Māʻi Castleman

ʻO ka maʻi Castleman kahi hui o nā maʻi maʻamau e pili ana i nā lymph node e ulu nui ana, i kapa ʻia ʻo nā lymph node nui, a me kahi ākea o nā hōʻailona. ʻO ke ʻano maʻamau loa o ka maʻi e pili ana i hoʻokahi lymph node nui. ʻO kēia lymph node ma ka umauma a i ʻole ka ʻāʻī maʻamau, akā hiki ke loaʻa ma nā wahi ʻē aʻe o ke kino pū kekahi. Kīaʻia kēia ʻano o ka maʻi he unicentric Castleman disease (UCD).

Hoʻopili ka multicentric Castleman disease (MCD) i nā ʻāpana he nui o nā lymph node nui, nā hōʻailona inflammatory a me nā pilikia me ka hana o nā loko. Aia he ʻekolu ʻano o ka MCD:

• HHV-8-associated MCD. Hoʻopili kēia ʻano i ka human herpes virus type 8, i kapa ʻia ʻo HHV-8, a me ka human immunodeficiency virus (HIV).
• Idiopathic MCD. ʻAʻole maopopo ke kumu o kēia ʻano. Kīaʻia kēia he HHV-8-negative MCD.

ʻO ke ʻano koʻikoʻi loa o kēia ʻano o ka MCD i ʻike ʻia ʻo iMCD-TAFRO. Loaʻa i kēia kūlana kona inoa mai nā hōʻailona e hana ai.

• POEMS-associated MCD. Hoʻopili kēia ʻano i kahi kūlana ʻē aʻe i kapa ʻia ʻo POEMS syndrome. ʻO ka POEMS syndrome kahi maʻi koko maʻamau e hōʻino i nā aʻalolo a hoʻopili i nā ʻāpana ʻē aʻe o ke kino.

Idiopathic MCD. ʻAʻole maopopo ke kumu o kēia ʻano. Kīaʻia kēia he HHV-8-negative MCD.

ʻO ke ʻano koʻikoʻi loa o kēia ʻano o ka MCD i ʻike ʻia ʻo iMCD-TAFRO. Loaʻa i kēia kūlana kona inoa mai nā hōʻailona e hana ai.

I kekahi manawa, hiki i nā kānaka ke loaʻa 2-3 lymph node nui a me nā hōʻailona māmā ʻaʻole kūpono i nā kūlana diagnostic no ka MCD. Hiki i kēia poʻe ke loaʻa kahi maʻi ʻē aʻe, a i ʻole hiki iā lākou ke loaʻa ka subtype o ka maʻi Castleman i wehewehe hou ʻia i kapa ʻia ʻo oligocentric Castleman disease. He maʻamau kēia subtype.

Hoʻololi a me ka nānā ʻana e hilinaʻi ana i ke ʻano o ka maʻi Castleman āu e loaʻa nei. Hiki ke mālama maikaʻi ʻia ka unicentric Castleman disease, ʻo ia ke ʻano e pili ana i hoʻokahi lymph node nui wale nō, me ka ʻoki kino.

ʻAʻole maopopo ka mālama maikaʻi loa no ka oligocentric Castleman disease, kahi e pili ana i kekahi mau lymph node nui a loaʻa nā hōʻailona palena, akā manaʻo ʻia e like me ka mālama ʻana no ka unicentric Castleman disease.

ʻOiai ʻaʻole nā ​​​​kānaka a pau me ka MCD e pane i ka mālama mua, aia nā lāʻau lapaʻau e hana ana e mālama i ka HHV-8-associated MCD a me ka idiopathic MCD.

He nui ka poʻe me ka maʻi Castleman unicentric ʻaʻole i ʻike i kekahi hōʻailona a i ʻole nā ​​hōʻailona. Hiki ke loaʻa ka lymph node i hoʻonui ʻia i ka wā o kahi hoʻokolohua kino a i ʻole kahi hoʻokolohua kiʻi no kahi pilikia ʻē aʻe. Hiki i kekahi poʻe me ka maʻi Castleman unicentric ke loaʻa nā hōʻailona a me nā hōʻailona i ʻike pinepine ʻia i ka maʻi Castleman multicentric. ʻO kēia mau mea paha: Fever. Pau ke kaumaha me ka ho'āʻo ʻole. Luhi. Hoʻoheheʻe i ka pō. Pehu. Hoʻonui ʻia ke ake a i ʻole ka spleen. He haʻahaʻa ka helu o nā punahele ʻulaʻula, i kapa ʻia hoʻi he anemia. Nui a haʻahaʻa paha nā helu platelet. Nui aʻe nā pae creatinine e hana ana ma muli o ka hana ʻole pono ʻana o nā puʻupaʻa. Nui aʻe nā pae o nā antibodies i ʻike ʻia he immunoglobulins. Haʻahaʻa nā pae o ka protein koko i kapa ʻia ʻo albumin. ʻO nā hōʻailona o ke ʻano ʻoi aku ka nui o ka idiopathic MCD i kapa ʻia ʻo iMCD-TAFRO: He haʻahaʻa ka helu platelet, i kapa ʻia hoʻi he thrombocytopenia. Pehu a me ka wai i loko o ke kino, i ʻike ʻia ʻo anasarca. Fever a i ʻole ka nui o ka C-reactive protein, kahi mea hōʻailona o ka mumū. Reticulin fibrosis, kahi i nānā ʻia e ka lawe ʻana i kahi laʻana o ka iwi ʻiwi. Pehu o nā kino, i kapa ʻia hoʻi ʻo organomegaly. Inā ʻike ʻoe i kahi lymph node i hoʻonui ʻia ma ka ʻaoʻao o kou ʻāʻī a i loko o kou underarm, collarbone a i ʻole ka wahi groin, e kamaʻilio me kāu kauka mālama ola. E kāhea pū i kāu hui mālama inā loaʻa iā ʻoe kahi manaʻo piha mau i kou umauma a i ʻole ka ʻōpū, ke kuni, ka luhi, a i ʻole ka nalowale kaumaha ʻaʻole hiki iā ʻoe ke wehewehe.

Inā ʻike ʻoe i kahi lymph node i hoʻonui ʻia ma ka ʻaoʻao o kou ʻāʻī a i ʻole ma kou underarm, collarbone a i ʻole ka wahi groin, e kamaʻilio me kāu limahana mālama ola. E kāhea pū i kāu kime mālama inā loaʻa iā ʻoe kahi manaʻo piha mau i loko o kou umauma a i ʻole ka ʻōpū, ke kuni, ka luhi, a i ʻole ka pohō kaumaha ʻaʻole hiki iā ʻoe ke wehewehe.

ʻAʻole maopopo ka mea e hoʻoulu ai i ka maʻi unicentric Castleman a i ʻole ka maʻi idiopathic multicentric Castleman (MCD). Eia naʻe, ua ʻike ʻia ka MCD HHV-8-positive i nā kānaka ʻaʻole i loaʻa ka hana maʻamau i ko lākou ʻōnaehana pale kino ma muli o ka maʻi HIV a i ʻole nā kumu ʻē aʻe.

Hiki i ka maʻi Castleman ke hoʻopili i nā kānaka o kēlā me kēia makahiki a i ʻole ka wahine. Hoʻomaʻamaʻa pinepine ʻia nā kānaka me ka maʻi Castleman i ka wā waena o ke ola, akā hiki ke hana i kēlā me kēia makahiki, me ka wā kamaliʻi.

ʻAʻohe mau kumu pilikia i ʻike ʻia no ka maʻi Castleman unicentric a i ʻole ka maʻi Castleman multicentric idiopathic. Hoʻonui ka maʻi ʻana me ka HIV a i ʻole ka loaʻa ʻana o kahi kūlana e hoʻemi ai i ka hana maikaʻi ʻana o ka ʻōnaehana pale kino i ka pilikia o ka loaʻa ʻana o ka maʻi Castleman multicentric HHV-8-positive.

ʻO ka poʻe me ka maʻi Castleman unicentric (UCD) maʻamau e ola maikaʻi ana ke lawe ʻia ka lymph node i pilikia, a ʻaʻole hoʻololi ʻia ka lōʻihi o ke ola. Akā, ʻoi aku ka nui o ka pilikia o ka loaʻa ʻana o kahi kūlana autoimmune maʻamau i kapa ʻia ʻo paraneoplastic pemphigus. Hiki i kēia kūlana ke hoʻoweliweli i ke ola. Hoʻokumu ka paraneoplastic pemphigus i nā ʻeha ma ka waha a ma ka ʻili e kuhi hewa pinepine ʻia. ʻOiai he haʻahaʻa ka pilikia o ka loaʻa ʻana o ka paraneoplastic pemphigus, he mea nui ka nānā ʻana i kēia kūlana inā loaʻa iā ʻoe ka UCD.

Hiki i ka maʻi Castleman multicentric idiopathic ke wikiwiki i ka ʻino e komo pū me nā pilikia hoʻoweliweli ola me ka hana o nā kino. Pono kēia i ka mālama koʻikoʻi me ka mīkini e kōkua ana i ka hanu, i kapa ʻia ʻo ka ventilator, a me nā lāʻau lapaʻau e kōkua ana i ka hana o nā kino, e like me ka dialysis a me nā transfusion.

Hiki i ka maʻi Castleman multicentric HHV-8-positive ke komo pū me nā maʻi hoʻoweliweli ola a me ka hāʻule ʻana o nā kino. ʻOi aku ka maikaʻi ʻole o ka poʻe i loaʻa pū kekahi me ka HIV/AIDS.

Ma hope o ka nānā ʻana i kāu moʻolelo lapaʻau a me ka hana ʻana i kahi hōʻoia kino kikoʻī, hiki i kāu limahana mālama ola ke ʻōlelo aku i kēia mau mea:

• Nā hoʻokolohua koko a me ka mimi. Kōkua kēia mau mea e kāpae i nā maʻi ʻē aʻe a i ʻole nā ​​maʻi. Hiki i kēia mau hoʻokolohua ke loaʻa hoʻi i ka anemia a me nā loli i nā protein koko e hiki ke maʻamau i ka maʻi Castleman.
• Nā hoʻokolohua kiʻi. Hiki i kēia mau hoʻokolohua ke loaʻa i nā lymph node nui a i ʻole ka ate a i ʻole ka spleen nui. Hiki ke hoʻohana ʻia kahi CT scan o ka ʻāʻī, ka umauma, ka ʻōpū a me ka pelvis. Hiki ke hoʻohana ʻia kahi positron emission tomography scan, i ʻike ʻia hoʻi ʻo ka PET scan, e ʻike i ka maʻi Castleman. Hiki i nā PET scan ke hōʻike inā ke hana nei kahi lapaʻau.
• Lymph node biopsy. He mea nui kēia hoʻokolohua e ʻike i ka maʻi Castleman a kāpae i nā maʻi pili, e like me ka lymphoma. I loko o ka biopsy, lawe ʻia kahi laʻana o ka ʻiʻo mai kahi lymph node nui a nānā ʻia i loko o kahi labo.

Aia ke mālama ma muli o ke ʻano o ka maʻi Castleman āu i loaʻa ai. ʻO ka maʻi Castleman Unicentric ʻO ka ʻokiʻoki e wehe i ka lymph node i pilikia ai ka mālama maʻamau no ka maʻi Castleman unicentric (UCD). Inā aia ka lymph node i loko o ka umauma a i ʻole ka ʻōpū, pono paha ka ʻokiʻoki nui. ʻO ka ʻokiʻoki e wehe i ka lymph node i hoʻonui ʻia e hoʻōla maʻamau i ka UCD. Eia naʻe, e hoʻi mai ana kekahi manawa ka UCD. Inā ʻaʻole hiki ke hana ʻokiʻoki, pono paha ʻoe i nā lāʻau lapaʻau i hoʻohana pinepine ʻia no ka maʻi Castleman multicentric. Inā ʻaʻole hana nā lāʻau lapaʻau, hiki i ka radiation therapy ke koho. Pono hou ka noiʻi i ke mālama ʻana i ka maʻi Castleman oligocentric, akā ʻo ka mālama ʻana he like ia me ka UCD. E pono paha iā ʻoe nā hoʻokolokolo hahai, me ka imaging a me nā hoʻokolohua labo, e nānā inā ʻaʻole i hoʻi mai ka maʻi. HHV-8-positive multicentric Castleman disease ʻO Rituximab (Rituxan) ka mālama mua maʻamau no ka HHV-8-positive MCD. He nui ka hopena o Rituximab, akā i kekahi manawa pono nā lāʻau i kapa ʻia ʻo antivirals a me nā chemotherapy. Hiki i nā lāʻau antiviral ke kāohi i ka hana a HHV-8 a i ʻole HIV, a hiki i nā chemotherapy ke hoʻopau i nā cell immune keu. Idiopathic multicentric Castleman disease ʻO Siltuximab (Sylvant) ka mālama mua maʻamau no ka idiopathic MCD. Ma ʻAmelika Hui Pū ʻIa, ʻo siltuximab wale nō ka lāʻau i ʻāpono ʻia e ka Food and Drug Administration (FDA) no ke mālama ʻana i ka idiopathic MCD. ʻO nā poʻe i maikaʻi ma hope o ka lawe ʻana i ka siltuximab e kūleʻa pinepine ana i ka mālama lōʻihi. Hoʻopau kēia lāʻau i ka hana o ka protein i kapa ʻia ʻo interleukin-6. Hoʻopuka nui nā kino o nā poʻe i loaʻa ka idiopathic MCD i kēia protein. Loaʻa pinepine i nā poʻe maʻi nui me ka idiopathic MCD ke mālama ʻia me nā lāʻau i kapa ʻia ʻo corticosteroids. Pono paha lākou i ka chemotherapy. Hiki i nā corticosteroids e like me ka prednisone ke kōkua i ka kaohi ʻana i ka mumū. Hiki i ka chemotherapy ke hoʻopau i nā cell immune e hana nei i nā pilikia. I ka wā ʻaʻole hana ka siltuximab, hiki ke hoʻohana ʻia nā mālama ʻē aʻe e like me rituximab (Rituxan) a me sirolimus (Rapamune). ʻIke hou aku Chemotherapy Radiation therapy Noi i kahi kūkā