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October 10, 2025
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ʻO Tagraxofusp-erzs he lāʻau lapaʻau i manaʻo ʻia no ka maʻi kanesa e kōkua ana i ka hakakā ʻana i kahi maʻi koko kakaʻikahi i kapa ʻia ʻo blastic plasmacytoid dendritic cell neoplasm (BPDCN). Hana kēia lapaʻau kūikawā ma ka hoʻopili ʻana i nā protein kikoʻī ma nā sela maʻi kanesa a hāʻawi i kahi mea ʻawaʻawa e luku iā lākou mai loko mai. Ua hoʻolālā ʻia no nā mākua a me nā keiki i loaʻa kēia ʻano maʻi koko, e hāʻawi ana i ka manaʻolana ke ʻaʻole kūpono nā lāʻau lapaʻau ʻē aʻe.
ʻO Tagraxofusp-erzs he lāʻau lapaʻau maʻi kanesa i kauoha ʻia e hui pū ana i ʻelua mau mea ikaika e hakakā i nā sela maʻi kanesa. Hana ka ʻāpana mua e like me ka missile alakaʻi, e ʻimi ana a hoʻopili i nā protein kikoʻī i kapa ʻia ʻo CD123 receptors ma nā sela maʻi kanesa. Hāʻawi ka ʻāpana ʻelua i kahi mea ʻawaʻawa e luku i kēia mau sela i manaʻo ʻia me ka waiho ʻana i nā sela olakino me ka ʻole o ka pōʻino.
No ka papa o nā lāʻau lapaʻau i kapa ʻia ʻo CD123-directed cytotoxins kēia lāʻau lapaʻau. E noʻonoʻo iā ia he pōkā akamai e hiki ke hoʻokaʻawale ma waena o nā sela olakino a me nā sela maʻi kanesa. Hāʻawi ʻia ka lāʻau lapaʻau ma o ka IV infusion, e ʻae ana iā ia e hele i loko o kou kahe koko e hiki ai i nā sela maʻi kanesa ma kahi e hūnā ai.
E hoʻomākaukau a hāʻawi kāu hui mālama ola i kēia lāʻau lapaʻau i loko o kahi keʻena lapaʻau kahi e hiki ai iā ʻoe ke nānā pono ʻia. Kōkua kēia ala mālama e hōʻoia i kou palekana a hiki ke pane koke inā loaʻa kekahi hopena ʻaoʻao i ka wā o ka mālama ʻana.
Ua ʻae ʻia ʻo Tagraxofusp-erzs e mālama i ka blastic plasmacytoid dendritic cell neoplasm (BPDCN) i nā mākua a me nā keiki 2 mau makahiki a ʻoi aku. He maʻi koko kakaʻikahi a hoʻomāhuahua ʻo BPDCN e pili ana i nā sela immune kūikawā i kapa ʻia ʻo plasmacytoid dendritic cells.
Hōʻike pinepine ʻia kēia maʻi maʻi ma ke ʻano he mau maʻi ma ka ʻili, nā ʻōpū lymph i pehu, a i ʻole e pili ana i ka iwi iwi a me ke koko. No ka mea he kakaikahi loa ka BPDCN, e pili ana i ka liʻiliʻi ma mua o 1 i loko o 100,000 poʻe, ua kaupalena ʻia nā koho lapaʻau i loaʻa ma mua o ka hoʻomohala ʻia ʻana o kēia lāʻau.
Hiki i kāu kauka ke ʻōlelo aku i kēia lapaʻau inā ua ʻike ʻia ʻoe me BPDCN a ʻaʻole i hana nā lapaʻau ʻē aʻe a ʻaʻole kūpono paha no kou kūlana. Ua hōʻike ka lāʻau lapaʻau i ka hoʻohiki i nā hoʻokolohua lapaʻau, me ka nui o nā maʻi e ʻike ana i ka hoʻomaikaʻi nui ʻana i kā lākou mau hōʻailona maʻi maʻi.
Hana ʻo Tagraxofusp-erzs ma o kahi kaʻina hana ʻelua ʻanuʻu i hoʻopaʻa ʻia i nā sela maʻi maʻi me ka pololei loa. Hoʻolālā ʻia ka lāʻau lapaʻau e ʻimi a paʻa i nā mea hoʻokipa CD123, i loaʻa i nā helu kiʻekiʻe ma nā sela maʻi BPDCN akā ʻaʻole maʻamau ma nā sela olakino.
Ke pili ka lāʻau lapaʻau i kēia mau mea hoʻokipa, kōkua maoli ka cell maʻi maʻi i ke kaʻina hana ma ka huki ʻana i ka lāʻau lapaʻau i loko ma o kahi kaʻina hana maoli i kapa ʻia ʻo endocytosis. E noʻonoʻo e like me ka cell maʻi maʻi e wehe ana i kona puka me ka ʻike ʻole e hoʻokuʻu i ka lāʻau lapaʻau i loko.
I loko o ka cell maʻi maʻi, hoʻokuʻu ka lāʻau lapaʻau i kāna ukana ʻona, e hoʻopilikia ana i ka hiki o ka cell ke hana i nā protein e pono ai no ke ola. Ke alakaʻi nei kēia i ka make ʻana o ka cell maʻi maʻi me ka hōʻemi ʻana i ka pōʻino i nā sela olakino ʻaʻole i nui nā mea hoʻokipa CD123.
Ua manaʻo ʻia kēia ʻano hana i hoʻopaʻa ʻia he ikaika a maikaʻi hoʻi no BPDCN, ʻoiai hiki iā ia ke hana i nā hopena ʻaoʻao nui no ka mea he nui nā sela olakino i nā mea hoʻokipa CD123, ʻoi aku hoʻi i ke ake a me nā kīʻaha koko.
Hāʻawi ʻia ʻo Tagraxofusp-erzs ma ke ʻano he intravenous (IV) infusion pololei i loko o kou kahe koko ma kahi keʻena lapaʻau. ʻAʻole hiki iā ʻoe ke lawe i kēia lāʻau lapaʻau ma ka home, a pono e hoʻomākaukau ʻia a lawelawe ʻia e nā poʻe loea mālama ola i hoʻomaʻamaʻa ʻia i ka mālama ʻana i ka maʻi maʻi.
ʻO ka papa hana maʻamau e pili ana i ka loaʻa ʻana o ka lāʻau lapaʻau i hoʻokahi manawa i ka lā no nā lā ʻelima mua o ka pōʻai 21-lā. E hoʻokomo kāu hui mālama ola i kahi laina IV i loko o kahi vein i kou lima a i ʻole ma o kahi laina waena inā loaʻa iā ʻoe kekahi. Ma kahi o 15 mau minuke e pau ai ka hoʻokomo ʻana.
Ma mua o kēlā me kēia hoʻokomo ʻana, e loaʻa iā ʻoe nā lāʻau lapaʻau ma mua e kōkua ai i ka pale ʻana i nā hopena maʻi a me nā hopena ʻē aʻe. Hiki i kēia mau mea ke komo i nā antihistamines, corticosteroids, a me nā mea hoʻemi wela. E nānā pono kāu hui lapaʻau i kāu mau hōʻailona ola i ka wā a ma hope o ka hoʻokomo ʻana.
ʻAʻole pono ʻoe e hopohopo e pili ana i nā kapu meaʻai ma mua o kāu lapaʻau ʻana, akā he mea nui ka noho ʻana i ka wai. Hiki i kāu hui mālama ola ke ʻōlelo aku e inu i ka wai nui i nā lā e alakaʻi ana i kāu lapaʻau ʻana e kōkua i kou mau puʻupaʻa e hana pono i ka lāʻau lapaʻau.
ʻO ka lōʻihi o kāu lapaʻau tagraxofusp-erzs e hilinaʻi ana i ka maikaʻi o ka pane ʻana o kāu maʻi maʻi a me ka maikaʻi o kou ʻae ʻana i ka lāʻau lapaʻau. Loaʻa ka hapa nui o nā maʻi i nā pōʻaiapuni he nui o ka lapaʻau ʻana, me kēlā me kēia pōʻaiapuni e mau ana i 21 mau lā a me ka hoʻokomo ʻana i nā lā ʻelima o ka hoʻokele lāʻau lapaʻau.
E nānā mau kāu kauka i kāu maʻi maʻi ma o nā hoʻokolohua koko, nā haʻawina kiʻi, a me nā hoʻokolohua kino e hoʻoholo ai inā e hana ana ka lapaʻau. Inā e pane maikaʻi ana kāu maʻi maʻi a ke ʻae nei ʻoe i ka lāʻau lapaʻau me ka ʻole o nā hopena ʻaoʻao koʻikoʻi, hiki iā ʻoe ke hoʻomau i ka lapaʻau ʻana no nā pōʻaiapuni he nui.
Hiki i kekahi poʻe maʻi ke hoʻokō i ke kala ʻana ma hope o kekahi mau pōʻaiapuni, aʻo nā mea ʻē aʻe e pono ai ka lapaʻau lōʻihi. E hana pū kāu hui mālama ola me ʻoe e ʻimi i ke kaulike kūpono ma waena o ka hakakā ʻana i kāu maʻi maʻi a me ka mālama ʻana i kou ola maikaʻi.
Hoʻomaʻamaʻa nui ʻia nā hoʻoholo lapaʻau, a e noʻonoʻo kāu kauka i nā mea e like me kou olakino holoʻokoʻa, ka pane ʻana o ka maʻi maʻi, a me nā hopena ʻaoʻao āu e ʻike ai i ka wā e hoʻoholo ai i ka lōʻihi e hoʻomau ai i ka therapy.
Hiki i ka Tagraxofusp-erzs ke hana i nā hopena ʻaoʻao like ʻole, mai ka maʻalahi a i ka koʻikoʻi, no ka mea, ʻaʻole pili ka lāʻau lapaʻau i nā sela maʻi wale nō, akā i kekahi mau sela olakino i loaʻa nā mea loaʻa CD123. Hiki i ka hoʻomaopopo ʻana i kēia mau hopena hiki ke kōkua iā ʻoe a me kāu hui mālama ola e mālama pono iā lākou.
ʻO nā hopena ʻaoʻao maʻamau āu e ʻike ai, ʻo ia ka luhi, ka maʻi, ke kuni, a me ka pehu ʻana i kou mau lima, nā wāwae, a i ʻole ka maka. Nui nā maʻi e hoʻomohala i nā hopena ʻili, nā loli i nā hoʻokolohua hana ate, a me nā helu koko haʻahaʻa e hiki ke hoʻonui i kou pilikia o ka maʻi a i ʻole ke kahe ʻana.
Eia nā hopena ʻaoʻao e hana pinepine ʻia i nā maʻi e loaʻa ana i kēia lāʻau lapaʻau, ʻoiai ʻaʻole e ʻike nā mea a pau iā lākou:
Hiki ke mālama ʻia kēia mau hopena ʻaoʻao maʻamau me ka mālama kākoʻo a me nā lāʻau lapaʻau. E hana pū kāu hui mālama ola me ʻoe e hōʻemi i ko lākou hopena i kou ola i kēlā me kēia lā.
ʻOiai ʻaʻole maʻamau, hiki i kekahi mau maʻi ke ʻike i nā hopena ʻaoʻao koʻikoʻi e pono ai ka lāʻau lapaʻau koke a me ka nānā pono:
E nānā pono kāu hui lapaʻau iā ʻoe no kēia mau hopena ʻaoʻao koʻikoʻi a e hoʻōki i ka lapaʻau inā pono e pale i kou olakino. Nui nā hopena i hiki ke hoʻihoʻi ʻia me ka mālama olakino kūpono.
Hiki i kekahi poʻe maʻi ke ʻike i nā hopena ʻaoʻao laha, ʻoiai ʻaʻole maʻamau, he mea nui e ʻike:
Pono kēia mau hopena laha i ka lāʻau lapaʻau koke a hiki ke hopena i ka haukapila no ka nānā ʻana a me ka mālama ʻana.
ʻAʻole kūpono ʻo Tagraxofusp-erzs no nā mea a pau, a e loiloi pono kāu kauka inā palekana kēia lapaʻau iā ʻoe. ʻAʻole kūpono paha nā poʻe me kekahi mau maʻi olakino a i ʻole nā kūlana no kēia lāʻau.
ʻAʻole pono ʻoe e loaʻa i kēia lāʻau lapaʻau inā he maʻi ʻoe i ka tagraxofusp-erzs a i ʻole kekahi o kāna mau mea. E noʻonoʻo nō hoʻi kāu kauka i kou olakino holoʻokoʻa, me ka hana o ka naʻau, ke akepaʻa, a me nā puʻupaʻa, ma mua o ka ʻōlelo ʻana i kēia lapaʻau.
ʻAʻole hiki i nā maʻi me ka maʻi akepaʻa koʻikoʻi ke loaʻa i kēia lāʻau lapaʻau no ka mea hiki iā ia ke hoʻonui i nā pilikia o ke akepaʻa. Pēlā nō, ʻo ka poʻe me nā maʻi naʻau koʻikoʻi e pilikia paha i nā pilikia mai ka mālama wai a me nā hopena cardiovascular ʻē aʻe.
ʻAʻole pono nā wahine hāpai e loaʻa i ka tagraxofusp-erzs no ka mea hiki iā ia ke hōʻeha i ka pēpē e ulu ana. Inā he makahiki hānau keiki kāu, e kūkākūkā kāu kauka i nā ʻano hana hoʻomalu hānau pono e hoʻohana ʻia i ka wā o ka lapaʻau a no kekahi mau mahina ma hope.
Kūʻai ʻia ʻo Tagraxofusp-erzs ma lalo o ka inoa brand Elzonris. Hoʻohana ʻia kēia inoa brand ma ʻAmelika Hui Pū ʻIa a me nā ʻāina ʻē aʻe kahi i ʻae ʻia ai ka lāʻau lapaʻau no ka mālama ʻana i ka BPDCN.
Ke loaʻa iā ʻoe kāu lapaʻau, hiki iā ʻoe ke ʻike i kekahi inoa ma kāu moʻolelo olakino a i ʻole nā palapala ʻinikua. E pili ana nā inoa ʻelua i ka lāʻau lapaʻau hoʻokahi, no laila, mai huikau inā ʻoe e ʻike i nā inoa like ʻole i hoʻohana ʻia i nā wahi like ʻole.
Hana ʻia ʻo Elzonris e Stemline Therapeutics a loaʻa wale nō ma o nā kikowaena lapaʻau maʻi maʻi kūikawā a me nā haukapila i loaʻa ka ʻike me kēia ʻano o ka therapy i manaʻo ʻia.
No ka mea he maʻi maʻi laha ʻole ka BPDCN, he palena nā lapaʻau ʻē aʻe i loaʻa. Ma mua o ka ʻae ʻia ʻana o ka tagraxofusp-erzs, hoʻohana maʻamau nā kauka i nā hui pū ʻana o nā lāʻau chemotherapy e like me nā mea i hoʻohana ʻia no nā maʻi maʻi koko ʻē aʻe.
Hiki i nā regimens chemotherapy kuʻuna ke komo i nā hui pū ʻana o nā lāʻau e like me ka cyclophosphamide, doxorubicin, vincristine, a me prednisone. Eia naʻe, ʻaʻole pinepine kēia mau lapaʻau i ka pono kūpono i ka BPDCN a hiki ke hoʻoulu i nā hopena ʻaoʻao nui.
No kekahi poʻe maʻi, hiki ke noʻonoʻo ʻia ka hoʻololi ʻana i nā cell stem, ʻoiai inā loaʻa iā lākou ka remission me ka mālama mua. Hoʻokomo kēia hana ikaika i ka hoʻololi ʻana i ka iwi iwi maʻi me nā cell donor olakino.
Hiki nō hoʻi i nā hoʻokolohua lapaʻau ke hāʻawi i ke komo ʻana i nā lāʻau lapaʻau e hoʻokolokolo ʻia nei no BPDCN. Hiki i kāu oncologist ke kōkua iā ʻoe e ʻimi i nā koho a pau i loaʻa a hoʻoholo i ke ala maikaʻi loa no kāu kūlana kikoʻī.
Hōʻike ʻo Tagraxofusp-erzs i kahi holomua nui i ka mālama ʻana iā BPDCN i hoʻohālikelike ʻia me nā ala chemotherapy kuʻuna. Ua hōʻike nā hoʻokolohua lapaʻau e hiki i kēia lāʻau lapaʻau i manaʻo ʻia ke ʻoi aku ka maikaʻi ma mua o nā lāʻau lapaʻau maʻamau no nā maʻi he nui me kēia maʻi maʻi laha.
ʻO ke ala i manaʻo ʻia o ka lāʻau lapaʻau, ʻo ia hoʻi, hiki iā ia ke hoʻouka i nā cell maʻi maʻi me ka pololei aʻe me ka hiki ke hana i nā hopena ʻaoʻao koʻikoʻi e pili ana me ka chemotherapy ākea. Hiki i kēia ke alakaʻi i nā hopena maikaʻi a me ka maikaʻi o ke ola no nā maʻi he nui.
Eia naʻe,
E hoʻāʻo kāu kauka i ka hana o kou ate ma mua o ka hoʻomaka ʻana i ka lāʻau a e hoʻomau i ka nānā ʻana i ka wā o kāu lāʻau lapaʻau. Inā he mau pilikia ate liʻiliʻi kāu, hiki nō paha iā ʻoe ke loaʻa ka lāʻau lapaʻau me ka nānā pono ʻana a me ka hoʻoponopono ʻana i ka dosage.
ʻAʻole kūpono ka poʻe me ka maʻi ate koʻikoʻi no kēia lāʻau lapaʻau no ka mea hiki i nā pilikia ke ʻoi aku ma mua o nā pōmaikaʻi. E hana pū kāu hui mālama ola me ʻoe e ʻimi i ke ala lapaʻau palekana a maikaʻi loa no kou kūlana.
Inā loaʻa iā ʻoe nā hopena ʻaoʻao koʻikoʻi e like me ka pilikia o ka hanu ʻana, ka pehu nui ʻana, ka ʻeha nui o ka ʻōpū, a i ʻole nā loli i ke kūlana noʻonoʻo, pono ʻoe e ʻimi koke i ka lāʻau lapaʻau. Mai kali e ʻike inā e hoʻomaikaʻi nā hōʻailona iā lākou iho.
E hoʻokaʻaʻike koke i kāu hui mālama ola inā loaʻa iā ʻoe ke kuni, nā hōʻailona o ka maʻi, ke kahe ʻana o ke koko a i ʻole ka ʻeha maʻamau, a i ʻole ka luhi nui e pale ai iā ʻoe mai ka hana ʻana i nā hana o kēlā me kēia lā. He mau hōʻailona paha kēia o nā pilikia koʻikoʻi e pono ai ka lāʻau lapaʻau wikiwiki.
Loaʻa i kāu hui lapaʻau ka ʻike i ka mālama ʻana i nā hopena ʻaoʻao o kēia lāʻau lapaʻau a hiki ke hāʻawi i nā lāʻau lapaʻau e kōkua iā ʻoe e ʻoi aku ka maikaʻi. Hiki iā lākou ke hoʻoponopono i kāu papa hana lāʻau lapaʻau, hāʻawi i ka mālama kākoʻo, a i ʻole e hoʻomaha i ka lāʻau lapaʻau no ka manawa inā pono.
E mālama i kahi papa inoa o nā helu pili pilikia mai kāu hui mālama ola a e ʻike i kahi haukapila a i ʻole ke kikowaena lapaʻau e hele ai inā pono ʻoe i ka mālama koke ʻana ma waho o nā hola hana maʻamau.
E nānā kāu kauka i kāu pane ʻana i ka tagraxofusp-erzs ma o nā hoʻokolohua koko maʻamau, nā hoʻokolohua kino, a me nā haʻawina kiʻi. Hiki iā ʻoe ke hoʻomaka e ʻike i nā hoʻomaikaʻi ʻana i nā hōʻailona e like me ka luhi, nā ʻeha ʻili, a i ʻole nā ʻōpū lymph i pehu i loko o nā pōʻai lapaʻau mua.
E hōʻike nā hoʻokolohua koko i nā loli i nā pūnaewele maʻi 'aʻai a me nā helu koko holoʻokoʻa, aʻo nā haʻawina kiʻi e like me nā scans CT a i ʻole nā scans PET e hōʻike ana inā e emi ana nā maʻi. E wehewehe kāu kauka i ka mea e hōʻike ai kēia mau hoʻokolohua a me ke ʻano o lākou no kāu hoʻolālā lapaʻau.
Hiki i kekahi poʻe maʻi ke manaʻo i ka ʻino i ka wā mua i ka wā e luku ʻia ai kā lākou mau pūnaewele maʻi 'aʻai, hiki ke hoʻonui i kekahi mau hopena ʻaoʻao no ka manawa. ʻAʻole pono kēia i ka manaʻo ʻaʻole e hana ana ka lapaʻau, akā e kōkua kāu hui mālama ola iā ʻoe e hoʻomaopopo i ka mea e manaʻo ai.
Hiki ke ʻokoʻa loa ka pane ʻana i ka lapaʻau ma waena o nā maʻi, a hiki ke lawe i kekahi mau pōʻai ma mua o kou a me kāu kauka e loiloi piha i ka maikaʻi o ka hana ʻana o ka lāʻau lapaʻau iā ʻoe.
Hiki i nā maʻi he nui ke hoʻomau i kekahi mau hana maʻamau i ka wā lapaʻau, akā pono paha ʻoe e hana i nā hoʻoponopono e pili ana i kou manaʻo a me kāu mau hopena ʻaoʻao. He mea maʻamau ka luhi, no laila pono paha ʻoe e hoʻomaha ma mua o ka maʻamau a me ka wikiwiki i kāu mau hana.
Pono ʻoe e pale i nā wahi lehulehu a me nā poʻe maʻi no ka mea hiki i kēia lāʻau lapaʻau ke hoʻohaʻahaʻa i kāu helu keʻokeʻo koko, e hoʻonui ai iā ʻoe i nā maʻi. E hāʻawi kāu hui mālama ola i nā alakaʻi kikoʻī e pili ana i ka wā e palekana ai ke noho me nā poʻe ʻē aʻe.
Hiki ke maikaʻi ka hoʻoikaika kino māmā e like me ka hele wāwae inā ʻoe e manaʻo ana, akā e pale i nā hana ikaika e hoʻonui ai i kou pilikia o ka ʻeha, ʻoiai inā haʻahaʻa kāu helu platelet. Pono e pale ʻia ka ʻauʻau ʻana i nā loko wai lehulehu ma muli o ka pilikia o ka maʻi.
E kamaʻilio me kāu hui mālama ola e pili ana i kāu hana, nā hoʻolālā huakaʻi, a me nā hana ʻē aʻe. Hiki iā lākou ke kōkua iā ʻoe e hana i nā hoʻoholo i ʻike ʻia e pili ana i ka mea palekana a kūpono i ka wā o kāu lapaʻau.
ʻO ka hapa nui o nā maʻi e loaʻa ana iā tagraxofusp-erzs ma ke ʻano he lāʻau lapaʻau outpatient, ʻo ia hoʻi hiki iā ʻoe ke hoʻi i ka home i ka lā hoʻokahi ma hope o kēlā me kēia infusion. Eia naʻe, e koi ana kāu mau lāʻau lapaʻau mua i ka nānā pono ʻana, a pono paha ʻoe e noho ma ke kikowaena lapaʻau no kekahi mau hola ma hope o kēlā me kēia infusion.
Pono paha kekahi mau maʻi e hoʻopaʻa ʻia i ka haukapila, ʻoiai inā e loaʻa iā lākou nā hopena ʻaoʻao koʻikoʻi e like me ka capillary leak syndrome a i ʻole nā pilikia ate koʻikoʻi. E hoʻoholo kāu hui mālama ola i ke ʻano palekana loa no kāu lapaʻau ʻana e pili ana i kāu mau mea pilikia pilikino.
Inā noho mamao ʻoe mai ke kikowaena lapaʻau, hiki i kāu kauka ke ʻōlelo aku e noho kokoke i ka wā o kāu mau pōʻai lapaʻau i hiki iā ʻoe ke loaʻa ke kōkua wikiwiki inā pono. Nui nā kikowaena maʻi maʻi e hiki ke hāʻawi i ka ʻike e pili ana i nā koho noho no nā maʻi a me nā ʻohana.
E kūkākūkā kāu hui lapaʻau i ka hoʻolālā nānā ʻana me ʻoe a kōkua iā ʻoe e hoʻomaopopo i ka mea e manaʻo ai i ka wā a ma hope o kēlā me kēia kau lapaʻau. E hāʻawi pū lākou i nā ʻōlelo aʻo maopopo e pili ana i ka manawa e ʻimi ai i ka lāʻau lapaʻau koke.
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