Sézary Syndrome: Nā Hōʻailona a me nā Manaʻo Ola

Anter

• He aha nā hōʻailona a me nā hōʻailona?
• ʻO ke kiʻi o erythroderma
• ʻO wai ka pilikia?
• He aha ke kumu?
• Pehea ia ʻike?
• Pehea e hoʻokumu ʻia ai ka maʻi Sézary?
• Pehea e mālama ʻia ai?
• ʻO Psoralen a me UVA (PUVA)
• Extracorporeal photochemotherapy / photopheresis (ECP)
• Lapaʻau hoʻomākala
• ʻO Chemotherapy
• Immunotherapy (biologic Inc)
• Nā hoʻāʻo hoʻokolohua
• Outlook

He aha ka maʻi Sézary?

ʻO Sézary Syndrome kahi ʻano o ka lymphoma T-cell cutaneous. ʻO nā hunaola Sézary kekahi ʻano o ke keʻokeʻo koko keʻokeʻo. I kēia kūlana, hiki ke loaʻa nā hunaola maʻi ʻino i ke koko, ka ʻili, a me nā pūnana lymph. Hiki ke laha pū ka maʻi ʻaʻai i nā kino ʻē aʻe.

ʻAʻole maʻamau ka maʻi Sézary, akā hana ia i 3 a 5 mau pākēneka o ka cutaneus T-cell lymphomas. Lohe paha paha ʻoe i kapa ʻia ʻo Sézary erythroderma a i ʻole Sézary's lymphoma.

He aha nā hōʻailona a me nā hōʻailona?

ʻO ka hōʻailona hōʻailona o Sézary Syndrome ʻo ia ka erythroderma, kahi ʻulaʻula, a me ka ʻehaʻeha hiki ke uhi a hiki i ka 80 pākēneka o ke kino. ʻO nā hōʻailona ʻē aʻe a me nā ʻōuli e like me:

• pehu o ka ʻili
• nā pā o ka ʻili a me nā tumors
• hoʻonui ʻia nā lymph node
• ka mānoanoa o ka ʻili ma nā poho lima a me nā wāwae
• abnormalities o nā manamana lima a me nā manamana wāwae
• maka o lalo e huli i waho
• poho lauoho
• pilikia i ka hoʻoponopono ʻana i ka mahana o ke kino

Hiki i ka maʻi Sézary ke hana i kahi spleen i hoʻonui ʻia a i ʻole nā ​​pilikia me nā akemāmā, ke ake, a me nā ʻōpū o ka gastrointestinal. ʻO ka loaʻa ʻana o kēia ʻano kūpilikiʻi o ka maʻi ʻaʻai e hoʻonui i ka makaʻu o ka hoʻomohala ʻana i nā maʻi ʻē aʻe.

ʻO ke kiʻi o erythroderma

ʻO wai ka pilikia?

Hiki i kekahi ke hoʻomohala i ka maʻi Sézary, akā ʻo ka mea paha e hoʻopili i ka poʻe ma mua o 60 mau makahiki.

He aha ke kumu?

ʻAʻole maopopo ke kumu kūpono. Akā ʻo ka hapanui o ka poʻe me Sézary Syndrome he chromosomal abnormalities i loko o ka DNA o nā hunaola maʻi ʻaʻai, ʻaʻole naʻe i nā keʻa olakino. ʻAʻole nā ​​hoʻoilina hoʻoilina kēia, akā hoʻololi i hiki ke ola.

ʻO nā mea maʻamau maʻamau ka nalo ʻana o ka DNA mai nā chromosome 10 a me 17 a i ʻole nā ​​hoʻohui DNA i nā chromosome 8 a me 17. Eia nō naʻe, ʻaʻole maopopo leʻa kēia mau mea maʻamau i ke kanesa.

Pehea ia ʻike?

ʻO kahi hoʻokolokolo kino ʻana i kou ʻili e hoʻomaopopo ai i ke kauka i ka hiki ke loaʻa ʻo Sézary Syndrome. Hiki i nā hoʻokolohua diagnostics ke hoʻopili i nā hoʻokolohua koko e ʻike ai i nā māka (antigens) ma ka ʻili o nā hunaola i loko o ke koko.

E like me nā maʻi ʻino ʻē aʻe, ʻo kahi biopsy ke ala ʻoi loa e hiki ai i kahi hōʻailona. No kahi biopsy, e lawe ke kauka i kahi laʻana o ka ʻili o ka ʻili. E nānā kahi pathologist i ka laʻana ma lalo o kahi microscope e nānā no nā hunaola maʻi ʻaʻai.

Hiki ke biopsied ʻia nā lymph node a me ka iwi iwi. ʻO nā hoʻokolohua kiʻi ʻana, e like me CT, MRI, a me nā scan PET, hiki ke kōkua i ka hoʻoholo inā ua laha ka maʻi ʻaʻai i nā lymph node a i ʻole nā ​​mea ʻē aʻe.

Pehea e hoʻokumu ʻia ai ka maʻi Sézary?

Hōʻike ke kahua i ka lōʻihi o ka palahalaha ʻana o ka maʻi ʻaʻai a me nā koho ʻoi loa.Hoʻokomo ʻia ʻo Sézary Syndrome penei:

• 1A: Ma lalo o 10 pākēneka o ka ʻili i uhi ʻia i nā ʻulaʻula a i ʻole nā ​​pā.
• 1B: Ma mua o 10 pākēneka o ka ʻili ʻulaʻula.
• 2A: Pili kekahi nui o ka ʻili. Hoʻonui ʻia nā lymph aka, ʻaʻole naʻe he maʻi ʻaʻa.
• 2B: Hoʻokahi aʻoi aku paha nā tumors i ʻoi aku ma mua o 1 kenimika i hana ʻia ma ka ʻili. Hoʻonui ʻia nā lymph aka, ʻaʻole naʻe he maʻi ʻaʻa.
• 3A: ʻUlaʻula ka hapa nui o ka ʻili a loaʻa paha i nā tumors, nā peleka, a i ʻole nā ​​pākuʻi. ʻO nā lymph nodes kahi maʻamau a hoʻonui ʻia paha, akā ʻaʻole maʻi maʻi maʻi. ʻAʻohe paha i loko o ke koko kekahi mau huna Sézary.
• 3B: Aia nā leona ma ka hapanui o ka ʻili. Hiki a hoʻonui ʻia paha nā lymph nodes. ʻO ka helu o nā huna Sézary i ke koko he haʻahaʻa.
• 4A (1): Uhi nā ʻili i nā ʻili i nā ʻāpana o ka ʻili. Hiki a hoʻonui ʻia paha nā lymph nodes. ʻO ka helu o nā huna Sézary i ke koko kiʻekiʻe.
• 4A (2): Uhi nā ʻili i nā ʻili i nā ʻāpana o ka ʻili. Nui nā lymph node a hoʻonui ʻia nā hunaola ma lalo o ka nīnaninau microscopic. ʻAʻole paha i loaʻa i nā hunaola Sézary i loko o ke koko.
• 4B: Uhi nā ʻili i nā ʻili i nā ʻāpana o ka ʻili. He maʻamau a maʻa ʻole paha nā lymph nodes. ʻAʻole paha i loaʻa i nā hunaola Sézary i loko o ke koko. Ua pālahalaha nā hunaola Lymphoma i nā mea ʻē aʻe a i ʻole nāʻaʻaʻa.

Pehea e mālama ʻia ai?

He nui nā kumu i hoʻohuli i kahi ʻoi aku ka maikaʻi no ʻoe. Aia i waena o lākou nā:

• kahua ma ke kuhi
• makahiki
• nā pilikia olakino ʻē aʻe

ʻO nā aʻe aʻe kekahi o nā lapaʻau ʻana no ka maʻi Sézary.

ʻO Psoralen a me UVA (PUVA)

ʻO kahi lāʻau i kapa ʻia psoralen, kahi e ʻohiʻohi ai i nā hunaola maʻi ʻaʻai, i loko o ke aa. Hoʻomaʻamaʻa ʻia ke hōʻike ʻia i ke kukui ultraviolet A (UVA) i kuhikuhi ʻia i kou ʻili. Luku kēia hana i nā hunaola maʻi ʻaʻai me ka hōʻemi iki ʻana i nāʻiʻo olakino.

Extracorporeal photochemotherapy / photopheresis (ECP)

Ma hope o ka loaʻa ʻana o nā lāʻau lapaʻau kūikawā, lawe ʻia kekahi mau huna koko mai kou kino. Mālama ʻia lākou me ke kukui UVA ma mua o ka hoʻoiho hou ʻia ʻana i kou kino.

Lapaʻau hoʻomākala

Hoʻohana ʻia nā kukuna X ikaika nui e luku i nā hunaola maʻi ʻaʻai. I ka radiation radiation waho, hoʻouna kahi mīkini i nā kukuna i nā wahi i kuhi ʻia o kou kino. Hiki i ka radiation radiation ke hōʻoluʻolu i ka ʻeha a me nā hōʻailona ʻē aʻe pū kekahi. Hoʻohana ka radiation radiation electron (TSEB) radiation radiation i kahi mīkini radiation waho e kuhikuhi i nā electrons i ka ʻili o kou kino holoʻokoʻa.

Hiki iā ʻoe ke loaʻa iā UVA a me ultraviolet B (UVB) radiation radiation e hoʻohana ana i kahi kukui kūikawā i kau ʻia i kou ʻili.

ʻO Chemotherapy

ʻO Chemotherapy kahi lapaʻau ʻōnaehana kahi e hoʻohana ʻia ai nā lāʻau ikaika e pepehi i nā hunaola maʻi ʻaʻai a i ʻole hoʻokū i kā lākou mahele. Loaʻa kekahi mau lāʻau chemotherapy i ka palapala pila, a ʻo nā mea ʻē aʻe pono e hāʻawi intravenous.

Immunotherapy (biologic Inc)

Hoʻohana ʻia nā lāʻau e like me interferons e hoʻoulu i kāu ʻōnaehana pale ponoʻī e kaua i ka maʻi ʻaʻai.

ʻO nā lāʻau i hoʻohana ʻia e mālama iā Sézary Syndrome me:

• alemtuzumab (Campath), kahi monoclonal antibody
• bexarotene (Targretin), kahi retinoid
• brentuximab vedotin (Adcetris), kahi mea hoʻopili lāʻau hoʻowalewale
• chlorambucil (Leukeran), kahi lāʻau chemotherapy
• corticosteroids e hōʻoluʻolu i nā ʻōuli o ka ʻili
• cyclophosphamide (Cytoxan), kahi lāʻau chemotherapy
• denileukin difitox (Ontak), mea hoʻololi pane biologic
• gemcitabine (Gemzar), kahi chemotherapy antimetabolite
• interferon alfa a i ʻole interleukin-2, stimulants immune
• lenalidomide (Revlimid), kahi mea kūpoki angiogenesis
• liposomal doxorubicin (Doxil), kahi lāʻau chemotherapy
• methotrexate (Trexall), kahi antimetabolite chemotherapy
• pentostatin (Nipent), kahi chemotherapy antimetabolite
• romidepsin (Istodax), kahi mea hoʻopuka histone deacetylase
• vorinostat (Zolinza), kahi mea hoʻopuka histone deacetylase

Kau kauka paha i kau kauka o nā lāʻau a i ʻole nā ​​lāʻau a me nā hoʻoponopono ʻē aʻe. E hoʻokumu ʻia kēia ma ke kahua o ka maʻi ʻaʻai a pehea ʻoe e pane ai i kahi ʻano lapaʻau.

Hoʻokomo ʻia paha ka mālama ʻana no ka pae 1 a me 2:

• corticosteroids kumuhana
• retinoids, lenalidomide, histone deacetylase nā mea kāohi
• PUVA
• pāhawewe me TSEB a i ʻole UVB
• ʻo biologic therapy e ia iho a i ʻole me ka hoʻōla ʻana i ka ʻili
• chemotherapy kumuhana
• chemotherapy ʻōnaehana, hui pū ʻia paha me ka ʻili o ka ʻili

Hiki ke mālama ʻia nā pae 3 a me 4 me:

• corticosteroids kumuhana
• lenalidomide, bexarotene, histone deacetylase nā mea kāohi
• PUVA
• ʻO ECP wale nō a i ʻole me TSEB
• pāhawewe me TSEB a i ʻole UVB a me UVA pāhawewe
• ʻo biologic therapy e ia iho a i ʻole me ka hoʻōla ʻana i ka ʻili
• chemotherapy kumuhana
• chemotherapy ʻōnaehana, hui pū ʻia paha me ka ʻili o ka ʻili

Inā ʻaʻole e hana hou ʻia nā mālama ʻana, hiki i kahi transplant cell stem ke koho.

Nā hoʻāʻo hoʻokolohua

Ke hoʻomau nei ka noiʻi ʻana i nā mālama ʻana no ka maʻi ʻaʻai, a ʻo nā hoʻokolohua hoʻokolohua kekahi ʻāpana o kēlā hana. I kahi hoʻokolohua hoʻokolohua, hiki iā ʻoe ke komo i nā therapies wailana honua i loaʻa ʻole ma kahi ʻē aʻe. No ka ʻike hou aku e pili ana i nā hoʻokolohua hoʻokolohua, e nīnau i kāu oncologist a i ʻole kipa iā ClinicalTrials.gov.

Outlook

ʻO Sézary Syndrome kahi maʻi ʻaʻai ikaika loa. Me ka lapaʻau ʻana, hiki paha iā ʻoe ke hoʻolohi i ka holomua o ka maʻi a i ʻole ke hele i ke kala. Akā hiki i kahi ʻōnaehana pale nāwaliwali ke waiho iā ʻoe nāwaliwali i ka maʻi maʻi a me nā maʻi ʻē aʻe.

He 2 a 4 mau makahiki ke ola ʻana, akā ke hoʻomaikaʻi nei kēia helu me nā lāʻau hou.

E ʻike i kāu kauka a hoʻomaka i ka lāʻau i ka wā hiki ke hōʻoia i ka nānā pono loa.