mālama i nā hōʻailona a hoʻomaikaʻi i kou ola.

ʻO nā koho lapaʻau myelofibrosis mua me nā lāʻau, chemotherapy, radiation, transplants cell stem, transfusions of blood, and surgery.

Nā lāʻau e mālama ai i nā ʻōuli

Hiki i kekahi mau lāʻau ke kōkua i ka mālama ʻana i nā hōʻailona e like me ka luhi a me ke kāwili ʻana.

Hiki i kāu kauka ke koi aku i ka aspirin haʻahaʻa a i ʻole ka hydroxyurea e hōʻemi i ka makaʻu o ka thrombosis venous hohonu (DVT).

ʻO nā lāʻau e mālama ai i ka helu o ke koko ʻulaʻula haʻahaʻa (anemia) i hoʻopili ʻia iā MF e komo pū ana:

• androgen therapy
• steroid, e like me prednisone
• thalidomide (Thalomid)
• lenalidomide (Revlimid)
• nā mea hoʻonāukiuki erythropoiesis (ESAs)

Mea kāohi iā JAK

Mālama nā mea pāpā ʻo JAK i nā hōʻailona MF ma ka pale ʻana i ka hana o ka JAK2 ʻāpana a me ka protein JAK1. ʻO Ruxolitinib (Jakafi) a me fedratinib (Inrebic) nā lāʻau ʻelua i ʻae ʻia e ka Food and Drug Administration (FDA) e mālama i ka makaʻu waena a i ʻole ka pilikia ʻo MF. Ke hoʻāʻo ʻia nei kekahi mau mea hoʻopaʻa ʻo JAK i kēia manawa i nā hoʻokolohua hoʻokolohua.

Ua hōʻike ʻia ʻo Ruxolitinib e hōʻemi i ka hoʻonui ākea a hoʻemi i kekahi mau hōʻailona pili i ka MF, e like me ka hōʻoluʻolu o ka ʻōpū, ʻehaʻeha o ka iwi, a me ka ʻeha. Hoʻoemi ia i nā kiʻekiʻe o nā cytokine pro-inflammatory i ke koko. Hiki paha i kēia ke kōkua i ka hōʻoluʻolu i nā hōʻailona MF me ka luhi, ke kuni, ka hou o ka pō, a me ka pohō kaumaha.

Hāʻawi pinepine ʻia ʻo Fedratinib ke hana ʻole ʻo ruxolitinib. He mea paʻa ikaika loa ʻo JAK2 mea kāohi. Hāpai ia i kahi wahi liʻiliʻi o ka hōʻeha lolo a weliweli paha i ʻike ʻia ʻo encephalopathy.

Mea hoʻoliʻiliʻi keleawe

ʻO kahi transplant cell allogeneic stem cell (ASCT) ka mea ola maoli wale nō no MF. ʻIke ʻia kekahi transplant o ka iwi iwi, pili ia i ka loaʻa ʻana o kahi infusion o nā hunaola kūpuna mai kahi mea hāʻawi maikaʻi. Hoʻololi kēia mau pūnaewele kūmole olakino i nā hunaola kīnā ʻole.

ʻO ke kaʻina hana he pilikia nui i nā hopena ʻino e hoʻoweliweli nei i ke ola. E nānā pono ʻia ʻoe ma mua o ka hoʻohālikelike ʻia me kahi mea hāʻawi. Nānā wale ʻia ʻo ASCT no nā poʻe me ka makaʻu waena a i ʻole pilikia kiʻekiʻe ʻo MF ma lalo o ka makahiki 70.

ʻO Chemotherapy a me radiation

Hiki i nā lāʻau Chemotherapy me hydroxyurea ke kōkua i ka hoʻēmi ʻana i kahi spleen hoʻonui ʻia e pili iā MF. Hoʻohana ʻia kekahi kukuna uila i kekahi manawa ke ʻaʻole lawa nā mea kūpale ʻo JAK a me ka chemotherapy e hoʻēmi ai i ka nui o ka spleen.

Hoʻopili ke koko

Hiki ke hoʻohana ʻia kahi transfusion o ke koko ʻulaʻula olakino e hoʻonui i ka helu o ke kaola ʻulaʻula a mālama i ka anemia.

ʻOki kino

Inā hoʻonui ʻia kahi ʻōpala i nā ʻōuli koʻikoʻi, hiki i kāu kauka ke koi aku i kekahi manawa i ka lawe ʻana i ka ʻōpala. ʻIke ʻia kēia hana ma ke ʻano he splenectomy.

Nā hoʻokolohua hoʻokolohua o kēia manawa

Ke nānā ʻia nei he mau lāʻau lapaʻau he nui no ka mālama ʻana i ka myelofibrosis mua. Hoʻopili kēia i nā lāʻau ʻē aʻe e kāohi iā JAK2.

Mālama ka MPN Research Foundation i kahi papa inoa o nā hoʻokolohua hoʻokolohua no MF. Ua hoʻomaka kekahi o kēia mau hoʻāʻo e hoʻāʻo. ʻO nā mea ʻē aʻe e ʻohi nei i nā mea maʻi. ʻO ka hoʻoholo e komo i kahi hoʻokolohua hoʻokolohua pono e hana pono ʻia me kāu kauka a me kou ʻohana.

Hele nā ​​lāʻau i loko o nā hanana ʻehā o nā hoʻokolohua hoʻokolohua ma mua o ka ʻae ʻia ʻana e ka FDA. Aia wale kekahi mau lāʻau hou i kēia manawa i ka pae III o nā hoʻokolohua hoʻokolohua, e like me pacritinib a me momelotinib.

Hōʻike nā ho'āʻo hoʻokolohua Phase I a me II e hiki i ka everolimus (RAD001) ke kōkua i ka hōʻemi ʻana i nā hōʻailona a me ka nui o ka spleen i ka poʻe me MF. Hoʻopili kēia lāʻau i kahi ala i nā hunaʻi hana koko e hiki ai ke alakaʻi i ka ulu ulu maʻamau i MF.

Hoʻololi ka nohona

E kaumaha paha ʻoe i ka naʻau ma hope o ka loaʻa ʻana o kahi diagnosis MF mua, ʻoiai inā ʻaʻohe ou mau ʻōuli. He mea nui e noi no ke kākoʻo mai ka ʻohana a me nā hoaaloha.

Hiki i ka hui ʻana me kahi kahu a i ʻole limahana kaiaulu ke hāʻawi iā ʻoe i ka ʻike nui e pili ana i ka hopena o ka hopena o ka maʻi ʻaʻai i kou ola. E makemake paha ʻoe i kāu kauka e pili ana i ka hana ʻana me kahi ʻoihana laikini noʻonoʻo laikini.

Hiki i nā loli nohona ʻē aʻe ke kōkua iā ʻoe e hoʻokele i ke koʻikoʻi. ʻO ka noʻonoʻo ʻana, ʻo yoga, nā hele wāwae kūlohelohe, a i ʻole ke hoʻolohe ʻana i nā mele hiki ke kōkua i ka hoʻoikaika ʻana i kou ʻano a me ka nohona holoʻokoʻa.

Outlook

ʻAʻole hiki i ka MF MF ke hana i nā ʻōuli i kona wā mua a hiki ke mālama ʻia me nā ʻano lāʻau like ʻole. Ke paʻakikī nei i ka wanana a me ke ola ʻana no MF. ʻAʻole holomua ka maʻi no kekahi manawa lōʻihi i kekahi poʻe.

Aia ke kuhi o ke ola ma muli o ke kanaka ma kahi haʻahaʻa, waena, a i ʻole kahi kūlana kiʻekiʻe. Hōʻike kekahi mau noiʻi i kēlā mau mea i ka hui haʻahaʻa haʻahaʻa i loaʻa nā helu ola like no nā makahiki 5 mua ma hope o ka ʻike ʻia ʻana ma ke ʻano he heluna lehulehu, a ma ia manawa hoʻomaka ka emi ʻana o nā helu ola. Ua ola nā poʻe i ka pūʻulu kūpilikiʻi kiʻekiʻe a hiki i 7 mau makahiki.

Hiki iā MF ke alakaʻi i nā pilikia koʻikoʻi i ka manawa. Holomua ʻo Primary MF i kahi maʻi ʻoi loa a paʻakikī hoʻi e mālama i ka maʻi ʻaʻai koko i ʻike ʻia ma ke ʻano he myeloid leukemia (AML) ma kahi o 15 a 20 pākēneka o nā hihia.

ʻO ka hapa nui o nā lapaʻau ʻana no ka MF mua e kau i ka mālama ʻana i nā pilikia pili iā MF. Hoʻopili kēia i ka anemia, nā spleen hoʻonui ʻia, nā ʻōpala o ke kō ʻana o ke koko, nā keu keokeo a i ʻole nā ​​platelet, a me nā helu platelet haʻahaʻa. Mālama hoʻi nā mālama i nā ʻōuli e like me ka luhi, sweats i ka pō, ʻili ʻili, fiva, ʻeha pū, a me ka gout.

Lawe aku

ʻO ka MF MF kahi maʻi ʻaʻai laha ʻole e hoʻopili i kou mau hunaola koko. ʻAʻole ʻike ka poʻe he nui i nā ʻōuli ma mua a hiki i ka holomua ʻana o ka maʻi ʻaʻai. ʻO ka mea hiki ke hoʻōla ʻia no ka MF mua kahi transplant cell stem, akā aia kekahi mau ʻano lapaʻau ʻē aʻe a me nā hoʻokolohua hoʻokolohua e mālama ʻia nei e hoʻomaʻamaʻa i nā hōʻailona a hoʻomaikaʻi i kou ola.