He aha ka Sézary Syndrome? Pehea kona hopena i kou ʻili?

Ua ʻike anei ʻoe ua ʻulaʻula koke kou ʻili, ʻānai nui, a lilo i ʻeleʻele ma kekahi mau wahi? Manaʻo paha ʻoe he maʻi ʻili maʻamau wale nō ia, akā i nā hihia kakaikahi, hiki ke lilo i mea ʻoi aku ka koʻikoʻi. ʻO ia ka mea a mākou e kamaʻilio nei i kēia lā, ʻo Sézary Syndrome, kahi maʻi ʻaʻai kakaikahi e hoʻopilikia i ka ʻili, ke koko, a me nā lymph node. Hiki paha iā ʻoe ke huikau iki i ka inoa, akā he mea nui e makaʻala i ia mea.

He aha ka Sézary Syndrome?

I ka ʻōlelo maʻalahi, ʻo Sézary Syndrome kahi ʻano maʻi ʻili kakaʻikahi loa a laha koke. Ma ke ʻano lapaʻau, no kahi hui o nā maʻi ʻaʻai i kapa ʻia ʻo cutaneous T-cell lymphoma. ʻO nā T-lymphocytes, a i ʻole nā ​​​​T-cells no ka pōkole, he ʻano cell koʻikoʻi i loko o ka ʻōnaehana pale o ko mākou kino. I loko o kēia maʻi, lilo kahi ʻano cell i kapa ʻia ʻo T-cells i maʻi ʻaʻai a hōʻiliʻili i loko o kou ʻili, koko, a me nā lymph nodes.

E noʻonoʻo e like me nā koa e pale ana i ko mākou kino, ʻo kēia mau `(nā pūnaewele T)`. Akā ke hele hewa kēia mau koa a hoʻomaka e hana kūʻē iā mākou, ʻo ia ka mea e hana ʻia. ʻO kēia ke kumu e loli ai ke kala o kou ʻili a ʻulaʻula. I kekahi manawa hiki i ka ʻili ke lilo i ʻeleʻele. Aia kekahi hiki ke ʻānai nui a me nā ʻeha ʻeha a me nā ʻōpala. ʻO ka mea weliweli loa, hiki i kēia mau hunaola kanesa i loko o ke koko ke laha i nā ʻāpana ʻē aʻe o ke kino, ʻo ia hoʻi, i nā ʻōpū o loko. ʻOiai ʻaʻohe lāʻau lapaʻau piha no kēia i kēia manawa, mai hopohopo , he nui nā lāʻau lapaʻau e kāohi i nā hōʻailona a hāʻawi iā ʻoe i ka hōʻoluʻolu.

Pehea ka maʻamau o kēia kūlana?

He mea kākaʻikahi loa kēia. ʻO ia hoʻi, he maʻi ia i ʻike pinepine ʻole ʻia. E noʻonoʻo, ʻoiai ma kahi ʻāina e like me ʻAmelika, hoʻokahi wale nō i loko o ka miliona o ka poʻe i hōʻike ʻia e loaʻa i kēia maʻi i kēlā me kēia makahiki. No laila, ʻaʻole kēia he mea i ʻike pinepine ʻia ma Sri Lanka. ʻO ia ke kumu he mea nui e makaʻala i kēia, no ka mea, inā ʻike ʻia nā hōʻailona, ​​​​hiki ke ʻike koke ʻia.

He aha nā hōʻailona o ka Sézary Syndrome?

ʻO ka hōʻailona nui o kēia maʻi he ʻulaʻula ʻokoʻa (erythroderma) . ʻAʻole kēia he ʻūlū wale nō, he ʻūlū loa ia, ʻeha, i kekahi manawa e ʻili ka ʻili a pālahalaha koke. I kekahi poʻe, ʻoiai ka poʻe me ka ʻili ʻeleʻele, hiki ke ʻike ʻia he mau kiko ʻeleʻele (hiki ke hina, poni, a ʻeleʻele paha). I nā pae mua, hiki ke paʻakikī ke ʻike i kēia mau hōʻailona no ka mea ua like lākou me nā maʻi ʻili maʻamau e like me ka eczema a i ʻole psoriasis. Manaʻo ka poʻe he nui he maʻi allergy maʻalahi kēia.

I ka holomua ʻana o ka Sézary Syndrome, e hoʻomaka ana kou ʻili e ulu i nā puʻupuʻu liʻiliʻi a piʻi aʻe (papules). Hiki i kekahi mau wahi ke manaʻo ʻoi aku ka mānoanoa a me ka paʻa ma mua o nā mea ʻē aʻe (plaque). I ka hopena, hiki ke ulu mai ka maʻi ʻili (nā tumors). Aia kekahi mau hōʻailona ʻē aʻe, a mākou e nānā ai:

• Nā kīpili lymph pehu - ma nā wahi e like me ka ʻāʻī, nā armpits, a me ka ʻūhā.
• Fiva - Ke kuni e hele mai me ke kumu ʻole.
• Luhi mau - He manaʻo o ka luhi me ka nānā ʻole i ka nui o kāu hiamoe ʻana.
• Ka pohō kaumaha i wehewehe ʻole ʻia.
• Pohō ka lauoho - I kekahi manawa hiki ke nalowale nā ​​kuʻemaka a me ka lauoho o ke kino.
• ʻEctropion (ka maka i hoʻohuli ʻia).
• Ka hoʻonui ʻia ʻana o ke akepaʻa (hepatomegaly).
• ʻO ka pehu ʻana (edema), ʻoi aku hoʻi ma nā wāwae haʻahaʻa.
• Ke hoʻomanawanui ʻole i ke anu - ʻO ke ʻano kēia ua ʻoi aku kou anu ma mua o nā poʻe ʻē aʻe, ʻoiai ma ka mahana lumi maʻamau.

He aha ke kumu o ka Sézary Syndrome?

Hana ʻia kēia no ka mea, ʻo nā ʻāpana olakino i kapa ʻia ʻo T-cell i loko o ko kākou kino e hana i nā hoʻololi genetic, e hoʻolilo ana iā lākou i mau ʻāpana maʻi kanesa ʻē. Māhele koke kēia mau ʻāpana ʻē a hoʻonui me ka ʻole o ke kāohi ʻia, a laila laha i ka ʻili, nā lymph node, a me ke koko. Ua like ia me ka nāhelehele, e laha ana ma nā wahi āpau me ke kāohi ʻole ʻia. Eia naʻe, ʻaʻole maopopo i nā loea ka mea e hoʻoulu ai i kēia mau loli genetic. ʻO ia hoʻi, he paʻakikī ke ʻōlelo pololei i ke kumu.

He aha nā kumu pilikia no kēia?

ʻOiai hiki i kekahi ke loaʻa i kēia maʻi, hiki i kekahi mau mea ke hoʻonui i kou pilikia. No ka laʻana, inā ua loaʻa iā ʻoe ka maʻi leukemia T-cell kanaka (HTLV), ua manaʻo ʻia ʻoe he piʻi iki ka pilikia o ka loaʻa ʻana o ka maʻi Sézary. Eia nō naʻe, he mea nui e hoʻomanaʻo ʻaʻole e loaʻa i nā mea āpau i loaʻa i kēia maʻi ka maʻi.

He aha nā pilikia e hiki mai ana o kēia maʻi?

ʻO ka Sézary Syndrome kahi maʻi e hiki ke hoʻopilikia nui i ke ʻano o ke ola. E noʻonoʻo, pehea lā ʻoe e maikaʻi ai ke ʻānai mau ʻoe i kou ʻili a me ka ʻeha? Hiki iā ia ke hana i nā mea e like me ka hiamoe ʻole, ka hopohopo mau, a me ke kaumaha. Aia kekahi mau pilikia ʻē aʻe:

• Hoʻolaha nā pūnaewele T kanesa i kou mau māmā, ate, spleen, a me ka iwi iwi.
• ʻO ka hoʻonui ʻia o ka pilikia o ka hoʻomohala ʻana i kahi ʻano lymphoma ʻē aʻe (ka maʻi ʻaʻai o ka ʻōnaehana lymphatic).
• Hoʻonui ʻia ka pilikia o nā maʻi pinepine no ka mea ua hōʻino ʻia ka papa pale o ka ʻili, e ʻae ana i nā germs e komo maʻalahi.

Pehea e ʻike ai nā kauka i kēia maʻi?

Ke hele ʻoe e ʻike i ke kauka, e hana mua ʻo ia i kahi hoʻokolokolo kino a nīnau iā ʻoe e pili ana i kāu mau hōʻailona. E nīnau pū ʻo ia e pili ana i kou mōʻaukala olakino i hala. E hana ke kaukaInā kānalua ʻia ka Sézary Syndrome, hiki iā ia ke hana i kekahi mau hoʻokolohua, e like me:

• Nā hoʻāʻo koko: He mea nui loa kēia. Hiki i kēia ke komo pū me ka "Helu Koko Piha (CBC)" (nānā kēia i ka pakeneka o nā ʻano ʻano cell like ʻole i loko o ke koko, ʻoiai nā cell koko keʻokeʻo), kahi "helu koko Sézary" (nāna e nānā i ka helu o nā "cell Sézary" (nā cell kanesa maʻamau o kēia maʻi) i loko o ke koko), a me kahi "pahu koko peripheral" (kahi hoʻokolokolo microscopic o kahi hāpana koko).
• ʻIli, lymph node, a i ʻole nā ​​biopsies iwi: Ma kēia, lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai kahi ʻāpana i hoʻopilikia ʻia o ka ʻili a i ʻole kahi puʻupuʻu a hoʻouna ʻia i kahi keʻena hoʻokolohua. Ma laila, nānā ke kauka lapaʻau i ka hāpana ʻiʻo ma lalo o kahi microscope. Hiki i kēia ke nānā i nā mea e like me nā māka tumor a me nā loli i loko o nā cell.
• Nā hoʻāʻo e ʻike ai inā ua laha ke kanesa: Inā hōʻoia nā hoʻāʻo koko a me nā ʻiʻo ua loaʻa iā ʻoe ka maʻi Sézary, e hana ʻia nā hoʻāʻo e ʻike ai inā ua laha ke kanesa i nā ʻāpana ʻē aʻe o ke kino. Hiki i kēia ke komo pū me kahi X-ray umauma, nā scan CT (computerized tomography), a me kahi scan Positron Emission Tomography (PET) (nāna e nānā i ka hana o nā cell i loko o ke kino).

He aha nā lāʻau lapaʻau no ka Sézary Syndrome?

E kuhikuhi kāu kauka i ka lāʻau lapaʻau e pili ana i kāu mau hōʻailona a me ke kahua o kou maʻi ʻaʻai (pehea ka mamao o kona laha ʻana). Aia nā ʻano lāʻau lapaʻau like ʻole no kēia. ʻAʻole loaʻa i nā kānaka a pau ka lāʻau lapaʻau like.

• Phototherapy: Hoʻohana kēia i nā ʻano kukui kūikawā e luku i nā hunaola kanesa i loko o ka ʻili. ʻO kekahi ʻano hana i kapa ʻia ʻo extracorporeal photopheresis. Ma kēia, lawe ʻia nā hunaola koko keʻokeʻo mai kou koko (leukapheresis), mālama ʻia me kahi lāʻau lapaʻau a hōʻike ʻia i ka mālamalama ultraviolet (kukui UV), a laila hoʻihoʻi ʻia i kou kino. ʻOkoʻa, hiki ke hāʻawi pololei ʻia ka phototherapy UV i ka ʻili.
• Lapaʻau radiation: Pili kēia i ka hoʻohana ʻana i nā kukuna ikehu kiʻekiʻe e luku i nā hunaola kanesa. Hiki ke hana ʻia kēia me ka hoʻohana ʻana i ka radiation therapy waho (me ka hoʻohana ʻana i kahi mīkini e hāʻawi i nā kukuna) a i ʻole ka radiation therapy electron beam ʻili holoʻokoʻa (total skin electron beam radiation therapy).
• Kemoterapia: Pili kēia i ka hāʻawi ʻana i nā lāʻau lapaʻau e pepehi i nā hunaola kanesa. Aia nā ʻano i hoʻopili ʻia i ka ʻili (kemoterapia topical) a me nā ʻano e hoʻopilikia i ke kino holoʻokoʻa (kemoterapia ʻōnaehana) (i hāʻawi ʻia ma ke ʻano he pila a i ʻole he inikini). ʻO Vorinostat (Zolinza®) kahi laʻana o kahi lāʻau lapaʻau i hāʻawi ʻia i ke kino holoʻokoʻa.
• Lapaʻau i kuhikuhi ʻia: Hoʻouka kēia mau lāʻau i nā pahuhopu kikoʻī i loaʻa wale ma nā hunaola kanesa. ʻO ka lāʻau lapaʻau antibody monoclonal kekahi o ia ʻano lāʻau lapaʻau i kuhikuhi ʻia i hoʻohana ʻia no ka maʻi Sézary.
• Immunotherapy: Pili kēia i ka hoʻoulu ʻana i ka ʻōnaehana pale ponoʻī o kou kino e hakakā i nā hunaola kanesa. Hiki ke hana ʻia kēia me ka hoʻohana ʻana i nā lāʻau lapaʻau e like me Interferon (Intron A®, Intron A Multidose Pen.®)).
• Nā lāʻau lapaʻau ʻē aʻe: Hoʻohana ʻia nā Corticosteroids (nā lāʻau ʻano steroid) a me nā kirīmi a i ʻole gels ʻano retinoid (e like me bexarotene (Targretin®)) e kāohi i nā hōʻailona ʻili.

"Akā e hoʻomanaʻo, ʻaʻole like kēia mau lāʻau lapaʻau a pau no nā kānaka a pau. Na kāu kauka e hoʻoholo i ka lāʻau lapaʻau maikaʻi loa no kou kūlana. Na ia e wehewehe i nā mea a pau iā ʻoe."

He aha nā hopena ʻaoʻao o ka lāʻau lapaʻau?

Loaʻa i ka hapa nui o nā lāʻau lapaʻau maʻi kanesa kekahi mau hopena ʻaoʻao. He mea maʻamau kēia. He mea nui e makaʻala iā lākou ma mua. Ma keʻano laulā, hiki iā ʻoe ke manaʻo i nā mea e like me ka luhi, ka nausea, ka luaʻi ʻana, ka nalowale o ka makemake, a me ka nalowale o ka lauoho. Hiki i ka ʻili (Phototherapy) ke hoʻopilikia i ka ʻili - i kekahi manawa maloʻo ka ʻili, ka ʻulaʻula. Hiki i ka ʻili (Targeted therapy) (nā lāʻau lapaʻau e hoʻololi i ka ʻōnaehana pale) a me ka ʻili (immunotherapy) ke hana i ka pehu ʻana, ka maʻi diarrhea, ka luhi, a me nā loli i nā ʻāpana koko (nā ʻano ʻino o ka helu koko) ke hana. He manaʻo maikaʻi e kamaʻilio me kāu kauka e pili ana i kēia a e makaʻala iā ia ma mua. Ma kēlā ala, inā loaʻa kekahi mea, hiki iā ʻoe ke maopopo ʻaʻole e hopohopo.

Hiki ke ho'ōla loa ʻia kēia?

ʻO kēia ka pilikia nui loa no nā kānaka he nui. ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no ka Sézary Syndrome. Akā , ʻaʻole ia he manaʻo e haʻalele ʻoe i ka manaʻolana . Nui nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona, ​​​​e hoʻōki i nā ʻāpana ʻē mai ka māhele ʻana a me ka ulu ʻana, a hoʻolohi i ka laha ʻana o ka maʻi kanesa. Hiki i kēia mau lāʻau lapaʻau ke hoʻomaikaʻi i kou ʻano ola.

Pehea ka lōʻihi o kēia maʻi?

He maʻi mau ka Sézary Syndrome. ʻO ke ʻano kēia, ʻaʻole ia he maʻi e hele mai a hele aku. Pono paha ʻoe i kekahi pae o ka mālama ʻana a me ka nānā ʻana o ke kauka no ke koena o kou ola.

Pehea ka nui o nā manawa e ola ai?

He pilikia koʻikoʻi kēia, a ʻaʻole ia e hoʻopilikia like i nā kānaka a pau. Wahi a ka ʻikepili o kēia manawa, ma kahi o 24% o ka poʻe me ka Sézary Syndrome e ola ana no ka liʻiliʻi o ʻelima mau makahiki ma hope o ka maʻi. Akā e hoʻomanaʻo, he kuhi wale nō kēia ma muli o nā ʻike o haʻi. He ʻokoʻa nā kānaka a pau. Hiki ke hoʻomaikaʻi ʻia kēia kūlana i ka wā e loaʻa ai nā lāʻau lapaʻau hou a ʻoi aku ka maikaʻi. No ka mea, he maʻi kakaikahi loa ka Sézary Syndrome, he paʻakikī ke hōʻiliʻili i ka ʻike lawa. Inā he mau nīnau kāu e pili ana i kou kūlana, ʻoi aku ka maikaʻi o ka nīnau ʻana i kāu kauka. Hiki iā ia ke wehewehe i nā ʻoiaʻiʻo e pili ana i kou kūlana.

Pehea wau e mālama ai iaʻu iho?

ʻO ke ola ʻana me ka Sézary Syndrome ʻo ia hoʻi, pono ʻoe e mālama pono iā ʻoe iho i ka wā e mālama ʻia ai ke ola holoʻokoʻa. Pono paha ʻoe e hana i nā hana hou aʻe e pale i kou ʻili.

• He mea nui loa ia e pale aku i ka lā pololei . Hiki i kekahi mau lāʻau lapaʻau ke hoʻopilikia i ka ʻili i ka lā.
• E hahai pono i nā ʻōlelo a ke kauka. E lawe i kāu lāʻau lapaʻau i ka manawa kūpono.
• E mālama i kou ʻili maʻemaʻe a hoʻohana i ka moisturizer (ma hope o ke kūkākūkā ʻana me ke kauka).
• E ʻai i ka meaʻai olakino.
• E ho'āʻo e hauʻoli i ka noʻonoʻo e like me ka hiki.

Āhea wau e ʻike ai i ke kauka?

Hiki i nā hōʻailona o ka Sézary Syndrome ke loli a ʻoi aku ka ʻino. No laila e makaʻala i nā loli i kou kino. Inā ʻike ʻoe i kekahi o kēia mau mea, e haʻi koke i kāu kauka:

• Inā ʻike ʻoe i nā loli i kou ʻili - ka ʻulaʻula a i ʻole ka ʻeleʻele e pālahalaha ana i nā wahi hou aʻe o kou ʻili, nā puʻupuʻu liʻiliʻi e nui ana, a i ʻole nā ​​puʻupuʻu hou e ʻike ʻia ana - he mau hōʻailona kēia e ʻoi aku ka ʻino o kou kūlana.
• Inā loaʻa pinepine iā ʻoe nā maʻi a lōʻihi ka manawa e ho'ōla ai. Inā loaʻa pinepine kou kuni a me nā ʻeha kino.

He aha nā nīnau āu e nīnau ai i ke kauka?

Inā loaʻa iā ʻoe ka Sézary Syndrome, nui paha kāu mau nīnau. He mea kōkua ke kūkākūkā me kāu kauka. Hiki iā ʻoe ke nīnau i kāu kauka i nā nīnau penei:

• He aha nā lāʻau lapaʻau maikaʻi loa iaʻu?
• He aha nā hopena ʻaoʻao e hiki iaʻu ke manaʻo mai kēia mau lāʻau lapaʻau? Pehea e hiki ai iaʻu ke hana me lākou?
• Aia kekahi mau hoʻokolohua lapaʻau (noiʻi ʻana i nā lāʻau lapaʻau hou) hiki iaʻu ke komo?
• Hiki ke hoʻi hou mai ke kanesa ma hope o ka mālama ʻia ʻana? He aha inā e hoʻi mai?
• He aha nā hoʻololi e pono iaʻu e hana i koʻu nohona?

He aha ka ʻokoʻa ma waena o ka Mycosis Fungoides a me ka Sézary Syndrome?

Ua lohe paha ʻoe i kēia mau inoa ʻelua ma mua. ʻO Mycosis fungoides kekahi ʻano lymphoma T-cell cutaneous e hoʻopilikia ana i ka ʻili. ʻO ia hoʻi, aia ia i loko o ka ʻohana like me ka maʻi Sézary. Hoʻoulu pū ia i kahi ʻulaʻula a ʻeleʻele paha ma ka ʻili. Eia naʻe, ʻo ka ʻokoʻa nui, ʻaʻole like me ka maʻi Sézary, ʻaʻohe nui o nā cell cancer (nā cell Sézary) e kahe ana i loko o ko lākou koko i ka poʻe me ka mycosis fungoides. Eia naʻe, i kekahi manawa hiki i ka mycosis fungoides ke lilo i mea koʻikoʻi a ulu a lilo i Sézary syndrome. No laila aia kahi pilina ma waena o nā mea ʻelua.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

He maʻi ʻaʻai laha ʻole ka Sézary Syndrome, ulu wikiwiki, e hoʻopilikia ana i ka ʻili a me ke koko. Hiki iā ia ke laha i nā wahi ʻē aʻe o ke kino. I ka wā mua, like paha nā hōʻailona me kahi maʻi ʻili maʻamau e like me ka eczema, akā e ʻoi aku ka wikiwiki o nā hōʻailona o ka Sézary Syndrome a ʻaʻole e nalowale maʻalahi. ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia ke ʻano, hiki i nā kauka ke mālama i kāu mau hōʻailona a pale i ka laha ʻana o ka maʻi mai ka ʻili a me ke koko i nā ʻōpū ʻē aʻe.

No laila, inā ʻike ʻoe i kekahi ʻano ʻē, mau, ʻeha nui, ʻulaʻula, nā kiko ʻeleʻele, a i ʻole ka mānoanoa o kou ʻili, he mea nui e ʻike i ke kauka, ʻoiai ke kauka dermatologist, i ka wā hikiwawe loa. ʻO ka hiki wawe ke ʻike ʻia, ʻo ka hiki wawe ke hoʻomaka i ka lāʻau. Hiki iā ia ke hana i kahi ʻokoʻa nui i kou ola.

ʻO ka maʻi Sézary, ka maʻi ʻili, ka lymphoma T-cell, nā hōʻailona ʻili, erythroderma, ka mālama ʻana i ka maʻi ʻaʻai, ka maneʻo ʻili