Nā mea e pono ai ʻoe e ʻike e pili ana i ka maʻi Castleman

Ua loaʻa anei iā ʻoe kahi puʻupuʻu, ka mea a mākou e kapa nei he "puʻupuʻu," ma kou ʻāʻī, armpit, a i ʻole groin? I kekahi manawa, pehu nā puʻupuʻu e like me kēia ke loaʻa iā ʻoe kahi anu maʻamau a i ʻole kekahi ʻano maʻi. He mea maʻamau kēlā. Akā inā loaʻa iā ʻoe nā puʻupuʻu pehu e like me kēia me ke kumu ʻole, no ka manawa lōʻihi, a ke manaʻo nei hoʻi ʻoe i ka luhi a me ka emi ʻana o ke kaumaha, pono ʻoe e hopohopo iki. I kēia lā, e kamaʻilio mākou e pili ana i kahi maʻi kakaikahi e hiki ke kānalua ʻia i kēlā mau manawa. ʻO ia ka maʻi Castleman.

I ka ʻōlelo maʻalahi, he aha ka maʻi Castleman?

E noʻonoʻo e like ko kākou kino me kahi ʻāina. A laila ʻo ko kākou ʻōnaehana pale kino ka pūʻali koa e pale aku i kēlā ʻāina. Ke komo kahi germ (ʻenemi) i loko o ke kino mai waho mai, hele kēia pūʻali koa i ke kaua a lanakila i kēlā ʻenemi. A pau ke kaua, hoʻi ka pūʻali koa i ka hale koa a noho mālie. ʻO kēia ka mea e hana ʻia i loko o ke kino o ke kanaka olakino.

Akā, me kekahi me ka maʻi ʻo Castleman, ʻokoʻa ia. ʻO ka mea e hana ʻia ma ʻaneʻi, ʻo ka pūʻali koa i hele i ke kaua, ʻo ia hoʻi ka ʻōnaehana pale, ʻaʻole i mālie ma hope o ka pau ʻana o ke kaua. Noho ikaika lākou. Me he mea lā ua mākaukau mau lākou no ke kaua. Hoʻoulu kēia i ka mumū lōʻihi i loko o ke kino. Hiki i kēia ke hōʻino i ko kākou mau kino.

I kēia manawa, ʻo ko mākou mau lymph nodes, kēlā mau mea liʻiliʻi, ua like ia me nā kahua koa. Hana lākou i ka nui o ka hana, e like me ke kānana ʻana i nā germs a me ka mālama ʻana i nā cell immune. I kēia manawa, no ka mea e hana mau ana ka ʻōnaehana immune, ke hana mau nei kēia mau lymph nodes. ʻO ka hopena, hoʻomaka nā cell i loko o lākou e ulu nui. ʻO ia ke kumu e pehu ai nā lymph nodes i ka maʻi Castleman. ʻAʻole ia he pehu wale nō, akā ke kumu o nā loli i loko o nā ʻiʻo o kēlā mau lymph nodes.

He aha nā ʻano nui o ka maʻi Castleman?

Ua māhele ʻia ka maʻi Casselman i ʻelua ʻano nui. He ʻokoʻa loa nā hōʻailona a me nā lāʻau lapaʻau no kēlā me kēia. No laila, he mea nui e hoʻomaopopo pono i kēia mau ʻano ʻelua.

Aia kekahi mau ʻano like ʻole o ka maʻi Castleman multicentric (MCD)?

ʻAe, ua māhele hou ʻia ʻo MCD i kekahi mau ʻano like ʻole. Hana ʻia kēia hoʻokaʻawale ʻana ma muli o ke kumu a me nā kūlana ʻē aʻe e kū pū me ia.

• MCD e pili ana iā POEMS: He maʻi koko kakaikahi loa ʻo POEMS. I kekahi manawa hiki ke hana pū ʻia ʻo MCD me POEMS.
• ʻO ka MCD e pili ana iā HHV-8: Hoʻokumu ʻia kēia e ka maʻi me kahi maʻi i kapa ʻia ʻo Human Herpes Virus-8 (HHV-8). He mea maʻamau kēia ʻano i ka poʻe i loaʻa i ka maʻi HIV a i ʻole ka poʻe i hoʻopilikia ʻia ka pale ʻana no nā kumu ʻē aʻe.
• ʻO ka Idiopathic MCD (iMCD): ʻO ke ʻano lapaʻau " idiopathic " ʻo ia hoʻi "ʻaʻohe kumu i loaʻa." Loaʻa i ka hapa nui o nā maʻi MCD kēia ʻano. ʻO ke ʻano kēia, ʻaʻole i ʻike ʻia ke kumu pololei.

Ua māhele hou ʻia kēia ʻano iMCD i ʻekolu mau ʻāpana liʻiliʻi:

1. iMCD e pili ana me TAFRO: ʻO TAFRO kahi inoa i loaʻa mai ka hui pū ʻana o kekahi mau hōʻailona. ʻO ia hoʻi, ʻo Thrombocytopenia (nā platelets koko haʻahaʻa), Anasarca (ka pehu ʻana o ke kino me ka wai), Fiva (ke kuni), Renal dysfunction (nāwaliwali o ka hana puʻupaʻa) a me Organomegaly (ka hoʻonui ʻia o ke akepaʻa a i ʻole ka spleen) nā hōʻailona e ʻike ʻia ma kēia.

2. iMCD me ka idiopathic plasmacytic lymphadenopathy (iMCD-IPL): I kēia ʻano, hiki ke piʻi aʻe ka helu platelet. Hiki nō hoʻi ke hana nui ʻia o nā antibodies e nā keʻena koko keʻokeʻo.

3. iMCD, ʻaʻole i kuhikuhi ʻia ma kekahi ʻano ʻē aʻe (iMCD-NOS): ʻO nā maʻi iMCD ʻaʻole pili i nā kūlana TAFRO a ʻaʻohe kumu kikoʻī ʻē aʻe e hiki ke loaʻa i loko o kēia māhele.

He aha nā hōʻailona o kēia maʻi?

ʻOkoʻa nā hōʻailona ma muli o ke ʻano o ka maʻi Castleman āu e loaʻa ai.

ʻO ka maʻi Unicentric Casselman (UCD) ʻaʻohe ona hōʻailona . ʻO ka hōʻailona wale nō he puʻupuʻu lymph pehu (adenoid). I kekahi manawa, ʻike wale ʻia nā hōʻailona (e.g., ʻeha, pōkole o ka hanu) ke kaomi ka puʻupuʻu pehu i kekahi ʻano ʻē aʻe.

Eia nō naʻe, i ka maʻi Castleman multicentric (MCD), ʻoi aku ka maopopo o nā hōʻailona. Ma waho aʻe o nā glands pehu, hiki iā ʻoe ke ʻike:

• Kuni pinepine
• Manaʻo nui i ka luhi a me ka luhi ʻole no ke kumu ʻole (hiki ke loaʻa kēia ma muli o ka anemia)
• Ka hou nui ʻana i ka pō
• Ka luaʻi a me ka luaʻi
• Pohō kaumaha i manaʻo ʻole ʻia
• Ka pehu ʻana o nā wāwae, nā kuʻekuʻe wāwae, a i ʻole ka ʻōpū
• Ka nui o ka spleen (splenomegaly) a i ʻole ke akepaʻa i hoʻonui ʻia (hepatomegaly)
• ʻO ka ʻeha ma nā lima a me nā wāwae (peripheral neuropathy)

No ke aha i loaʻa ai kēia maʻi? Aia kekahi mau mea pilikia?

ʻO kaʻoiaʻiʻo, ʻaʻole i loaʻa i nā kānaka ʻepekema kahi kumu kūikawā no ka maʻi Castleman unicentric (UCD) .

Ua pili ka maʻi Multicentric Castleman (MCD) me ka maʻi HHV-8 i ʻōlelo ʻia ma luna. Eia nō naʻe, ke noiʻi nei nā mea noiʻi inā paha he mau maʻi ʻē aʻe, nā hoʻololi ʻana o ka gene, a i ʻole nā ​​​​​​pane autoimmune ke kuleana no ke ʻano i ʻike ʻole ʻia o iMCD.

I ka wā e pili ana i nā kumu pilikia, ʻaʻohe kumu pilikia i ʻike ʻia no UCD a me iMCD. Eia nō naʻe, inā loaʻa iā ʻoe ka maʻi HIV a i ʻole kekahi ʻōnaehana pale ʻē aʻe i nāwaliwali, ʻoi aku kou pilikia no ka hoʻomohala ʻana i ka MCD e pili ana iā HHV-8.

ʻOiai hiki ke ulu kēia maʻi i nā kānaka o kēlā me kēia makahiki, ʻoi aku ka maʻamau o ia i nā kānaka ma waena o 30 a me 60 mau makahiki .

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

Hiki i ka maʻi Castleman ke hoʻonui iki i kou pilikia o ka hoʻomohala ʻana i nā kūlana ʻē aʻe, ʻoiai nā maʻi kanesa , e like me ka lymphoma, kahi maʻi kanesa o ka ʻōnaehana lymphatic.

Eia kekahi, ʻo ka poʻe me UCD e pilikia ana i ka loaʻa ʻana o kahi maʻi ʻili kakaikahi akā koʻikoʻi i kapa ʻia ʻo paraneoplastic pemphigus (PNP). ʻOi aku ka nui o ka pilikia o ka poʻe me MCD i ka loaʻa ʻana o nā maʻi like ʻole. Inā ʻaʻole mālama ʻia, hiki i kēia mau maʻi ke hōʻino i nā kino a me ka make.

Akā, mai hopohopo, e nānā mau ana kāu kauka i kēia kūlana a hana i nā hana e pono ai e kōkua i ka pale ʻana i kēia mau pilikia i ka hiki.

Pehea e ʻike pololei ai ke kauka i kēia maʻi?

Ma muli o ke ʻano like o nā hōʻailona o ka maʻi Castleman me nā maʻi ʻē aʻe, e like me ke anu maʻamau a i ʻole ka maʻi palū, e nānā mua ke kauka i nā maʻi maʻamau. A laila wale nō lākou e kānalua ai i ka maʻi Castleman a hana i nā hoʻokolohua kikoʻī.

Hana nui ʻia nā hoʻokolohua ma lalo nei:

• Nā hoʻokolohua lab: E nānā ʻia kou koko ma lalo o kahi microscope e nānā i nā ʻano ʻē i ka helu o kāu mau cell koko a me nā ʻano ʻē aʻe. Inā kānalua ʻia ka MCD e pili ana iā HHV-8, hiki ke hana ʻia kahi hoʻokolohua HIV.
• Nā hoʻāʻo kiʻi: Hiki i nā scan CT a me nā scan PET ke kōkua i ke kuhikuhi ʻana i kahi o nā lymph node pehu i loko o ke kino. Hiki iā lākou ke nānā i ka hoʻonui ʻia ʻana o nā ʻōpū e like me ke akepaʻa a me ka spleen.
• ʻO ka biopsy o nā kiko lymph: ʻO kēia wale nō ke ala maopopo loa e hōʻoia ai i ka maʻi . Pili kēia i ka lawe ʻana i kahi ʻāpana liʻiliʻi loa o ka ʻiʻo mai ka kiko lymph pehu a nānā iā ia ma lalo o kahi microscope. A laila hiki i kēia ke haʻi maopopo inā he mau loli kikoʻī o nā cell e pili ana me ka maʻi Casselman.

He aha nā lāʻau lapaʻau no kēia?

ʻOkoʻa loa nā ʻano lapaʻau ma muli o ke ʻano o ka maʻi.

Ka Lapaʻau ʻana o ka Maʻi Casselman Unicentric (UCD)

ʻO ka lāʻau lapaʻau nui a kūleʻa loa no UCD, ʻo ia ka wehe ʻoki ʻana i ka node lymph i hoʻopilikia ʻia . I ka hapanui o nā hihia, ua ho'ōla loa ʻia ka maʻi ma hope o kēia ʻoki ʻana.

I kekahi manawa, inā nui ka puʻupuʻu, hāʻawi ʻia ka radiation therapy a i ʻole ka immunotherapy ma mua o ke ʻoki ʻana e hoʻēmi iā ia a maʻalahi hoʻi ka wehe ʻana.

Inā aia ka puʻupuʻu ma kahi i hiki ʻole ke wehe ʻia me ke ʻoki ʻana, a ʻaʻohe ou hōʻailona, ​​​​hiki i kāu kauka ke hoʻoholo ʻaʻole e mālama iā ia a nānā wale iā ia. Eia nō naʻe, no kekahi mea ʻaʻole i koho ʻia no ke ʻoki ʻana a loaʻa mau nā hōʻailona, ​​​​hiki ke hāʻawi ʻia ka lāʻau like me MCD.

Ka mālama ʻana i ka maʻi Casselman multicentric (MCD)

No ka mea, he maʻi ka MCD e laha ana i nā ʻāpana he nui o ke kino, ʻaʻole hiki ke ho'ōla ʻia me ke ʻoki kino a i ʻole ka radiation therapy. No laila, hoʻohana ʻia nā lāʻau lapaʻau ʻē aʻe. Aia ka lāʻau lapaʻau i ka loaʻa ʻana o ka HHV-8 iā ʻoe a me ke koʻikoʻi o ka maʻi.

ʻO nā lāʻau lapaʻau ma lalo nei e hāʻawi nui ʻia:

• Corticosteroids: Kōkua kēia mau lāʻau lapaʻau i ka kaohi ʻana i nā hōʻailona ma ka hoʻemi ʻana i ka mumū i loko o ke kino.
• Nā lāʻau lapaʻau Chemotherapy: Hana kēia ma ke kāohi ʻana i ka wikiwiki o ka ulu ʻana o nā cell i loko o ka ʻōnaehana lymphatic. Hoʻohana pinepine ʻia ʻo Rituximab no ka MCD e pili ana iā HHV-8.
• Immunotherapy: Hana kēia mau lāʻau lapaʻau ma ka hōʻoluʻolu ʻana i ka ʻōnaehana pale kino ikaika o kou kino. ʻO Siltuximab (Sylvant®) wale nō ka lāʻau i ʻāpono ʻia no iMCD.
• Nā lāʻau lapaʻau antiviral: Inā loaʻa iā ʻoe ka MCD e pili ana iā HHV-8, hiki ke hāʻawi ʻia kēia mau lāʻau iā ʻoe e kāohi i ka maʻi HIV a i ʻole HHV-8.

I ka manawa hea e pono ai iaʻu ke ʻike i ke kauka?

Inā ʻike ʻoe i kahi puʻupuʻu a puʻupuʻu paha ma kou ʻāʻī, armpit, a i ʻole groin hiki iā ʻoe ke manaʻo, e haʻi pono i kāu kauka e pili ana i ia mea.

Eia kekahi, inā mau nā hōʻailona i ʻōlelo ʻia ma kēia ʻatikala, e like me ke kuni i wehewehe ʻole ʻia, ka pohō kaumaha, a me ka luhi nui, no kekahi mau pule, e ʻimi pono i ke aʻo lapaʻau.

Hiki ke ola me kēia maʻi?

ʻO ka pane i kēia nīnau e pili ana i ke ʻano o Casselman āu.

Hiki i ke kanaka me UCD ke manaʻo i nā hopena maikaʻi loa . Ke wehe ʻia ka puʻupuʻu ma ke ʻoki ʻana, hoʻōla pinepine ʻia ka maʻi. Ma hope o ka mālama ʻana, ʻaʻole kēia e hoʻopilikia i kou ola maʻamau.

He paʻakikī iki ka wānana no ka poʻe me MCD . ʻOkoʻa ia ma muli o ke ʻano a me ke koʻikoʻi o ka maʻi. No kekahi poʻe, hiki i nā hōʻailona ke lilo koke i mea koʻikoʻi a hoʻoweliweli i ke ola. No kekahi poʻe, hiki i nā hōʻailona ke lōʻihi ka manawa, akā aia ka manaʻolana. Ma waena o 65% a me 75% o ka poʻe i ʻike ʻia me MCD e ola ana no nā makahiki ʻelima . Ke holomua nei kēia me nā lāʻau lapaʻau hou.

ʻAʻohe pane maʻalahi a kūpono i nā mea āpau no ka maʻi Casselman. E hilinaʻi ia i kou ʻike, ke ʻano o ka maʻi āu, a inā he mau maʻi ʻē aʻe kāu, e like me ka HIV, TAFRO, a i ʻole POEMS. I kekahi manawa, e lawa ka ʻoki kino a me ka nānā mau ʻana i ke kauka. A i ʻole pono paha ʻoe e loaʻa i nā lāʻau lapaʻau he nui i kou ola. No laila, ʻoi aku ka maikaʻi o ke kamaʻilio ākea me kāu kauka e pili ana i kou kūlana, kāu lāʻau lapaʻau, a me kou wā e hiki mai ana.