He aha ka maʻi Castleman? E kamaʻilio maʻalahi kākou e pili ana!

Ua ʻike anei ʻoe e loaʻa ana kēia mau puʻupuʻu liʻiliʻi ma kou ʻāʻī a i ʻole nā ​​​​​​armpits? I kekahi manawa hana lākou i ka wā e loaʻa ai iā ʻoe ke anu a i ʻole ka maʻi flu. Akā, ʻaʻole kēia e pili ana i kahi mea maʻalahi e like me kēlā. He kūlana kakaikahi kēia akā he mea nui e makaʻala. Kāhea mākou iā ia ʻo Castleman Disease .

I ka ʻōlelo maʻalahi, ʻo ka Maʻi Castleman kahi kūlana e lilo ai ka ʻōnaehana pale o ko mākou kino i mea hana nui . E noʻonoʻo, i ka wā e komo ai kahi huakino e hoʻoulu ai i ka maʻi i loko o ko mākou kino, e hakakā kā mākou ʻōnaehana pale me ia e like me ke koa e hele ana i ke kaua. Ke ho'ōla ʻia ka maʻi, e noho mālie hou kēlā koa. Akā i loko o ke kanaka me ka Maʻi Castleman, ke 'hoʻāla' mau nei kēia ʻōnaehana pale. Hoʻoulu kēia i ka mumū lōʻihi i loko o ke kino, hiki ke hōʻino i ko mākou mau kino.

I kēia manawa, hana ko kākou mau lymph nodes - ʻo ia mau puʻupuʻu liʻiliʻi ka mea a kākou e kapa aku nei iā lākou - e like me ke kānana. Kānana ʻia nā germs a me nā cell immune āpau ma o lākou. No laila, no ka mea e hana mau ana ka ʻōnaehana immune, ke hana nei kēia mau lymph nodes i ka manawa keu. A laila ulu nui nā cell i loko o lākou, a pehu nā lymph nodes. ʻAʻole wale kēia pehu ʻana, akā i ka maʻi Castleman, aia kekahi mau loli i loko o nā ʻiʻo o kēia mau lymph nodes.

He maʻi laha ʻole kēia. No ka laʻana, ma ʻAmelika Hui Pū ʻIa, he 4,300 a 5,200 wale nō mau hihia hou i ʻike ʻia i kēlā me kēia makahiki. No laila, ke aʻo nui nei nā kauka e pili ana i kēia. Eia nō naʻe, inā ua ʻike ʻia ʻoe me kēia maʻi, e loaʻa iā ʻoe nā lāʻau lapaʻau kikoʻī e pili pono ana iā ʻoe.

He aha nā ʻano nui o ka maʻi Castleman?

ʻElua ʻano nui o kēia maʻi.

ʻO ka maʻi Castleman Unicentric (UCD)

ʻO ka maʻi Unicentric Castleman (UCD) ka wā e hoʻonui ʻia ai hoʻokahi a i ʻole kekahi mau lymph node ma kekahi wahi o kou kino . E noʻonoʻo wale i kou ʻāʻī, a i ʻole kou mau armpits wale nō. Ma kahi o ʻekolu hapahā o nā maʻi Castleman e hāʻule ana i loko o kēia māhele.

ʻO ka maʻi Castleman Multicentric (MCD)

ʻO ka maʻi Multicentric Castleman (MCD) kahi maʻi kahi e pehu ai nā lymph nodes ma nā wahi he nui o ke kino, ma mua o hoʻokahi wale nō. I mea e pololei ai, ua hoʻokaʻawale hou nā kauka i kēia ʻano MCD i:

• MCD e pili ana iā POEMS: He maʻi koko kakaikahi loa ka POEMS. I kekahi manawa, hiki ke hana pū ʻia ka POEMS me MCD.
• ʻO ka MCD e pili ana iā HHV-8: Hoʻokumu ʻia kēia ʻano e ka maʻi me kahi maʻi i kapa ʻia ʻo Human Herpes Virus-8 (HHV-8) . Inā loaʻa iā ʻoe ka maʻi HIV a i ʻole ua palupalu kou pale ʻana i ka maʻi, ʻoi aku ka nui o kou loaʻa ʻana i kēia ʻano MCD.
• MCD o ke kumu i ʻike ʻole ʻia (Idiopathic MCD - iMCD):ʻO kēia ke ʻano maʻamau o MCD. ʻO ke ʻano o 'Idiopathic' ʻaʻole ʻike ʻia ke kumu pololei.

I kēia manawa, ʻekolu mau ʻāpana o kēia kumu i ʻike ʻole ʻia ʻo iMCD:

• ʻO ka iMCD e pili ana me ka maʻi TAFRO: I kekahi manawa hiki ke hele pū ʻia ka iMCD me kahi maʻi i kapa ʻia ʻo TAFRO syndrome. Ua loaʻa ka inoa TAFRO mai nā leka mua o kāna mau hōʻailona. ʻO kēia nā: Thrombocytopenia (nā platelets koko haʻahaʻa), Anasarca (pehu ʻana o ke kino me ka wai), Fiva , Renal (kidney) dysfunction, a me Organomegaly (hoʻonui ʻia o ka spleen a i ʻole ke akepaʻa).
• ʻO iMCD me ka Idiopathic Plasmacytic Lymphadenopathy (iMCD-IPL): Ma kēia ʻano, hiki ke kiʻekiʻe nā platelets. Hiki i kāu mau keʻokeʻo ke hana i nā antibodies he nui loa.
• ʻAʻole i kuhikuhi ʻia ʻo iMCD (iMCD-NOS): ʻAʻole ʻike ʻia ke kumu kikoʻī o kēia ʻano, i kapa ʻia ʻo iMCD-NOS, a ʻaʻole pili ia iā TAFRO.

He aha nā hōʻailona o ka maʻi Castleman?

ʻOkoʻa nā hōʻailona o ka maʻi Castleman ma muli o ke ʻano āu e loaʻa ai. I ka hapanui o nā hihia, inā loaʻa iā ʻoe ka maʻi Castleman unicentric (UCD), ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona nui. ʻO nā lymph node pehu paha ka hōʻailona wale nō āu e ʻike ai. Eia nō naʻe, i kekahi manawa hiki ke loaʻa nā hōʻailona inā kaomi kahi lymph node pehu i kahi ʻano ʻē aʻe kokoke.

Eia nō naʻe, inā loaʻa iā ʻoe ka MCD, ʻoi aku ka maopopo o nā hōʻailona. Ma waho aʻe o nā lymph nodes pehu, ʻike paha ʻoe i nā hōʻailona e like me:

• kuni
• Luhi (hiki i kēia ke hōʻailona o ka anemia)
• Ke hou nei i ka pō
• Ka luaʻi a me ka luaʻi
• ʻO ka pohō kaumaha me ke kumu ʻole
• ʻO ka pehu ʻana i kou mau 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)

He aha ke kumu o ka maʻi Castleman?

ʻAʻole maopopo ke kumu kikoʻī o ka maʻi Castleman unicentric (UCD), akā ua ʻike ʻia ka maʻi me kahi virus i kapa ʻia ʻo HHV-8 he kumu ia i ka ulu ʻana o ka maʻi Castleman multicentric (MCD).

Ke noiʻi nei nā kānaka ʻepekema i nā kumu ʻē aʻe o UCD a me iMCD. No ka laʻana, ke nānā nei lākou i nā maʻi ʻē aʻe ma mua o HHV-8, nā hoʻololi ʻana o ka gene , a me nā pane autoimmune .

ʻO wai ka mea i pilikia nui i ka loaʻa ʻana o kēia maʻi?

ʻAʻole i ʻike ʻia nā kumu pilikia kikoʻī no ka UCD a i ʻole iMCD. Eia nō naʻe, inā loaʻa iā ʻoe ka maʻi HIV a i ʻole kekahi maʻi ʻē aʻe e hoʻonāwaliwali ai i kāu ʻōnaehana pale, ua piʻi aʻe kou pilikia o ka hoʻomohala ʻana i ka MCD e pili ana iā HHV-8.

Hiki ke loaʻa ka maʻi Castleman i kēlā me kēia makahiki, akā ʻoi aku ka nui o ka poʻe i loaʻa i ka maʻi ma waena o 30 a me 60 mau makahiki .

He aha nā pilikia e hiki ke loaʻa mai ka maʻi Castleman?

Hoʻonui ka maʻi Castleman i kou pilikia o ka loaʻa ʻana o ka maʻi kanesa, ʻoiai ka lymphoma .

Eia kekahi, ʻoiai he mea kakaikahi loa, aia ka poʻe me UCD i ka pilikia o ka loaʻa ʻana o kahi maʻi koʻikoʻi i kapa ʻia ʻo Paraneoplastic Pemphigus (PNP) . Hoʻonui pū ka MCD i kou pilikia o ka loaʻa ʻana o nā maʻi e hiki ke hōʻino i kou mau kino. Hiki i kēia hōʻino ke hoʻoweliweli i ke ola inā ʻaʻole mālama pono ʻia.

Mai hopohopo, e nānā mau ana kāu kauka i kou kūlana a kōkua iā ʻoe e pale i ia mau pilikia i ka wā hiki.

Pehea e ʻike ai nā kauka i ka maʻi Castleman?

Hiki ke like nā hōʻailona o ka maʻi Castleman me nā maʻi maʻamau ʻē aʻe, e like me ka maʻi palū a i ʻole ke anu. No laila e kāpae mua kāu kauka i kēlā mau kūlana. A laila, e hana lākou i nā hoʻokolohua e ʻike ai i ka maʻi Castleman a hoʻoholo i ke ʻano.

Hiki i nā hoʻokolohua a me nā kaʻina hana ke komo pū me:

• Nā hoʻāʻo lab: E nānā kāu kauka i ka helu o kāu mau cell koko no nā ʻano ʻē aʻe a me nā hōʻailona microscopic ʻē aʻe o ka maʻi Castleman. Hiki iā lākou ke kauoha i kahi hoʻāʻo HIV . Inā loaʻa iā ʻoe ka MCD e pili ana iā HHV-8, hiki ke maikaʻi kāu hoʻāʻo HIV.
• Nā hoʻokolohua kiʻi: Hiki i nā hoʻokolohua e like me nā scan CT a me nā scan PET ke kōkua i ka ʻike ʻana i nā lymph node pehu i loko o kou kino, a me ka nānā ʻana i nā hōʻailona ʻē aʻe o ka maʻi Castleman, e like me ka ate i hoʻonui ʻia a i ʻole ka spleen.
• ʻO ka biopsy o nā kīʻaha lymph: ʻO ke ala wale nō e ʻike pono ai inā loaʻa iā ʻoe ka maʻi Castleman, ʻo ia ka loaʻa ʻana o kahi biopsy. Pili kēia i ka lawe ʻana i kahi ʻāpana liʻiliʻi o ka ʻiʻo mai kahi kīʻaha lymph a nānā iā ia ma lalo o kahi microscope. E hoʻoholo kēia inā he mau hōʻailona o ka maʻi.

Pehea e mālama ʻia ai ka maʻi Castleman?

ʻOkoʻa ka mālama ʻana no ka maʻi Castleman ma muli o ke ʻano o kou maʻi.

Ka Lapaʻau ʻana o ka Maʻi Castleman Unicentric (UCD)

ʻO ke ʻano nui o ka lāʻau lapaʻau no ka UCD, ʻo ia ka wehe ʻana i nā lymph node i hoʻopilikia ʻia ma ke ʻoki kino . I kekahi manawa, pono paha ʻoe i ka radiation therapy a i ʻole ka immunotherapy ma mua o ke ʻoki kino. Hoʻemi kēia mau lāʻau lapaʻau i nā ulu ʻana i loko o nā lymph node, e maʻalahi ai ka wehe ʻana.

Akā inā ʻaʻole hiki i nā kauka lapaʻau ke wehe i ka lymph node i hoʻopilikia ʻia, a ʻaʻohe ou hōʻailona, ​​​​hiki i kāu kauka ke nānā iā ia ma kahi o ka mālama ʻana. Inā loaʻa iā ʻoe nā hōʻailona a ʻaʻole hiki ke hana ʻoki kino, hiki iā lākou ke ʻōlelo i nā lāʻau lapaʻau no MCD.

Ka mālama ʻana i ka maʻi Castleman multicentric (MCD)

ʻOi aku ka paʻakikī o ka mālama ʻana i ka MCD ma mua o ka UCD. No ka mea ua laha a puni ke kino, ʻaʻole hoʻohana pinepine ʻia ke ʻoki kino a i ʻole ka radiation therapy. Akā, pili ka mālama ʻana i ka loaʻa ʻana o ka maʻi HHV-8 iā ʻoe a me ke koʻikoʻi o ka maʻi.

Hiki i kāu kauka ke hoʻohana i nā lāʻau lapaʻau e like me:

• Corticosteroids: Kōkua kēia mau lāʻau i ka hōʻemi ʻana i ka mumū a hāʻawi i ka hōʻoluʻolu mai nā hōʻailona.
• Nā lāʻau lapaʻau Chemotherapy: Hoʻomalu nā lāʻau lapaʻau Chemotherapy i ka ulu nui ʻana o nā cell i loko o kāu ʻōnaehana lymphatic. ʻO Rituximab kahi lāʻau i kuhikuhi pinepine ʻia no ka MCD e pili ana iā HHV-8.
• Immunotherapy: Pili kēia lāʻau i ka hoʻohana ʻana i nā mea e like me nā monoclonal antibodies . Kōkua kēia i ka hōʻoluʻolu ʻana i ka pane pale o kou kino. ʻO Siltuximab (Sylvant®) wale nō ka lāʻau i ʻāpono ʻia e ka U.S. Food and Drug Administration (FDA) no iMCD.
• Nā lāʻau antiviral: Inā loaʻa iā ʻoe ka MCD e pili ana iā HHV-8, pono paha ʻoe i nā lāʻau antiviral e mālama ai i ka maʻi HIV a i ʻole HHV-8.

Āhea wau e ʻike ai i ke kauka?

Inā ʻike ʻoe i kahi puʻupuʻu a i ʻole kahi ulu ʻana ma kou ʻāʻī, armpit, a i ʻole groin, e ʻike koke i ke kauka . Eia kekahi, inā loaʻa iā ʻoe kekahi o nā hōʻailona ʻē aʻe o ka maʻi Castleman i ʻōlelo ʻia ma luna nei ʻaʻole i hoʻomaikaʻi ma hope o kekahi mau pule, e haʻi i kāu kauka e pili ana iā lākou.

Pehea ka lōʻihi e hiki ai iā ʻoe ke ola me kēia maʻi?

No ka hapa nui o ka poʻe me UCD, maikaʻi loa ka prognosis, a i ʻole ka nānā ʻana. Hiki i ka wehe ʻana i nā lymph nodes i hoʻopilikia ʻia ke ho'ōla i ka maʻi. Me ka mālama ʻana, ʻaʻole pinepine ka UCD e hoʻopilikia i ka manaʻolana ola.

Akā, ʻo ka nānā ʻana no ka poʻe me MCD e pili ana i ke ʻano o ka MCD i loaʻa iā lākou a me ke koʻikoʻi o ka maʻi. No kekahi poʻe, hiki i nā hōʻailona ke ulu koke a lilo i mea hoʻoweliweli i ke ola. No kekahi poʻe, hiki i nā hōʻailona ke lōʻihi ka manawa. Ma waena o 65% a me 75% o ka poʻe i ʻike ʻia me MCD e ola mau ana ma hope o ʻelima mau makahiki . Me nā lāʻau lapaʻau hou, aia ka manaʻolana e hoʻomaikaʻi kēia kūlana.

ʻAʻohe pane maʻalahi i ka wā e pili ana i ka maʻi Castleman. E hilinaʻi kou ʻike i ke ʻano o ka maʻi Castleman āu. Hiki i nā kūlana ʻē aʻe, e like me ka HIV, a i ʻole nā ​​​​kūlana pili, e like me TAFRO a me POEMS, ke hoʻopilikia i kou ʻike. I kekahi mau hihia, ʻo ke ʻoki kino a me ka nānā pono ʻana paha nā mea e pono ai. A i ʻole, pono paha ʻoe i nā ʻano lāʻau like ʻole i kou ola e pale aku i nā pilikia. E nīnau i kāu kauka pehea e hoʻopilikia ai kāu hōʻailona i kāu lāʻau a me kou nānā ʻana.

No laila, he aha nā mea nui e pono ai kākou e hoʻomanaʻo mai kēia?

• He maʻi kakaikahi ka maʻi Castleman akā hiki ke koʻikoʻi i ka ʻōnaehana pale kino kahi e hana nui ai kā mākou ʻōnaehana pale kino.
• ʻElua ʻano nui: UCD a me MCD. I ka UCD, pehu nā lymph nodes ma kahi hoʻokahi, ʻoiai ma MCD, pehu lākou ma nā wahi he nui.
• ʻOkoʻa nā hōʻailona. I kekahi manawa ʻaʻohe hōʻailona iki, he puʻupuʻu pehu wale nō. A i ʻole he mau hōʻailona e like me ke kuni, ka luhi, a me ka pohō kaumaha.
• ʻAʻole maopopo mau nā kumu, akā ua pili ka maʻi HHV-8 i ka MCD. ʻOi aku ka nui o ka pilikia o ka poʻe me ka HIV.
• Hiki i ka ʻike mua ʻana a me ka mālama kūpono ke alakaʻi pinepine i nā hopena maikaʻi. No laila, inā ʻike ʻoe i kahi puʻupuʻu ʻē, pehu, a i ʻole nā ​​​​hōʻailona ʻē aʻe ma kou kino, e ʻimi koke i ke kōkua lapaʻau. ʻO ka mea nui loa, ʻo ia ka noho ʻike a mai hopohopo.

ʻO ka maʻi Castleman, nā Lymph Nodes, Lymphadenopathy, ʻōnaehana pale, UCD, MCD, HHV-8, nā hōʻailona, ​​​​​​lāʻau lapaʻau