Ke luhi mau nei ʻoe a me ka luhi ʻole me ke kumu ʻole? Loaʻa iā ʻoe nā palapū ma kou kino a pau? Ke kaumaha iki nei ʻoe a i ʻole ka manaʻo o ka piha ma ka ʻaoʻao hema o kou ʻōpū? Aia paha kekahi kumu koʻikoʻi ma hope o kēia mau mea a mākou i noʻonoʻo ʻole ai. He ʻano ia i kapa ʻia ʻo myelofibrosis. Mai makaʻu ke lohe ʻoe i kēia inoa. E kamaʻilio mākou e pili ana i ke ʻano o ia mea, no ke aha e hana ʻia ai, he aha nā hōʻailona, a he aha nā lāʻau lapaʻau ma ke ʻano maʻalahi loa e hiki ai iā ʻoe ke hoʻomaopopo.
I ka ʻōlelo maʻalahi, he aha ka myelofibrosis?
E noʻonoʻo i nā iwi nui i loko o ko kākou kino e like me nā hale hana koko. Kāhea mākou i kēia mau hale hana he iwi iwi . Aia i loko o kēia iwi iwi e hana ʻia ai nā ʻano hunaola koko ʻekolu e pono ai ko kākou kino.
1. Nā Kelepona Koko ʻUlaʻula: Lawe i ka oxygen ma ke kino holoʻokoʻa.
2. Nā Kelepona Koko Keʻokeʻo: Ke kaua nei i nā maʻi a me nā germs.
3. Nā Platelets: Hoʻokumu lākou i nā ʻūpā koko a hoʻōki i ke kahe ʻana o ke koko.
He ʻano maʻi koko laha ʻole ka Myelofibrosis kahi e piha mālie ai ka iwi iwi, ka hale hana hana koko, me nā ʻiʻo ʻeha. E like me ka noho mālie ʻana o nā nāhelehele ma luna o kahi mahina momona, pale kēia ʻiʻo ʻeha i ka iwi iwi mai ka hana ʻana i nā hunaola koko ma ke ʻano maʻamau.
No ka mea, ʻaʻole i hana pono ʻia kēia hana, hoʻāʻo ko kākou spleen , ʻo ia hoʻi ke kino ma ka ʻaoʻao hema o luna o ka ʻōpū, e kōkua i kēia hana. ʻO ia hoʻi, hoʻomaka ka spleen e hana i ke koko. Akā, ʻaʻole ia kāna hana nui. Ma muli o kēia, ua nui loa ka spleen, a hoʻomaka mālie ia e nui a pehu. Hoʻomohala ka nui o nā maʻi i nā hōʻailona me kēia pehu o ka spleen.
He maʻi kēia no ke ola holoʻokoʻa, e holomua mālie ana. Eia nō naʻe, i kekahi manawa hiki ke lilo i mea ʻino koke, a i kekahi poʻe hiki ke holomua i ka leukemia koʻikoʻi. ʻO ia ke kumu e nānā pono ai kāu kauka iā ʻoe ma hope o kou ʻike ʻia ʻana.
Aia kekahi mau ʻano nui o ka myelofibrosis?
ʻAe, ʻelua mau ʻano nui o kēia.
- ʻO ka Myelofibrosis Mua: ʻO kēia ke ʻano maʻamau. Hiki koke mai ia, me ka ʻole o ka mana o kekahi maʻi ʻē aʻe.
- ʻO ka Myelofibrosis Lua:Hana ʻia kēia ke lilo nā maʻi ʻē aʻe e pili ana i ke koko i mea koʻikoʻi. No ka laʻana, hiki i kekahi me ka Essential Thrombocythemia a i ʻole Polycythemia Vera ke loaʻa i ka myelofibrosis i ka hala ʻana o ka manawa. No loko o kahi hui o nā maʻi ʻaʻai koko i kapa ʻia ʻo Myeloproliferative Neoplasms (MPNs) kēia mau maʻi a pau. I ka ʻōlelo maʻalahi, ʻo kēia mau maʻi a pau ke kumu o ka hana ʻana o ka iwi iwi i hoʻokahi a ʻoi aku paha nā ʻano o nā ʻano koko ma mua o ka mea e pono ai.
He aha nā hōʻailona o kēia maʻi? Pehea mākou e ʻike ai?
I ka hapa nui o ka manawa, lohi loa ka ulu ʻana o kēia maʻi. No laila, hiki ke hoʻomau no nā makahiki me ka ʻole o ka hōʻike ʻana i nā hōʻailona. Ke hoʻomaka ka ʻike ʻia ʻana o nā hōʻailona, ʻo ka mea mua e hiki mai ana i ka noʻonoʻo, ʻo ia ka luhi nui . ʻO kēia ma muli o ka nele o nā hunaola koko ʻulaʻula i loko o ke kino, ʻo ia hoʻi ka anemia. Me kēia, ma muli o ka spleen i hoʻonui ʻia, hiki iā ʻoe ke manaʻo i ke kaumaha, ka ʻeha, a i ʻole ka manaʻo o ka piha ma ka ʻaoʻao hema o luna o ka ʻōpū.
E nānā kākou i nā hōʻailona ʻē aʻe ma ka papa ma lalo nei.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| ʻEha iwi a me nā hono | ʻEha e puka mai ana mai loko mai o nā iwi ma muli o nā loli i loko o ka iwi. |
| ʻEha maʻalahi a kahe koko paha | Ma muli o ka emi ʻana o nā platelets, hiki i kahi puʻupuʻu liʻiliʻi ke hoʻoulu i ka ʻeha a me ke kahe ʻana o ke koko. |
| Ake nui | E like me ka spleen, hiki i ke akepaʻa ke pehu i kona hoʻāʻo ʻana e hana i ke koko. |
| kuni | Wela me ke kumu ʻole. |
| Nā maʻi pinepine | Hoʻolaha maʻalahi nā maʻi no ka mea ua emi nā hunaola koko keʻokeʻo o ke kino, ka mea e hana ma ke ʻano he mau hunaola pale o kona kino. |
| Manaʻo piha ʻoiai ma hope o ka ʻai iki ʻana | No ka mea, ua pehu ka ʻōpū a ua ʻōhū ka ʻōpū, ʻoiai ma hope o ka ʻai ʻana i kahi laiki liʻiliʻi, ua piha ka ʻōpū. |
| ʻIli ʻāʻī | Hiki ke loaʻa ka maneʻo nui ma ke kino holoʻokoʻa. |
| Ka hou nui ʻana i ka pō | E hou nui ana i ka pō a pulu nā moena moe. |
| ʻO ka pohō kaumaha me ke kumu ʻole | Inā ʻoe e lilo i ke kaumaha me ka hoʻāʻo ʻole. |
No ke aha e hana ʻia ai kēia? He aha nā kumu?
ʻAʻole maopopo i nā kauka ke kumu pololei o ka myelofibrosis mua, akā ua maopopo iā lākou ke ʻano o ka hana ʻana.
Hoʻomaka ia me kahi mutation i loko o kahi gene i loko o ke kelepona kumu, ʻo ia ke kelepona hoʻokumu koko mua i loko o ka iwi iwi . ʻO ke kelepona me ka gene hewa e lilo i kelepona maʻi kanesa, e māhele wikiwiki ana a hana i nā kope nona iho. Hoʻopiha kēia mau kelepona maʻi kanesa i ka iwi iwi holoʻokoʻa.
ʻAʻole noho wale kēia mau pūnaewele ʻino ma laila. ʻO nā kemika a lākou e hoʻokuʻu ai e hōʻino i ka iwi iwi a hoʻomaka ka hana ʻana o nā ʻiʻo ʻeha. Ua kapa ʻia kēia he fibrosis.
Ua ʻike nā kauka he nui ka poʻe me ka maʻi i loaʻa i kekahi mau hoʻololi maʻamau i loko o ko lākou mau genes. ʻO nā mea nui o kēia mau mea:
- JAK2 (JACK-TWO)
- CALR (Cal-R)
- MPL
ʻO kēia mau hoʻololi ʻana o ka genetic ke kumu o kēia maʻi i loko o ka iwi iwi.
ʻO wai ka mea i ʻoi aku ka pilikia o ka loaʻa ʻana o kēia maʻi?
ʻOiai hiki i kekahi ke loaʻa i kēia maʻi, ʻoi aku ka nui o ka pilikia o kekahi poʻe.
- Ma luna o 60 mau makahiki: ʻIke pinepine ʻia kēia maʻi i waena o ka poʻe ʻelemakule.
- Ke alo ʻana o nā maʻi koko ʻē aʻe:No ka poʻe me nā maʻi e like me 'Essential Thrombocytosis' a i ʻole 'Polycythemia Vera', a mākou i kamaʻilio ai ma mua.
- Ka ʻike ʻia ʻana i ka radiation ionizing: Ka ʻike ʻia ʻana i nā nui kiʻekiʻe o ka radiation. (He mea kākaʻikahi loa kēia).
- Ka ʻike ʻia ʻana i kekahi mau kemika: Ka ʻike lōʻihi ʻia ʻana i nā kemika i hoʻohana ʻia i loko o nā hale hana, e like me ka benzene (he mea kākaʻikahi loa kēia).
He aha nā pilikia e hiki ke kū mai ma muli o ka maʻi?
I ka holomua ʻana o ka myelofibrosis, hiki ke loaʻa nā pilikia like ʻole.
Ua emi ka hana ʻana o nā ʻāpana koko
Ke hōʻiliʻili ʻia nā ʻiʻo ʻeha i loko o ka iwi iwi, ʻaʻole hiki iā ia ke hana i nā hunaola koko olakino. Hiki i kēia ke alakaʻi i ka emi ʻana o nā hunaola koko ʻulaʻula, e hoʻoulu ai i ka anemia , a me ka emi ʻana o nā platelets, e hoʻonui ana i ka pilikia o ke kahe ʻana o ke koko.
Ka hoʻokumu ʻia ʻana o nā hunaola koko extrahepatic mai ka iwi iwi
Ke hiki ʻole i ka iwi iwi ke hana i kāna hana, hoʻomaka nā ʻōpū e like me ka spleen a me ke akepaʻa e hana i ke koko. Hiki i kēia ke hoʻoulu i kēlā mau ʻōpū e pehu a hoʻopilikia i kā lākou hana.
Ke koko kiʻekiʻe ma ka vein portal
ʻO ke aʻa koko portal ke kīʻaha koko nui e lawe ana i ke koko mai ka spleen i ke akepaʻa. Hiki i ka spleen i hoʻonui ʻia ke ālai i ke kahe o ke koko ma o kēia aʻa koko, e hoʻonui ana i ke kaomi i loko. Hiki i kēia ke alakaʻi i ke kahe koko nui.
Ke lilo nei i ka maʻi leukemia koʻikoʻi
I kekahi mau maʻi, hiki i ka myelofibrosis ke lilo i ʻano leukemia koʻikoʻi a laha koke i kapa ʻia ʻo Acute Myeloid Leukemia (AML).
Pehea e ʻike pololei ai ke kauka i kēia maʻi?
Ma hope o ka hoʻolohe ʻana i kāu mau hōʻailona a nānā iā ʻoe, e kauoha ke kauka i kekahi mau hoʻokolohua e hōʻoia i ka maʻi.
1. Nā Hoʻāʻo Koko: Nānā kēia mau mea inā he maʻamau a ʻano ʻē paha ka helu o kāu mau cell koko (ʻulaʻula, keʻokeʻo, platelets). Nānā pū lākou inā he ʻano ʻē ke ʻano o nā cell koko.
2. Nā Hoʻāʻo Kiʻi: Hiki ke hana ʻia kahi scan CT a i ʻole ultrasound scan e ʻike inā ua hoʻonui ʻia kou spleen a i ʻole ke akepaʻa. Hiki i kahi scan MRI ke kōkua i ka ʻike inā he mau ʻeha ma ka iwi iwi.
3. Biopsy o ka iwi iwi: ʻO kēia ka hoʻāʻo koʻikoʻi loa e hōʻoia i ka maʻi. Ma kēia, lawe ʻia kahi laʻana liʻiliʻi o ka iwi iwi, maʻamau mai ka iwi ʻūhā a i ʻole kekahi iwi nui ʻē aʻe, ma lalo o kahi anesthetic māmā. Hoʻouna ʻia i kahi lab e hoʻoholo pono i ka nui o ka ʻiʻo ʻeha i loaʻa, inā he mau hunaola kanesa, a inā he mau hoʻololi genetic e like me ka `JAK2` a mākou i kamaʻilio ai ma mua.
Ma muli o nā hopena o kēia mau hoʻokolohua, e hoʻoholo kāu kauka inā paha kou maʻi i nā pae mua (prefibrotic) a i ʻole nā pae holomua (overt). E loiloi pū lākou i kou pae pilikia (haʻahaʻa, waena, a i ʻole kiʻekiʻe-pilikia) ma muli o kāu mau hōʻailona a me ke ʻano o ka hoʻololi ʻana o ka genetic. Hoʻolālā ʻia ka lāʻau lapaʻau ma muli o kēia pae pilikia.
He aha nā lāʻau lapaʻau no kēia?
ʻO ke ʻano hana lapaʻau e pili ana i ka pae o ka pilikia o kou maʻi, ke koʻikoʻi o kou mau hōʻailona, kou mau makahiki, a me kou olakino holoʻokoʻa.
ʻO ka mea nui, ʻo ka mea i haʻahaʻa ka pilikia a ʻaʻohe ona hōʻailona, ʻaʻole paha e hāʻawi ʻia kekahi lāʻau lapaʻau i ka wā mua. Akā, e nānā mau ke kauka iā ʻoe (Watchful Waiting).
Aia kekahi mau lāʻau lapaʻau no nā kūlana waena a me ke kūlana kiʻekiʻe . ʻO ka pahuhopu nui o ka lāʻau lapaʻau, ʻo ia ke kāohi i nā hōʻailona a pale i ka maʻi mai ka ʻoi aku ka ʻino.
| Hōʻailona/kūlana e mālama ʻia nei | Nā ʻano lapaʻau i hoʻohana ʻia |
|---|---|
| No nā hōʻailona laulā a me ka spleen i hoʻonui ʻia | Nā lāʻau lapaʻau i kuhikuhi ʻia i kapa ʻia ʻo JAK inhibitors. Nā laʻana: Ruxolitinib (Jakafi®), Fedratinib (Inrebic®), Pacritinib (Vonjo®), Momelotinib (Ojjaara®). Hoʻemi kēia i ka nui o ka spleen a kāohi i nā hōʻailona e like me ka luhi a me ka hou ʻana i ka pō. |
| No ka Anemia | - Nā lāʻau lapaʻau e kōkua i ka hana ʻana i nā hunaola koko ʻulaʻula (e like me Erythropoietin) - Nā lāʻau lapaʻau ʻē aʻe (Androgens, Immunomodulators, Corticosteroids) - Nā hoʻoili koko inā pono |
| No ka spleen i hoʻonui ʻia (nā ʻano hana ʻē aʻe) | - Nā lāʻau lapaʻau chemotherapy - Lapaʻau radiation i ka spleen - ʻAʻole hoʻohana pinepine ʻia kēia. - Splenectomy - He hana kakaikahi loa kēia a he pilikia hoʻi. |
Hoʻoili Kelepona Kumu
ʻO kēia wale nō ka lāʻau lapaʻau e hiki ke ho'ōla loa i ka myelofibrosis. Eia nō naʻe, he hana pilikia loa ia me nā hopena ʻaoʻao koʻikoʻi. No laila, ʻaʻole kūpono ia no nā maʻi āpau. ʻO ka maʻamau, paipai nā kauka i kēia lāʻau lapaʻau no nā poʻe ʻōpio me ka pilikia kiʻekiʻe a me nā poʻe me ka ʻole o nā maʻi koʻikoʻi ʻē aʻe.
ʻO kēia ka hoʻopau loa ʻana i ka iwi maʻi o ka mea maʻi a me ka hāʻawi ʻana i nā hunaola kumu i loaʻa mai kahi mea hāʻawi olakino.
Leka Lawe-Home
- He maʻi ʻaʻai koko ka Myelofibrosis e hoʻoulu ai i ka ʻeha a me ka ulu ʻana o ka iwi iwi. ʻOiai he mea weliweli kēia, mai hopohopo.
- Inā loaʻa iā ʻoe nā hōʻailona e like me ka luhi nui i wehewehe ʻole ʻia, ke kaumaha ma ka ʻaoʻao hema o kou ʻōpū, a i ʻole ka maʻalahi o ka palapū, mai hoʻowahāwahā iā lākou a e ʻike koke aku i ke kauka.
- He mea nui nā hoʻāʻo koko a me ka biopsy iwi no ka ʻike pololei.
- Inā ʻoe e pono ai i ka lāʻau lapaʻau, a me ke ʻano o ka lāʻau lapaʻau kūpono, ma muli ia o ka pae o ka pilikia o kou maʻi a me kāu mau hōʻailona.
- E kamaʻilio ākea me kāu kauka. E nīnau i kou mau makaʻu a pau, nā kānalua, a me nā nīnau. E makaʻala i nā hopena ʻaoʻao o nā lāʻau lapaʻau.
- I ka holomua ʻana o ka ʻepekema lapaʻau, ke puka mau mai nei nā lāʻau lapaʻau hou no kēia maʻi. No laila, e kū i ka lāʻau lapaʻau me ka manaʻolana a me ka maikaʻi.
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