He aha kēia mau puʻupuʻu ma ka ʻāʻī a me nā ʻauwae? E aʻo kākou e pili ana i ka maʻi Rosai-Dorfman!

He mea maʻamau ka makaʻu iki ʻana i ka wā e ʻike ai kākou i nā loli i ko kākou kino, ʻeā? ʻOiai inā ʻike kākou i nā puʻupuʻu liʻiliʻi ma ko kākou ʻāʻī, nā ʻāʻī lima, a i ʻole ka ʻūhā, manaʻo mākou, 'He aha kēia?' I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi laha ʻole e hiki ai ke hana i ia mau puʻupuʻu, akā ʻaʻole nui ka poʻe i lohe. Ua kapa ʻia kēia ʻo ka maʻi Rosai-Dorfman.

Ua ʻike anei ʻoe i ke ʻano o ka maʻi Ross-Dorffman (RDD)?

I ka ʻōlelo maʻalahi, loaʻa i ko mākou kino kahi ʻano o nā hunaola koko keʻokeʻo i kapa ʻia ʻo nā histiocytes . Ua like kēia me nā mākaʻi i loko o ko mākou kino, e hakakā ana i nā germs e hoʻoulu ai i nā maʻi, a he ʻāpana koʻikoʻi lākou o kā mākou ʻōnaehana pale. Eia nō naʻe, ke ulu nui kēia mau histiocytes, ua kapa ʻia ʻo ia he maʻi Ross-Dorffmann (RDD). ʻAʻole kēia he maʻi kanesa (benign) , ʻo ia hoʻi, ʻaʻole ia he maʻi weliweli e laha ana mai kekahi ʻāpana o ke kino a i kekahi. Eia nō naʻe, ke hōʻiliʻili nui ʻia kēia mau hunaola, hiki iā mākou ke ʻike i ka ʻoluʻolu ʻole a me nā hōʻailona.

ʻO ka hapa nui o ka manawa, hōʻiliʻili kēia mau histiocytes keu i loko o nā lymph nodes i loko o ko kākou ʻāʻī. A laila pehu lākou e like me nā puʻupuʻu ma ka ʻāʻī. I kekahi manawa kapa nā kauka i kēia pehu o nā nodes '(lymphadenopathy).' Akā hiki iā ia ke hoʻopilikia ʻaʻole wale i ka ʻāʻī, akā i nā nodes ma nā wahi ʻē aʻe o ke kino. I kekahi manawa, me nā nodes, hiki i kēia mau cell ke hōʻiliʻili ma nā wahi ʻē aʻe o ke kino (nā wahi extranodal).

Ua kapa ʻia kēia maʻi ʻo 'sinus histiocytosis me ka lymphadenopathy nui'. He maʻi laha ʻole kēia no ka hui 'non-Langerhans cell histiocytosis'.

He aha nā ʻano nui o ka maʻi Ross-Dorffmann (RDD)?

ʻAʻole like ka hopena o kēia maʻi i nā kānaka a pau. Loaʻa i kekahi poʻe ka pehu ma kahi hoʻokahi, aʻo kekahi poʻe e pehu paha ma kekahi mau wahi. Eia kekahi, hiki i kēia mau histiocytes ke hōʻiliʻili ʻaʻole wale ma nā puʻupuʻu akā ma nā wahi ʻē aʻe o ke kino. Penei ke ʻano o kona hoʻokaʻawale ʻia ʻana.

• ʻO ka maʻi Ross-Dorffmann maʻamau (Nodal) (Classic/Nodal RDD): ʻO kēia ke ʻano maʻamau. Hoʻopilikia nui ia i ka pehu ʻana o nā node ma ka ʻāʻī. Eia nō naʻe, hiki ke ʻokoʻa nā hōʻailona ma muli o ka nui o nā node i hoʻopilikia ʻia.
• ʻO Extranodal RDD: Ma kēia ʻano, hōʻiliʻili kēia mau histiocytes i loko o nā ʻiʻo ʻē aʻe ma mua o nā lymph nodes. ʻO ka ʻili ka mea i hoʻopilikia pinepine ʻia. Ua kapa ʻia kēia he cutaneous RDD (CRDD) . Eia kekahi, hiki i ke kūlana ke hoʻopilikia i ko mākou sinuses, nā maka a me nā lihilihi, nā iwi, a me ka ʻōnaehana nerve waena (CNS) - ʻo ia hoʻi, ka lolo a me ke kaula spinal. I kekahi manawa hiki ke hoʻopilikia ʻia ka ʻōnaehana hanu a me ka ʻōnaehana gastrointestinal.

Ma kahi o 40% o ka poʻe me RDD e hōʻiliʻili ʻia kēia mau pūnaewele histiocyte ma nā wahi ʻē aʻe o ke kino, me ka hoʻohui pū ʻana i nā warts, e like me kāu i ʻōlelo ai.

ʻO wai ka mea i loaʻa pinepine i kēia maʻi?

Hoʻopilikia nui ka maʻi Ross-Dorffmann i nā keiki, nā ʻōpio, a me nā mākua ʻōpio . ʻIke pinepine ʻia ia i ka poʻe i ko lākou mau makahiki 20. Eia nō naʻe, ua hōʻike ʻia hoʻi ka poʻe i ko lākou mau makahiki 70 ua loaʻa i ka maʻi.

ʻO RDD, ka mea e hoʻopilikia ai i nā testicles, ʻoi aku ka maʻamau i nā kāne o ka hanauna ʻApelika. Akā ʻo ke ʻano ʻili i ʻōlelo ʻia ma mua ʻo "CRDD" ʻoi aku ka maʻamau i nā wahine o ka hanauna ʻĀsia. ʻO "CRDD" ka mea maʻamau i nā poʻe i ko lākou mau makahiki 20, 30, a me 40.

Pehea ka laha ʻole o ka maʻi Ross-Dorfman?

He maʻi laha ʻole kēia. Hoʻopilikia ia i hoʻokahi i loko o ʻelua haneli tausani kānaka. No ka laʻana, ma ʻAmelika Hui Pū ʻIa, ua hōʻike ʻia ma kahi o 100 mau hihia hou i kēlā me kēia makahiki. ʻO ia hoʻi, ma Sri Lanka, ʻoi aku ka emi.

He aha nā hōʻailona o ka maʻi Rose-Dorfman (RDD)?

ʻO nā hōʻailona āu e ʻike ai e pili ana i kahi o kou kino e hōʻiliʻili ai nā histiocytes keu. Inā ʻo nā lymph node wale nō i kou ʻāʻī e pili ana, hiki ke maʻalahi a ʻaʻole paha kāu mau hōʻailona. Eia nō naʻe, inā hōʻiliʻili kēia mau cell a hoʻopilikia i ka hana o kahi ʻōkana, hiki ke ʻoi aku ka koʻikoʻi o kāu mau hōʻailona.

Nā hōʻailona maʻamau (nodal)

Hoʻākoakoa pinepine nā Histiocytes i loko o nā ʻiʻo ʻāʻī. No laila, ʻo ka hōʻailona maʻamau ka ʻike ʻia ʻana o nā puʻupuʻu pehu ʻole ʻeha ma nā ʻaoʻao ʻelua o ka ʻāʻī . E hoʻomanaʻo paha ʻoe i kekahi manawa e loaʻa ai kahi puʻupuʻu liʻiliʻi i kou ʻāʻī ke loaʻa iā ʻoe ke anu a i ʻole ka maʻi flu, ʻeā? Pololei kēlā, akā ʻaʻole paha he ʻeha. Eia nō naʻe, ma muli o kahi o ka ʻiʻo i hoʻopilikia ʻia, hiki nō hoʻi ke pehu ma nā wahi ʻē aʻe.

ʻO nā wahi nui o ka mumū e hiki ke kū mai ma muli o ka hoʻonui ʻia o nā histiocytes:

• Kou ʻāʻī
• Wahi ʻūhā
• Nā ʻauwae
• ʻO ka waena o ka umauma (mediastinum)

ʻAʻole paha ʻoe e ʻike i kekahi mau hōʻailona ʻē aʻe ma mua o ka pehu ʻana, a i ʻole e ʻike paha ʻoe i nā hōʻailona e like me:

• kuni
• ʻIli keʻokeʻo
• Luhi nui
• Nā hou o ka pō
• Ihu wai
• Ka pohō kaumaha i wehewehe ʻole ʻia

Nā hōʻailona extranodal

Hiki i ka maʻi Rose-Dorffmann (`CRDD`) ke hoʻopilikia i ka ʻili a hiki ke ʻike ʻia ma nā wahi āpau o ke kino. ʻO kēia mau puʻupuʻu, i hana ʻia e kahi hōʻiliʻili o nā pūnaewele histiocyte, ulu mālie pinepine. ʻO nā hōʻailona e ʻike ʻia ma ka ʻili:

• Nā puʻupuʻu pālahalaha a i ʻole i hāpai ʻia
• Nā puʻupuʻu piha i ka nahu a i ʻole nā ​​​​puʻupuʻu paʻa
• Nā puʻupuʻu melemele, poni, ʻulaʻula, a ʻeleʻele paha
• Hiki ke hohola ʻia ma luna o ka ʻili a i kaupalena ʻia paha i hoʻokahi wahi.

Ke hoʻopilikia kēia mau histiocytes keu i kahi ʻōkana a i ʻole ka ʻōnaehana kino, hiki ke loaʻa nā hōʻailona like ʻole. No ka laʻana, inā hoʻopilikia ka RDD i nā maka, hiki iā ia ke hana i ka ʻike pālua. Inā hoʻopilikia ia i ka ʻōnaehana nerve waena, hiki iā ia ke hana i nā hopu. Inā hoʻopilikia ia i nā māmā, hiki iā ia ke hana i ka ʻū mau. Pēlā nō, ʻokoʻa nā hōʻailona ma muli o ka wahi i hoʻopilikia ʻia.

He aha ke kumu o ka maʻi Ross-Dorffman (RDD)?

ʻAʻole ʻike nā kānaka ʻepekema i ke kumu kikoʻī o kēia maʻi. ʻOiai ʻokoʻa ka hopena o RDD i kēlā me kēia kanaka, hiki ke loaʻa kekahi mau kumu. No ka laʻana, hōʻike nā noiʻi hou nei ua hana ʻia ka maʻi ʻili "CRDD" e kekahi mea ʻē aʻe ma mua o nā mea like e hoʻoulu ai i ka RDD maʻamau.

Ua ʻike koke nei nā kānaka noiʻi ua loaʻa kekahi mau hoʻololi ʻana o ka gene i nā ʻano ʻē aʻe o RDD ma waho aʻe o CRDD. Hiki i kēia mau hoʻololi ke hoʻoulu i nā cell e ulu me ke kāohi ʻole ʻia.

He nui ka poʻe me RDD i loaʻa i kēia maʻi me nā maʻi olakino ʻē aʻe. No laila, aia paha kahi pilina ma waena o kēlā mau maʻi a me RDD. Pono ka noiʻi hou aku ma kēia.

Ua ʻike ʻia ua pili ʻo RDD me nā kūlana ma lalo nei:

• Nā maʻi viral: ʻO nā hiʻohiʻona e pili ana i ka herpes, ka maʻi Epstein-Barr, cytomegalovirus, a me ka maʻi HIV.
• Kanesa: Hodgkin lymphoma, non-Hodgkin lymphoma, a me nā maʻi ʻili e like me ka cutaneous clear-cell sarcoma. (Akā e hoʻomanaʻo, ʻaʻole ʻo RDD he maʻi ʻaʻai.)
• Nā kūlana Autoimmune: Lupus, Juvenile Idiopathic Arthritis, Autoimmune Hemolytic Anemia.

Pehea e ʻike ʻia ai ka maʻi Ross-Dorffman (RDD)?

E hana mua kāu kauka i kahi hoʻokolokolo kino a nīnau e pili ana i kāu mau hōʻailona. E nānā lākou i nā hōʻailona o RDD, e like me nā glands pehu a me nā puʻupuʻu ʻili. E nīnau pū lākou e pili ana i kāu mōʻaukala olakino e ʻike inā loaʻa iā ʻoe a i ʻole kekahi mau kūlana e pili ana me RDD.

Eia kekahi, hiki i ke kauka ke hana i kēia mau hoʻokolohua:

• Nā kaʻina hana kiʻi: Ma muli o ke ʻano o ka ʻiʻo i manaʻo ʻia he mau histiocytes, hiki i ke kauka ke kauoha i kahi X-ray, ultrasound, MRI, CT, PET, PET/CT, a i ʻole ka scan iwi. Hiki ke hoʻohana ʻia kēia mau mea e ʻike i nā mea i loko o ke kino.
• Nā hoʻāʻo koko: Hiki ke hana i kekahi mau hoʻāʻo koko, e like me ka helu koko piha (CBC) a me kahi panel metabolic piha, e ʻike i nā loli e hana nei i loko o ke kino.
• Biopsy: He hoʻokolohua koʻikoʻi loa kēia.Pili kēia i ka lawe ʻana i kahi hāpana liʻiliʻi o nā ʻiʻo i hoʻopilikia ʻia (e.g., kahi lymph node pehu) a me ka nānā ʻana i nā cell ma lalo o kahi microscope e ʻike inā he mau ʻano RDD ko lākou. Hiki i kēia biopsy ke hoʻoholo inā he maʻi ʻē aʻe ke kumu o ka ulu ʻana o nā cell maʻamau ʻole.

Pehea e mālama ʻia ai ka maʻi Ross-Dorffmann (RDD)?

I kekahi manawa, hiki i ka RDD ke hoʻomaha koke me ka ʻole o ka lāʻau lapaʻau. ʻO ke ʻano kēia e hoʻomaikaʻi wale ka maʻi. Eia nō naʻe, he paʻakikī ke wānana i ka manawa e pono ai. Hiki ke hala i nā mahina a i ʻole nā ​​​​makahiki e hoʻomaikaʻi ai. Eia nō naʻe, i kekahi mau hihia, ʻaʻole paha e nalowale iā ia iho, a i ʻole e hoʻi hou mai. Inā ʻaʻole mālama ʻia, e ʻoi aku ka ʻino o ka maʻi.

ʻO ka lāʻau āu e loaʻa ai e hilinaʻi ia i ka hopena o RDD iā ʻoe.

• Nānā: Inā ʻaʻohe ou hōʻailona e loaʻa ana ka hopena nui i kou ola, hiki i kāu kauka ke hoʻoholo e nānā i kou kūlana. Ua like kēia me ke ʻano "kali makaʻala".
• ʻOki kino: Hiki ke wehe ʻia nā puʻupuʻu ma ke ʻoki kino. Inā loaʻa iā ʻoe ka `CRDD` (cutaneous RDD), a inā paha e ālai ana nā puʻupuʻu i kou ala ea a i ʻole e hoʻopilikia ana i kou iwi kuamoʻo, hiki ke hana ʻia ke ʻoki kino.
• Lapaʻau radiation: Inā ʻaʻole hiki i ke ʻoki kino ke wehe i nā histiocytes, hiki ke hāʻawi ʻia ka lāʻau radiation. Pili kēia i ka hoʻohana ʻana i kahi mīkini e kuhikuhi i nā kukuna radiation i kuhikuhi ʻia i nā cell, e luku ana iā lākou.
• Kemoterapi: Inā koʻikoʻi ka maʻi, a inā ʻaʻole i hōʻemi nā lāʻau ʻē aʻe, e like me ke ʻoki ʻana, i nā hōʻailona, ​​hiki ke noʻonoʻo ʻia ka chemotherapy. Pili kēia i ka hoʻohana ʻana i nā lāʻau lapaʻau e pepehi i nā cell e ulu wikiwiki ana, e like me nā cell kanesa.
• Corticosteroids: Hiki i nā Corticosteroids, e like me ka prednisone, ke hōʻemi i ka pehu ʻana o nā hono a hōʻoluʻolu i nā hōʻailona. He ʻano lāʻau lapaʻau kēia e hōʻemi ai i ka mumū.
• Immunotherapy: Kōkua kēia mau lāʻau lapaʻau i kāu ʻōnaehana pale ponoʻī e ʻimi a luku i nā pūnaewele histiocyte keu me ka ʻoi aku ka maikaʻi.

He aha ka wā e hiki mai ana no kekahi me ka maʻi Ross-Dorffman (RDD)?

Aia kou wānana ma luna o kekahi mau kumu. ʻO ka helu o nā cell i hoʻopilikia ʻia, kahi o nā histiocytes keu i loko o kou kino, a pehea ka maikaʻi o kāu pane ʻana i ka lāʻau lapaʻau. ʻO ka hapa nui o ka manawa, hoʻopau ʻo RDD iā ia iho. Eia nō naʻe, i kekahi mau hihia, pono ka lāʻau lapaʻau, a i ʻole hiki ke loaʻa nā pilikia koʻikoʻi.

Ma keʻano laulā, ʻo ka liʻiliʻi o ka helu o nā cell i hoʻopilikia ʻia, ʻoi aku ka maikaʻi o ka hopena.Inā he extranodal RDD, ka mea e hoʻopilikia ai i ka ʻili, ka umauma, a i ʻole ke ala hanu o luna, ʻoi aku ka maikaʻi o ka hopena. Eia nō naʻe, inā loaʻa nā histiocytes ma ke ala hanu o lalo, nā puʻupaʻa, a i ʻole ke akepaʻa, ʻoi aku ka ʻino o ke kūlana.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Mai poina e kamaʻilio me kāu kauka e pili ana i kēia mau mea, no ka mea, he mea nui loa kou ʻike pono i kou kūlana:

• "E ke kauka, he aha nā ʻāpana o koʻu kino i hoʻopilikia ʻia e koʻu maʻi RDD?"
• "Pono anei au e hana i nā hoʻokolohua kiʻi hou aʻe (e like me nā scans) a i ʻole nā ​​hoʻokolohua koko?"
• "Pono anei au i ka lāʻau lapaʻau, a i ʻole hiki paha i koʻu maʻi RDD ke hoʻoponopono iā ia iho?"
• "Pehea koʻu hopohopo e pili ana i kēia kūlana RDD?"
• "He aha ka lāʻau lapaʻau maikaʻi loa a ke kauka e ʻōlelo mai nei noʻu?"
• "He aha nā hopena ʻaoʻao e hiki iaʻu ke manaʻo ma hope o ka lāʻau lapaʻau?"
• "He aha nā manawa kūpono e hoʻi hou mai ai koʻu maʻi RDD ma hope o ka mālama ʻana?"
• "ʻEhia pinepine e pono ai iaʻu e hele mai no nā hoʻokolohua e nānā i koʻu kūlana?"

He maʻi kanesa anei ka maʻi Ross-Dorffman (RDD)?

ʻAʻole. He nīnau kēia a ka poʻe he nui e nīnau ai. ʻAʻole ka maʻi Rose-Dorffman he maʻi kanesa (benign) . ʻO ia hoʻi, ʻaʻole ia e laha mai kekahi ʻāpana o ke kino i kekahi e like me ke kanesa (malignancy). Eia nō naʻe, hiki i nā puʻupuʻu i hana ʻia ma muli o RDD ke hoʻopilikia i nā ʻōpū, hoʻopilikia i kā lākou hana, a hoʻoulu i nā pilikia. ʻO kāu kauka ke kanaka maikaʻi loa e ʻike pehea e hoʻopilikia ai kou kūlana RDD i kou kino a inā paha ʻoe e hopohopo e pili ana i ia mea.

ʻO wai ka mea nāna i ʻike i ka maʻi Ross-Dorffman (RDD)?

He mea maikaʻi ke ʻike i kēia. I ka makahiki 1965, ua ʻike kekahi kanaka ʻepekema i kapa ʻia ʻo Pierre Paul Louis Lucien Destombes i nā hōʻailona RDD i loko o ʻehā mau maʻi. A laila, i ka makahiki 1969, ua aʻo ʻelua mau kānaka ʻepekema i kapa ʻia ʻo Juan Rosai lāua ʻo Ronald Dorfman i kahi hui ʻē aʻe o nā maʻi me nā hōʻailona like. Ua ʻike nui ʻia ka maʻi i kēia manawa ʻo ka maʻi Rosai-Dorfman, e hui pū ana i ko lākou mau inoa. ʻAʻole pinepine, ua kapa ʻia hoʻi ʻo ia ʻo ka maʻi Rosai-Dorfman-Destombes, no ka hoʻohanohano ʻana i nā kānaka ʻepekema ʻekolu.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

Hiki i ka maʻi Ross-Dorffmann (RDD) ke lilo i kahi hōʻailona paʻakikī no ka mea he mea kakaikahi loa ia a hoʻopilikia i kēlā me kēia kanaka i nā ʻano like ʻole . ʻAʻohe alakaʻi hoʻokahi no ka mālama ʻana no RDD. ʻO ia ke kumu he mea nui e kamaʻilio ākea me kāu kauka, e hoʻomaopopo i kāu hōʻailona, ​​a e makaʻala i kāu mau koho lapaʻau.

Loaʻa paha iā ʻoe ka RDD palupalu e hoʻopau wale ia. A i ʻole, pono paha ʻoe i ka hui pū ʻana o nā lāʻau lapaʻau. E nīnau i kāu kauka pehea e hoʻopilikino ʻia ai kāu hoʻolālā lapaʻau a me nā hopena i manaʻo ʻia i kou kūlana.

E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi . I ka wā e hana ai me ia ʻano maʻi laha ʻole, he mea nui loa ia e hahai i nā ʻōlelo aʻoaʻo lapaʻau a noho ikaika i ka noʻonoʻo. Inā he mau nīnau a kānalua paha kāu, e kūkākūkā me kāu kauka. Mai makaʻu, no ka mea, ʻo ka ʻike ka mea kaua maikaʻi loa e hakakā ai i ia mau mea.

ʻO ka maʻi Rosai-Dorfman, RDD, nā histiocytes, lymphadenopathy, nā puʻupuʻu ʻāʻī, nā maʻi ʻili, CRDD, nā maʻi laha ʻole, ʻōnaehana pale