ʻO ka Hepatosplenic gamma delta T-cell lymphoma he maʻi kanesa o ka ʻōnaehana lymphatic a ulu mai nā T lymphocytes (ke koko keʻokeʻo e hakakā ana i ka maʻi). ʻO kēia ʻano lymphoma he mea kakaʻikahi loa, paʻakikī (ke ulu wikiwiki) a hiki ke paʻakikī ke mālama.
Ka nānā 'ana o ka hepatosplenic gamma delta T-cell lymphoma
He kakaikahi loa ka Hepatosplenic gamma delta T-cell lymphoma Kūpa'a (ke ulu wikiwiki) T-cell Non-Hodgkin lymphoma (NHL). Hiki ke loaʻa kēia lymphoma ma ka ʻāʻī, ate, koko a me ka iwi iwi. Ua ʻike ʻia ʻo ia he lymphoma 'extra nodal' (loaʻa ma waho o nā lymph nodes).
Hepatosplenic gamma delta T-cell lymphoma ua hui pinepine ia ma lalo o ka umbrella term peripheral T-cell lymphoma (PTCL). He pūʻulu kēia o nā lymphoma T-cell non-Hodgkin koʻikoʻi. ʻO lākou ka 7% o nā hihia NHL āpau a me 10% o nā hihia lymphoma āpau.
Hepatosplenic gamma delta T-cell lymphoma (HSGDTCL) i kapa ʻia ʻo:
- Hepato: 'o ia ho'i 'pili i ke ake'
- Splenic: 'o ia ho'i 'pili i ka spleen'
Hepatosplenic gamma delta T-cell lymphoma pohihihi keia a paʻakikī ke mālama. ʻO ka lāʻau lapaʻau no kēia subtype chemotherapy. Nui ka poʻe hoʻi hou (hoʻi hou mai ka lymphoma) ma hope o ka lāʻau lapaʻau mua. Inā loaʻa ke kala ʻana (ʻaʻohe hōʻailona o ka maʻi kanesa), a ka hoʻololi ʻana i ke ʻano o ke ʻano o ke ʻano allogeneic ʻōlelo pinepine ʻia no kahi manawa o ho'ōla (ʻaʻole kūpono i nā mea a pau).
ʻO wai ka mea i hoʻopilikia ʻia e ka hepatosplenic gamma delta T-cell lymphoma
Hepatosplenic gamma delta T-cell lymphoma i loaʻa pinepine i ka poʻe ʻōpio, kahi o ka awelika o ka makahiki o ka maʻi ma kahi o 30 mau makahiki. He mea maʻamau kēia subtype i nā kāne.
ʻO ka poʻe i ʻoi aku ka nui o ka pilikia o ka hoʻomohala ʻana i ka hepatosplenic gamma delta T-cell lymphoma hiki ke komo:
- ʻO ka mōʻaukala o ka maʻi ʻāʻī o ka ʻōpū, ʻoiai inā ma ka systemic immunosuppressive therapy
- ʻO ka mōʻaukala o ka hoʻohana mau ʻana i ka immunosuppression (ka hoʻololi ʻana o ke kino paʻa a i ʻole ka hoʻololi ʻana o ka iwi iwi)
Nā hōʻailona o ka hepatosplenic gamma delta T-cell lymphoma
Hepatosplenic gamma delta T-cell lymphoma Kūpa'a lymphoma, ʻo ia hoʻi ke ulu wikiwiki nei. Hoʻomaka wikiwiki nā hōʻailona maʻamau i nā pule maʻamau no ka hōʻike ʻana i nā hōʻailona.
ʻO nā hōʻailona mua maʻamau o ka hepatosplenic gamma delta T-cell lymphoma ʻo ia ka puʻupuʻu a i ʻole ka hōʻoluʻolu i kou ʻōpū ('ōpū). ʻAʻole hiki i kēia lymphoma ke kumu maʻamau i ka pehu ʻana o nā kīwī lymph e like me nā lymphomas ʻē aʻe, no ka mea, ʻo ia ka 'extra nodal' (loaʻa ma waho o nā lymph nodes).
Hiki i nā ʻōuli ʻē aʻe ke:
- ʻO ka pehu ʻana o ke akepaʻa, e hoʻohūhū ai ʻoe, e hoʻonui ai i ka wai i loko o kou ʻōpū. A i ʻole e melemele kou mau maka keʻokeʻo (jaundice)
- ʻO ka pehu o ka ʻōpū, hiki ke hōʻeha i ka ʻeha ma hope o kou mau iwi ʻaoʻao ma ka ʻaoʻao hema, a i ʻole e piha koke ʻoe i kou ʻai ʻana.
- Anemia (helu koko ʻulaʻula haʻahaʻa)
- ʻO ka Thrombocytopenia (nā platelet haʻahaʻa)
- Neutropenia (helu neutrophil haʻahaʻa)
B nā hōʻailona ʻo ia nā mea a nā kauka i kapa ai i kēia mau hōʻailona a hiki ke komo:
- ʻO ke kuni mau (ʻoi aku i ka pō >38C),
- ʻO ka pohō kaumahaʻole
- Nā pōmele pō (ʻo kēlā lole moe a me nā moe moe)
ʻO ka hōʻailona o ka hepatosplenic gamma delta T-cell lymphoma
A nā lāʻau kanu koi mau no a ʻike, o ka hepatosplenic gamma delta T-cell lymphoma. ʻO ka biopsy kahi kaʻina hana no ka wehe ʻana i kahi ʻāpana kino ʻole. A laila, e nānā ke kanaka pathologist ma lalo o ka microscope 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.
Hepatosplenic gamma delta T-cell lymphoma hiki ke paʻakikī ke ʻike. Pono ka biopsy o ke ake a i ʻole ka spleen no ka hōʻoia ʻana.
Ke kali nei i nā hualoaʻa hiki ke lilo i manawa paʻakikī. Hiki ke kōkua ke kamaʻilio me kou ʻohana, nā hoaaloha a i ʻole a kahu maʻi maʻi kūikawā.
ʻO ke kau ʻana o ka hepatosplenic gamma delta T-cell lymphoma
I ka manawa a ʻike, no ka hepatosplenic gamma delta T-cell lymphoma i hana ʻia, pono nā hoʻāʻo hou e ʻike i kahi ʻē aʻe o ke kino i pā ai ka lymphoma. Kapa ʻia kēia ʻano hoʻokūkū. ʻO ke kau ʻana o ka lymphoma e kōkua i ke kauka e hoʻoholo i ka lāʻau maikaʻi loa no ka mea maʻi.
Ka hoʻopaʻa ʻana a me nā hoʻāʻo
ʻO nā scans a me nā hoʻāʻo e pono ai no ka hoʻonohonoho ʻana ma mua a ma hope o ka hoʻomaka ʻana o ka mālama ʻana hiki ke komo pū me:
- ʻO ke kiʻi kiʻi kiʻi kiʻi Positron emission (Pet).
- Ka pākuʻina o ka tomography (CT)
- Biopsy iwi ʻōiwi
- Hoʻokokoko hou aku
Hiki i nā maʻi ke loaʻa 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, nā hoʻāʻo a me nā scans (he awelika o 1-3 mau pule), akā he mea nui i nā kauka ke loaʻa i ke kiʻi piha o ka lymphoma a me ke olakino ākea o ka mea maʻi. i mea e hana ai i na olelo hooholo lapaau maikai 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 hepatosplenic gamma delta T-cell lymphoma
He kakaikahi loa a paʻakikī ke mālama ʻia ʻo Hepatosplenic gamma delta T-cell lymphoma. ʻO ka lāʻau lapaʻau mua no kēia subtype ʻo ia ka lāʻau lapaʻau. He nui nā kānaka hoʻi hou (hoʻi hou mai ka lymphoma) ma hope o ka lāʻau lapaʻau mua. Inā loaʻa ke kala ʻana (ʻaʻohe hōʻailona o ka maʻi kanesa), a ka hoʻololi ʻana i ke ʻano o ke ʻano o ke ʻano allogeneic hiki ke ʻōlelo ʻia no kahi manawa e ola ai (ʻaʻole kūpono no kēlā me kēia kanaka).
Ka mālama ʻana i ka hepatosplenic gamma delta T-cell lymphoma
Ke pau nā hualoaʻa mai ka biopsy a me nā scans 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 hui multidisciplinary (MDT).
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
Pono ke kauka a me ke kahu maʻi maʻi e wehewehe i ka hoʻolālā papahana i ka maʻi a me nā hopena ʻaoʻao. He paʻakikī ka mālama ʻana i ka lymphoma delta hepatosplenic gamma delta T, a he mea maʻamau ka hoʻi hou ʻana o kēia lymphoma (hoʻi mai).
ka maʻamau maʻamau laina mua chemotherapy Hiki i nā regimen i hoʻohana ʻia:
- kua (cyclophosphamide, doxorubicin, vincristine a me prednisolone)
- CHOEP (CHOP me ka hoʻohui o ka etoposide)
- hau (ifosfamide, carboplatin a me etoposide)
- IVAC (ifosfamide, etoposide a me ka cytarabine)
- Ke komo ʻana i ka hoʻokolohua lapaʻau
- Kemotherapy a ukali ʻia e ka hoʻololi ʻana i nā pūnaewele allogeneic.
- Kemotherapy a ukali ʻia e ka hoʻololi ʻana i nā pūnaewele pūʻali autologous
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 ʻāpana koko ʻulaʻula haʻahaʻa i ka oxygen a puni ke kino)
- Thrombocytopenia (nā platelets haʻahaʻa e kōkua ana i ke koko a me ka cotting)
- ʻO Neutropenia (kōkoʻo ke koko keʻokeʻo haʻahaʻa me ka palekana)
- ʻO ka naʻi a me ka luaʻi
- ʻO nā pilikia o ka ʻōpū e like me ka constipation a i ʻole ka maʻi maʻi
- 'Oloke (ka luhi a me ka nele o ka ikehu)
- Hoemi i ka momona
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 helu kelepona a me kahi e hele ai inā pilikia 7 lā o ka pule a me 24 mau hola i kēlā me kēia lā
Mālama i ka momona
Hiki i kekahi mau lapaʻau no ka lymphoma ke hoʻemi i ka momona a ʻoi aku paha kēia me kekahi mau protocol chemotherapy (hui pū ʻana o nā lāʻau lapaʻau) a me ka chemotherapy kiʻekiʻe i hoʻohana ʻia ma mua o ka hoʻololi ʻana i ke kelepona. Hoʻonui ka radiotherapy i ke pelvis i ka liʻiliʻi o ka momona. Hiki i kekahi mau lāʻau lapaʻau antibody ke hoʻopilikia i ka momona, akā ʻaʻole maopopo kēia.
Pono ke kauka e aʻo inā pili paha ka hānau ʻana a i ʻole e mālama ʻia ka hānai ma mua o ka hoʻomaka ʻana o ka mālama ʻana. Pono nā maʻi e nīnau mau e pili ana mālama mālama ʻana ma mua o ka hoʻomaka ʻana o ka mālama ʻana inā he mea nui kēia iā lākou.
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.
Hoʻihoʻi hou i ka hoʻokele refractory o ka hepatosplenic gamma delta T-cell lymphoma
No kekahi poʻe, ʻoi aku ka maikaʻi o ka mālama mua ʻana, a ʻaʻole hoʻi hou ka lymphoma ma hope o ka mālama ʻana. Eia naʻe, no kekahi poʻe, hoʻi hou ka lymphoma (kau hou) a i ʻole i nā hihia koʻikoʻi ʻaʻole i pane i ka lāʻau lapaʻau mua (hale hōʻoluʻolu). Inā loaʻa kēia, aia kekahi mau lāʻau lapaʻau e hiki ke holomua.
ka laina lua or lin koluHiki i nā lāʻau lapaʻau ke komo:
- ʻO ka lāʻau lapaʻau kiʻekiʻe a me ka hoʻololi ʻana i ke ʻano o nā pūnaewele kino
- ʻO ka lāʻau lapaʻau kiʻekiʻe a me ka hoʻololi ʻana o nā pūnaʻi kumu allogeneic.
- ʻO ka chemotherapy hui
- ʻO nā lāʻau olaola e like me ka romidepsin, brentuximab a i ʻole pralatrexate
- ʻO ke kelepona
- Tria kinol komo
Lapaʻau ma lalo o ka hoʻokolokolo ʻana no ka hepatosplenic gamma delta T-cell lymphoma
Nui nā lāʻau lapaʻau e hoʻāʻo ʻia nei i nā hoʻokolohua lapaʻau a puni ka honua no nā poʻe maʻi me ka lymphoma hou a me ka hoʻi hou ʻana. No ka papa inoa o nā lāʻau lapaʻau i kēia manawa e noiʻi ʻia nei no ka hepatosplenic gamma delta T-cell lymphoma e loaʻa:
- Nā monemona monoclonal
- Nā mea hoʻopaneʻe Proteasome
- ʻO ka mea hoʻopaneʻe histone deacetylase (HDAC)
ʻO nā ʻano subtypes T-cell lymphoma (PTCL).
No ka ʻike hou aku e pili ana i nā ʻano subtypes e ʻike ma lalo nei:
- ʻO Lymphoma T-cell pilipili - ʻaʻole i kuhikuhi ʻia (PTCL-NOS)
- Angioimmunoblastic T-cell lymphoma (AITL)
- Anaplastic large cell lymphoma (ALCL)
- ʻO lymphoma T-cell ʻōpū
- ʻO Enteropathy-Type T-Cell Lymphoma (EATL)
- Monomorphic epitheliotropic intestinal lymphoma (MEITL)
- Nasal NK/T-Cell Lymphoma (NKTCL)
- Hepatosplenic gamma delta T-cell lymphoma
- ʻO Lymphoma T-cell Cutaneous (CTCL)
- Sezary Syndrome
- Leukemia/Lymphoma (ATLL) makua
- T-cell lymphoblastic lymphoma (LL)
He aha ka hana ma hope o ka mālama ʻana?
Hoʻopau i ka mālama ʻana
ʻO ka hoʻopau ʻana i ka mālama ʻana he manawa paʻakikī ia no nā poʻe he nui, ʻoiai lākou e hoʻoponopono hou i ke ola ma hope o ka mālama ʻana. ʻAʻole paha e hoʻomaka kekahi poʻe e ʻike i kekahi o kēia mau pilikia no nā pule a i ʻole mau mahina ma hope o ka pau ʻana o ka mālama ʻana i ka maʻi kanesa, ke hoʻomaka nei lākou e noʻonoʻo i ko lākou ʻike a ʻaʻole manaʻo ua hoʻopau lākou, ke hoʻomaka nei lākou e noʻonoʻo i kā lākou ʻike a hana paha. ʻaʻole manaʻo e ola koke ana lākou e like me ka mea e pono ai. Hiki ke pili kekahi o nā pilikia 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.
