ʻO ka hōʻike ʻana o ka lymphoma T-cell cutaneous (CTCL)
ʻO ka ʻili (ʻili) T-cell lymphoma (CTCL) kahi ʻano kakaikahi o ka lymphoma non-Hodgkin (NHL).
ʻO nā pūnae T-mutated e neʻe i ka ʻili, kahi e hōʻike mua ai ʻo ia ma ke ʻano he ʻūhā. Hiki ke ʻeha a paʻakikī ke ʻike. ʻO ka hapa nui o nā lymphomas ʻili he molowa (ke ulu mālie) a noho i ka ʻāina (ma kahi like) i ka ʻili.
ano o ʻO ka lymphoma T-cell cutaneous (CTCL) he:
- ʻO Mycosis fungoides ʻO ia ka subtype maʻamau o CTCL, a ma kahi o 50% o nā hihia āpau o CTCL. Hiki i kēia subtype ke holo i kekahi manawa i nā ʻohana, akā kakaikahi loa kēia.
- ʻO ka lymphoma nui-cell nui anaplastic cutaneous he lymphoma molowa. Hiki i kēia ʻano subtype ke hoʻopili i nā kānaka o nā makahiki āpau, me nā keiki akā ʻoi aku ka maʻamau i ka hui 45-60-makahiki.
- Lymphomatoid papulosis he maʻi maʻi ʻole o ka ʻōnaehana pale. He mea mua kēia (pre-cancerous) no CTCL. Hiki ke loaʻa kēia maʻi i kēlā me kēia makahiki mai ka wā kamaliʻi a i ka makahiki waena.
- T-cell skin lymphoma ʻoi aku ka maʻamau o ka poʻe i loaʻa i ka transplant a i ʻole ka poʻe i loaʻa i ka maʻi HIV (human immunodeficiency virus).
ʻO wai ka mea i hoʻopilikia ʻia e ka cutaneous T-cell lymphoma (CTCL)?
ʻOi aku ka maʻamau o nā lymphomas T-cell Cutaneous (CTCL) i nā kāne ma mua o nā wahine. He mea maʻamau ka CTCL i nā poʻe maʻi kahiko akā pili pū kekahi i nā keiki. ʻO ka awelika o nā makahiki o ka maʻi maʻi he 55 mau makahiki a hiki ke komo nā kumu genetic i ka hoʻomohala ʻana i kekahi ʻano o ka lymphoma ʻili T-cell.
Nā hōʻailona o ka lymphoma T-cell cutaneous
Hoʻomaka ka hapa nui o nā lymphoma T-cell ma ke ʻano he ʻāpana maloʻo, a ʻeleʻele ka ʻili (ʻulaʻula maʻamau). Aia lākou ma ka ʻāʻī a i ʻole ma waena o ka pūhaka a me nā poʻohiwi (torso). Eia nō naʻe, hiki iā lākou ke ulu i nā wahi ʻē aʻe. Hiki paha i kēia mau ʻāpana ke ʻeha a hiki i nā hōʻailona ʻili ke komo pū kekahi:
- Nā ʻili o ka ʻili māmā a ʻeleʻele paha
- Nā ʻili o ka ʻili
- ʻO nā wahi ʻili paʻakikī a mānoanoa paha i kapa ʻia he plaques
- ʻO nā wahi paʻa liʻiliʻi liʻiliʻi o ka ʻili i kapa ʻia he papules
- ʻO nā ʻōhū nui aʻe o ka ʻili, i kapa ʻia nā nodules a i ʻole nā tumo, hiki ke haki i lalo (ulcerate) a me ka pehu.
- ʻO ka ʻulaʻula maʻamau o ka ʻili, hiki ke ʻeha nui, maloʻo a me ka scaly
- ʻO ka ʻili mānoanoa a māhā paha ma nā poho lima a i ʻole ka wāwae
Pehu lymph aka wahi ma ka ʻāʻī, ʻāʻī a i ʻole ka ʻāʻī paha.
B nā hōʻailona aia paha a hiki ke komo:
- Nā pōmele pō (ka lole moe a me ka moe moe)
- ʻO ke kuni mau
- ʻO ka pohō kaumahaʻole
He mea nui e haʻi i ke kauka e pili ana i nā hōʻailona āpau no ka mea hiki ke hoʻololi i ka hoʻoholo e pili ana i ke ʻano o ka mālama ʻana a me ka wā e hoʻomaka ai.
ʻO ka hōʻailona a me ke kau ʻana o CTCL
kekahi mau biopsies ili pono paha ma mua o ka hana ʻana i kahi hōʻailona CTCL. A nā lāʻau kanu he hana e wehe i a limu lymph a i ʻole nā mea ʻokoʻa ʻē aʻe e nānā iā ia ma lalo o ka microscope e ka mea maʻi maʻi e ʻike i ke ʻano o nā cell. Hiki ke hana ʻia ka biopsy ma lalo o ka anesthetic kūloko a i ʻole ma muli o ka ʻāpana o ke kino e biopsied. Hiki ke hana ʻia ka biopsy i kekahi o ʻekolu mau ala:
- Aspirate ka nila maikaʻi
- Biopsy kikine ʻehā
- ʻO ka biopsy node excision
An biopsy node excisional ʻo ia ka koho noiʻi maikaʻi loa, ʻoiai ʻo ia ka mea e hōʻiliʻili ai i ka nui o ka ʻiʻo e hiki ai ke hana i ka hoʻāʻo pono no kahi hōʻailona.
Pono ka hoʻomaʻamaʻa ʻana i ka hoʻoponopono pono ʻana o nā ʻike lapaʻau a me nā pathological e nā loea loea. Hiki i kekahi mau kauka ke komo i nā dermatologists, haematologists, radiotherapy oncologists, pathologists a me nā kauka lapaʻau kūikawā.
Ke kali nei i nā hopena hoʻāʻo hiki ke lilo i manawa paʻakikī. Hiki ke kōkua ke kamaʻilio me kou ʻohana, nā hoaaloha a i ʻole a kahu maʻi maʻi kūikawā.
Ka hoʻonohonoho ʻana o CTCL
I ka manawa a ʻike, o ka CTCL, pono nā ho'āʻo hou aku e ʻike i kahi ʻē aʻe o ke kino i pā ai ka lymphoma. Kapa ʻia kēia e hoʻokū nei.
Hana ʻia ka maʻi maʻi Sezary a me ka mycosis fungoides ma ke ʻano like. ʻO ka pae 1 a hiki i ka pae 4. Hoʻoholo ʻia nā pae e ka nui o ka ʻili i hoʻopili ʻia a inā he mau sela lymphoma maʻamau i loko o ke kahe koko. Ua wehewehe hou ʻia ke kahua ma nā ʻaoʻao pūnaewele no ka mycosis fungoides a me sezary syndrome.
Hoʻonohonoho ʻia nā lymphoma ʻili T-cell ʻē aʻe me kahi ʻōnaehana i kapa ʻia ʻo TNM. ʻO TNM ke kū nei no:
Tumour, Nodes a me Metastasis.
ka kūlana o kāu lymphoma e kōkua i kāu kauka e ʻike i ka lāʻau lapaʻau maikaʻi loa iā ʻoe.
He aha ka 'papa' o Lymphoma?
Hoʻohui pinepine ʻia nā Lymphomas e like me kekahi molowa or Kūpa'a. He lohi ka ulu ʻana o nā lymphoma maʻamau a ke ulu wikiwiki nei nā lymphoma koʻikoʻi. Ua kapa ʻia ka papa ʻo ka hana lapaʻau o ka lymphoma.
Ka hoʻopaʻa ʻana a me nā hoʻāʻo
ʻO nā scans a me nā hoʻāʻo e pono ai no ka hoʻonohonoho ʻana a ma mua o ka hoʻomaka ʻana o ka mālama ʻana e komo pū kekahi:
- ʻO ka ʻōmole helu Positron emoate (PET)
- Ka pākuʻina o ka tomography (CT)
- Biopsy iwi ʻōiwi
- Lumbar puncture & magnetic resonance imaging (MRI) - Inā kānalua ʻia ka lymphoma i loko o ka lolo a i ʻole ke kuamoʻo.
Hiki i nā maʻi ke hana i kekahi mau nā hoʻokolohua kumu ma mua o ka hoʻomaka ʻana o kekahi lapaʻau e nānā i nā hana o ke kino. Hoʻohana pinepine ʻia kēia mau mea i ka wā a ma hope o ka pau ʻana o ka mālama ʻana no ka loiloi inā ua pili ka lāʻau i ka hana o nā kino. I kekahi manawa pono e hoʻoponopono ʻia ka mālama ʻana a me ka mālama ʻana e kōkua i ka mālama ʻana i nā hopena ʻaoʻao. Hiki i kēia mau mea ke komo:
- Ke kaulike
- Nā ʻike koʻikoʻi (ke koko, ka mahana, a me ka helu pulse)
- ʻImi puʻuwai
- Kiʻi ʻāʻī
- Nā hoʻāʻo hanu
- Nā ho'āʻo koko
He manawa lōʻihi paha e hana ʻia ai nā biopsies a me nā hoʻāʻo e pono ai (he awelika o 1-3 mau pule), akā he mea nui i nā kauka ke loaʻa ke kiʻi piha o ka lymphoma a me ke olakino ākea o ka mea maʻi hana i nā hoʻoholo lapaʻau maikaʻi loa
Hana hou ʻia ka nui o ka hoʻāʻo ʻana i ka hoʻomaʻamaʻa ʻana a me ka hana o ke kino ma hope o ka mālama ʻana no ka nānā ʻana inā ua hana ka lapaʻau lymphoma a me ka hopena i loaʻa i ke kino.
ʻO ka wānana o ka lymphoma T-cell cutaneous (CTCL)
Hiki ke mālama maikaʻi ʻia ka CTCL me nā lāʻau lapaʻau o kēia manawa akā ʻo ka hapa nui o nā hihia i manaʻo ʻia he hiki ʻole ke hoʻōla. Eia naʻe, hiki ke mālama ʻia ka CTCL ma ke ʻano he maʻi maʻi a hiki i nā maʻi ke ola i nā ola maʻamau.
Lapaʻau ʻana i ka lymphoma T-cell cutaneous (CTCL)
Ke pau nā hopena a pau mai ka biopsy a me nā scan staging, e nānā ke kauka i kēia mau mea e hoʻoholo ai i ka lāʻau maikaʻi loa no ka mea maʻi. Ma kekahi mau keʻena maʻi 'aʻai, e hui pū ke kauka me kahi hui o nā loea e kūkākūkā i ka lāʻau lapaʻau maikaʻi loa a ua kapa ʻia kēia a hui multidisciplinary (MDT) halawai.
Noʻonoʻo nā kauka i nā kumu he nui e pili ana i ka lymphoma a me ke olakino maʻamau o ka mea maʻi e hoʻoholo ai i ka wā a me ka lāʻau e pono ai.
Hoʻokumu ʻia kēia ma:
- ʻO ka pae o ka lymphoma
- Nā hōʻailona (me ka nui a me kahi o ka lymphoma)
- Pehea ka hopena o ka lymphoma i ke kino
- makahiki
- Ka mōʻaukala olakino a me ke olakino maʻamau
- Ke ola kino a me ka noʻonoʻo o kēia manawa
- Nā makemake o ka poʻe maʻi
ka lapaʻau laina mua maʻamau no CTCL hiki ke komo:
ʻO ka lāʻau lapaʻau lymphoma T-cell mua
- ʻO nā corticosteroids (ke kumuhana a me ka waha)
- Interferon
- Methotrexate (Methoblastin)
- phototherapy
- PUVA- Psoralen Plus ultraviolet A (UVA) kukui
- ʻO ka lāʻau lapaʻau UVB
- ʻO ke kelepona
- Loaʻa paha iā ʻoe kekahi mau lāʻau lapaʻau ma ke ʻano he ʻāpana a ka ho'āʻo hoʻokolohua
ʻO ka lāʻau kiʻekiʻe o ka lāʻau T-cell lymphoma
- Brentuximab Vedotin (Adcetris)
- CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine a me prednisolone)
- ʻO nā corticosteroids (ke kumuhana a me ka waha)
- ʻO Photopheresis Extracorporal
- ʻO Gemcitabine (Gemzar)
- Nā mea hoʻopukapuka
- Methotrexate (Methoblastin)
- Pralatrexate (Folotyn)
- phototherapy
- ʻO ke kelepona
- Romidepsin (Istodax)
- Ka hoʻololi ʻana o ke kelepona (autologous a allogeneic)
- Vorinostat (Zolinza)
Kākoʻo Psychological he mea nui no hoi. Pono e mālama pinepine ʻia nā maʻi ʻili a me ka ʻeha ʻili.
Nā hopena maʻamau o ka lāʻau lapaʻau
Nui nā hopena ʻaoʻao like ʻole o ka lāʻau lapaʻau a pili kēia i ka lāʻau i hāʻawi ʻia. Hiki i ke kauka lapaʻau a/a i ʻole kahu maʻi maʻi ke wehewehe i nā hopena ʻaoʻao kikoʻī ma mua o ka mālama ʻana. ʻO kekahi o nā hopena ʻaoʻao maʻamau o ka lāʻau lapaʻau hiki ke komo:
- Anemia (lawe nā koko ʻulaʻula haʻahaʻa i ka oxygen a puni ke kino)
- ʻO Thrombocytopenia (nā platelets haʻahaʻa e kōkua i ke koko a me ka cotting)
- ʻO Neutropenia (ke koko keʻokeʻo haʻahaʻa e kōkua i ka pale ʻana)
- ʻO ka naʻi a me ka luaʻi
- ʻO nā pilikia o ka ʻōpū e like me ka constipation a i ʻole ka diarrhea
- ʻO ka luhi (ka luhi a me ka nele o ka ikehu
Pono ka hui lapaʻau, kauka, kahu maʻi maʻi a i ʻole lāʻau lapaʻau, e hāʻawi i ka ʻike e pili ana i:
- He aha ka lāʻau e hāʻawi ʻia
- He aha nā hopena maʻamau a hiki ke loaʻa no ka mālama ʻana
- He aha nā hopena ʻaoʻao āu e pono ai e hōʻike i ka hui lapaʻau
- He aha nā helu hoʻokaʻaʻike, a ma hea e hele ai inā pilikia 7 lā o ka pule a me 24 mau hola i kēlā me kēia lā
Malama hahai
Ke hoʻopau ʻia ka mālama ʻana, hana ʻia nā scan staging post treatment e nānā i ka maikaʻi o ka hana ʻana. E hōʻike ana nā scans i ke kauka inā loaʻa kahi:
- Pane piha (CR a ʻaʻohe hōʻailona o ka lymphoma i koe) a i ʻole
- Pane hapa (PR a i ʻole he lymphoma i kēia manawa, akā ua hoʻemi ʻia ka nui)
Inā maikaʻi nā mea a pau, e hana ʻia nā koho maʻamau no kēlā me kēia 3-6 mahina e nānā i kēia mau mea:
- E nānā i ka pono o ka lāʻau lapaʻau
- E nānā i nā hopena ʻaoʻao mai ka lāʻau lapaʻau
- E nānā i nā hopena hope loa mai ka mālama ʻana i ka manawa
- E nānā i nā hōʻailona o ka hoʻi hou ʻana o ka lymphoma
He mea koʻikoʻi nō hoʻi kēia mau koho i hiki i ka mea maʻi ke hāpai i kekahi hopohopo e pono ai lākou e kūkākūkā me ka hui lapaʻau. ʻO kahi hoʻokolohua kino a me nā hoʻāʻo koko he mau hoʻokolohua maʻamau no kēia mau koho. Ma waho aʻe o ka manawa ma hope o ka mālama ʻana e nānā i ka hana ʻana o ka mālama ʻana, ʻaʻole i hana pinepine ʻia nā scans ke ʻole he kumu no lākou. No kekahi poʻe maʻi hiki ke emi pinepine i ka manawa.
Hoʻoponopono hou ʻia a i ʻole ka hoʻoponopono ʻana i ka lymphoma T-cell cutaneous (CTCL)
Ma ke ʻano he kumu, mālama ʻia ka chemotherapy no ka hope o ka CTCL a inā nā mea maʻi hoʻi hou (hoʻi mai ka maʻi kanesa), hiki i nā lāʻau lapaʻau ke komo:
- ʻO ka lāʻau antibody me ka alemtuzumab a me brentuximab vedotin.
- ʻO ke kelepona
- Ke kuʻi ʻōnaʻi ʻōnaeho
Hoʻopaʻa ʻia ma lalo o ka hoʻokolokolo ʻana no ka lymphoma T-cell cutaneous (CTCL)
Nui nā lāʻau lapaʻau e hoʻāʻo ʻia nei i kēia manawa nā ho'āʻo hoʻokolohua a puni ka honua a ma Australia no nā poʻe maʻi me ka lymphoma hou i ʻike ʻia. Hiki ke komo i nā lāʻau lapaʻau hou no CTCL:
- Anti-CD47 monoclonal antibodies
- Carfilzomib (Kyprolis)
- Lenalidomide (Revlimid)
- Mogamulizumab (Poteligio)
He aha ka hana ma hope o ka mālama ʻana?
Nā hopena hope
I kekahi manawa a hopena ʻaoʻao hiki ke hoʻomau a ulu paha nā mahina a i ʻole mau makahiki ma hope o ka pau ʻana o ka mālama ʻana. Kapa ʻia kēia he hopena hope.
Hoʻopau i ka mālama ʻana
He manawa paʻakikī kēia no nā poʻe he nui a hiki ke pili kekahi o nā hopohopo maʻamau i:
- Helu
- ʻO ke ʻano noʻonoʻo
- Ola kino
- 'ana ma
- Hana, aʻo, a me nā hana kaiaulu
ʻO ke olakino a me ke olakino
ʻO ke ola olakino, a i ʻole kekahi mau loli ola maikaʻi ma hope o ka mālama ʻana he mea kōkua nui ia ma hope o ka pau ʻana o ka mālama ʻana. ʻO ka hana ʻana i nā hoʻololi liʻiliʻi e like me ka ʻai ʻana a me ka hoʻonui ʻana i ke olakino hiki ke hoʻomaikaʻi i ke olakino a me ka maikaʻi a kōkua i ke kino e ola hou. He nui nā hoʻolālā mālama ponoʻī hiki ke kōkua i ka wā ho'ōla.
