He mea noa te mataku ina kite tātou i ngā panonitanga o ō tātou tinana, kāore? Ina koa mēnā ka kite tātou i ngā puku iti i tō tātou kaki, i ō tātou kēkē, i tō tātou hope rānei, ka whakaaro tātou, 'He aha tēnei?' I tēnei rā ka kōrero tātou mō tētahi mate onge ka puta ai ēnei momo puku, engari kāore i te maha ngā tāngata kua rongo mōna. Ko te ingoa o tēnei ko te Mate Rosai-Dorfman.
E mōhio ana koe he aha te mate Ross-Dorffman (RDD)?
He kupu ngāwari noa iho, he momo toto mā kei roto i ō tātou tinana e kiia nei ko ngā histiocytes . He rite ēnei ki ngā pirihimana i roto i ō tātou tinana, e whawhai ana ki ngā iroriki e puta ai te mate, ā, he wāhanga nui rātou o tō tātou pūnaha ārai mate. Heoi, ina nui rawa te tipu o ēnei histiocytes, ka kiia ko te mate Ross-Dorffmann (RDD). Ehara tēnei i te mate pukupuku (pai) , arā, ehara i te mate kino e horapa ana mai i tētahi wāhanga o te tinana ki tētahi atu. Heoi, ina nui rawa te kohikohi o ēnei pūtau, ka pāngia tātou e te mamae me ngā tohu.
I te nuinga o te wā, ka kohia ēnei histiocytes taikaha ki roto i ngā kōpū lymph i roto i tō tātou kaki. Kātahi ka pupuhi pērā i ngā puku i roto i te kaki. I ētahi wā ka kiia tēnei e ngā tākuta he pupuhi o ngā kōpū '(lymphadenopathy).' Engari kāore e pā ki te kaki anake, engari ki ngā kōpū i ētahi atu wāhanga o te tinana. I ētahi wā, hei tāpiri atu ki ngā kōpū, ka kohia anō hoki ēnei pūtau ki ētahi atu wāhanga o te tinana (ngā wāhi extranodal).
E kiia ana hoki tēnei mate ko te 'sinus histiocytosis with massive lymphadenopathy'. He mate onge tēnei nō te rōpū 'non-Langerhans cell histiocytosis'.
He aha ngā momo matua o te mate Ross-Dorffmann (RDD)?
Kāore tēnei mate e pā ki te katoa i te ōrite. Ko ētahi tāngata he pupuhi i te wāhi kotahi, ko ētahi he pupuhi i ētahi wāhi. Waihoki, ka taea e ēnei histiocytes te kohikohi ehara i te mea i roto i ngā puku anake engari i roto hoki i ētahi atu wāhanga o te tinana. Koinei te whakarōpūtanga.
- Mate Ross-Dorffmann Matarohia (Nodal) (Cussic/Nodal RDD): Koinei te momo tino noa. Ko te nuinga o te wā ka pupuhi ngā pona o te kaki. Heoi, ka rerekē pea ngā tohu i runga i te maha o ngā pona e pāngia ana.
- RDD Extranodal: I tēnei momo, ka kohikohi ēnei histiocytes i roto i ngā kiko ehara i te mea ko ngā pona lymph. Ko te kiri te mea tino pāngia. Ka kiia tēnei ko te cutaneous RDD (CRDD) . Hei tāpiri, ka pā pea tēnei āhuatanga ki ō tātou sinuses, ngā kanohi me ngā kamo, ngā wheua, me te pūnaha io matua (CNS) – arā, te roro me te taurakira. I ētahi wā ka pāngia hoki te pūnaha manawa me te pūnaha gastrointestinal.
Tata ki te 40% o te hunga whai RDD ka kohia pea ēnei pūtau histiocyte ki ētahi atu wāhanga o te tinana, hei tāpiri atu ki ngā kiritona, e ai ki tāu i kōrero ai.
Ko wai te mea tino tūpono ka pāngia e tēnei mate?
Ko te mate Ross-Dorffmann ka pā ki ngā tamariki, ngā taiohi, me ngā taiohi . I te nuinga o te wā ka kitea tēnei mate i roto i te hunga kei roto i ō rātou tau 20. Heoi anō, kua pūrongohia hoki kei te pāngia te hunga kei roto i ō rātou tau 70.
Ko te RDD, e pā ana ki ngā poro, he mea noa ake i roto i ngā tāne nō te uri Āwherika. Engari ko te mate kiri kua whakahuatia i mua ake nei ko te "CRDD" he mea noa ake i roto i ngā wāhine nō te uri Āhia. Ko te "CRDD" he mea noa ake i roto i te hunga kei roto i ō rātou tau 20, 30, me te 40.
He pēhea te onge o te mate Ross-Dorfman?
He mate tino onge tēnei. Tata ki te kotahi i roto i te rua rau mano tāngata e pāngia ana. Hei tauira, i te Hononga o Amerika, tata ki te 100 ngā kēhi hou e pūrongohia ana i ia tau. Ko te tikanga he iti ake anō i Sri Lanka.
He aha ngā tohu o te mate Rose-Dorfman (RDD)?
Ka whakawhirinaki ngā tohumate ka pā ki a koe ki te wāhi o roto i tō tinana e kohia ai ngā histiocytes taikaha. Mena ko ngā pona lymph i tō kaki anake e pāngia ana, tera pea he ngawari noa iho ō tohumate, kāore rānei. Heoi, ki te kohia ēnei pūtau, ā, ka whakararuraru i te mahi a tētahi okana, tera pea ka kaha ake ō tohumate.
Ngā tohu tawhito (pūtau)
He maha ngā wā ka kohia ngā histiocytes ki roto i ngā kiko o te kaki. Nō reira, ko te tohu tino kitea ko te putanga mai o ngā puku pupuhi, kāore he mamae i ngā taha e rua o te kaki . Tērā pea ka mahara koe ki te wā ka puta he puku iti i roto i tō kaki ina pāngia koe e te makariri, e te rewharewha rānei, tika? Ae, engari kāore pea e mamae. Heoi, i runga i te wāhi o te kiko e pāngia ana, ka taea hoki te pupuhi ki ētahi atu wāhi.
Ko ngā wāhi matua o te mumura ka puta mai i te pikinga o ngā histiocytes ko:
- Tō kaki
- Te rohe o te huha
- Ngā kēkē
- Te waenganui o te uma (mediastinum)
Tērā pea kāore koe e kite i ētahi atu tohu i tua atu i te pupuhi, tērā pea ka kite koe i ēnei tohu:
- Kirika
- Kiri mā
- Ngenge rawa
- Te werawera o te pō
- Ihu rere
- Te whakahekenga taumaha kāore i te whakamāramahia
Ngā tohu o waho o te node
Ka pā te mate Rose-Dorffmann (`CRDD`) ki te kiri, ā, ka puta ki ngā wāhi katoa o te tinana. Ko ēnei puku, e hangaia ana e te kohinga pūtau histiocyte, ka puhoi te tipu. Ko ngā tohu ka kitea i runga i te kiri ko:
- Ngā puku papatahi, teitei rānei
- He puku kī tonu, he puku totoka rānei
- Ngā puku kōwhai, papura, whero, parauri rānei
- Ka horahia pea ki te kiri katoa, ka horahia rānei ki te wāhi kotahi.
Ina pāngia tētahi okana, tētahi pūnaha tinana rānei e ēnei histiocytes taikaha, ka puta ngā tohu rerekē. Hei tauira, ki te pāngia ngā kanohi e te RDD, ka taea e ia te kite rua. Ki te pā ki te pūnaha io matua, ka taea e ia te mate haurangi. Ki te pā ki ngā pukahukahu, ka taea e ia te maremare tonu. Waihoki, ka rerekē ngā tohu i runga i te wāhi e pāngia ana.
He aha te pūtake o te mate Ross-Dorffman (RDD)?
Kāore anō kia mōhiotia e ngā kaipūtaiao te tino take o tēnei mate. Nā te mea he rerekē te pānga o te RDD ki ia tangata, ki ia tangata, tera pea he maha ngā take. Hei tauira, e kī ana ngā rangahau hou ko te mate kiri "CRDD" i puta mai i tētahi mea ehara i te mea kotahi e puta ai te RDD noa.
Kua kitea e ngā kairangahau i ēnei wā tata nei kei roto i ētahi atu momo RDD ētahi whakarerekētanga ira, haunga te CRDD. Ka taea e ēnei whakarerekētanga te whakatupu i ngā pūtau me te kore e taea te whakahaere.
He tokomaha ngā tāngata whai RDD e pāngia ana e tēnei mate me ētahi atu mate hauora. Nō reira, tera pea he hononga i waenga i aua mate me te RDD. Me nui ake ngā rangahau mō tēnei.
Kua kitea te hononga o te RDD ki ngā āhuatanga e whai ake nei:
- Ngā mate urutā: Hei tauira, ko te herpes, te huaketo Epstein-Barr, te cytomegalovirus, me te mate HIV.
- Matepukupuku: Te lymphoma Hodgkin, te lymphoma non-Hodgkin, me ngā matepukupuku kiri pērā i te cutaneous clear-cell sarcoma. (Engari kia mahara, ehara te RDD i te matepukupuku.)
- Ngā āhuatanga ārai mate: Lupus, Juvenile Idiopathic Ruamihi, Autoimmune Hemolytic Anemia.
Me pēhea te tautuhi i te mate Ross-Dorffman (RDD)?
Ka tirohia tuatahitia e tō tākuta ō tohumate, ā, ka pātai atu mō ō tohumate. Ka rapua e rātou ngā tohu o te RDD, pērā i te pupuhi o ngā repe me ngā puku kiri. Ka pātai atu anō hoki rātou mō tō hītori hauora kia kitea ai mēnā kei a koe, kua pāngia rānei koe e ētahi āhuatanga e pā ana ki te RDD.
Hei tāpiri, ka taea e te tākuta te whakahaere i ēnei whakamātautau:
- Ngā tikanga whakaahua: I runga i te momo kiko e whakapaetia ana kei roto ngā histiocytes, ka tono pea te tākuta i tētahi matawai X-ray, ultrasound, MRI, CT, PET, PET/CT, wheua rānei. Ka taea te whakamahi i ēnei hei tiro i ngā mea kei roto i te tinana.
- Ngā whakamātautau toto: Ka taea te mahi i ētahi whakamātautau toto, pērā i te tatau toto katoa (CBC) me te tātaritanga pākia whānui, hei tirotiro i ngā huringa e puta ana i roto i te tinana.
- Koiora: He whakamātautau tino nui tēnei.Ko te tikanga o tēnei ko te tango i tētahi tauira iti o te kiko e pāngia ana (hei tauira, he kōpuku lymph e pupuhi ana) me te tirotiro i ngā pūtau i raro i te karuārai hei tirotiro mēnā he āhuatanga RDD kei roto. Ka taea hoki e tēnei koiora te whakatau mēnā kei te puta te tipu pūtau rerekē mai i tētahi atu mate.
Me pēhea te rongoā mō te mate Ross-Dorffmann (RDD)?
I ētahi wā, ka taea e te RDD te hoki whakamuri noa iho me te kore he maimoatanga. Ko te tikanga ka ora ake te mate i a ia anō. Heoi, he uaua ki te matapae i te wā e pau ana. Tērā pea ka roa ngā marama, ngā tau rānei hei oranga ake. Heoi, i ētahi wā, kāore pea e memeha noa iho, ka hoki mai anō rānei. Ki te kore e rongoatia, ka kino haere te mate.
Ka whakawhirinaki te maimoatanga ka whakawhiwhia ki a koe ki te pānga o te RDD ki a koe.
- Te Tirotiro: Ki te kore he tohu e pā nui ana ki tō oranga, ka whakatau pea tō tākuta ki te aroturuki i tō āhua. He rite tēnei ki te huarahi "tatari mataara".
- Pokanga: Ka taea te tango i ngā puku mā te pokanga. Mena kei a koe te `CRDD` (cutaneous RDD), kei te aukati rānei ngā puku i tō ara manawa, kei te pā rānei ki tō taurakira, ka taea te mahi pokanga.
- Whakaora irahiko: Ki te kore e taea e te pokanga te tango i ngā histiocytes, ka taea te hoatu i te rongoā irahiko. Ko te tikanga o tēnei ko te whakamahi i tētahi mīhini hei tuku i ngā hihi irahiko ki ngā pūtau, hei whakangaro i a rātou.
- Te rongoā matū: Mena he kino te mate, ki te kore rānei e whakaitihia ngā tohu e ētahi atu maimoatanga, pēnei i te pokanga, ka whakaarohia pea te rongoā matū. Kei roto i tēnei ko te whakamahinga o ngā rongoā e patu ana i ngā pūtau tere tipu, pēnei i ngā pūtau matepukupuku.
- Corticosteroids: Ka taea e ngā corticosteroids, pērā i te prednisone, te whakaiti i te pupuhi o ngā hononga me te whakaora i ngā tohu. He momo rongoā ēnei hei whakaiti i te mumura.
- Ārai Mate: Ka āwhina ēnei maimoatanga i tō ake pūnaha ārai mate ki te kimi me te whakangaro i ngā pūtau histiocyte taikaha kia whai hua ake.
He aha te heke mai mō te tangata e pangia ana e te mate Ross-Dorffman (RDD)?
Ka whakawhirinaki tō matapae ki ētahi āhuatanga. Te maha o ngā pūtau e pāngia ana, te wāhi o roto i tō tinana e noho ana ngā histiocytes taikaha, me te pai o tō urupare ki te maimoatanga. I te nuinga o te wā, ka memeha noa te RDD. Heoi, i ētahi wā, me rongoā, ki te kore ka puta he raruraru nui.
Ko te tikanga, ko te iti ake o ngā pūtau e pāngia ana, ko te pai ake o te hua.. Mena he RDD extranodal, e pā ana ki te kiri, te uma, te ara manawa o runga rānei, he pai ake te putanga. Heoi, ki te mea kei te ara manawa o raro, ngā whatukuhu, te ate rānei ngā histiocytes, tera pea ka kino ake te āhua.
He aha ngā pātai me pātai atu e au ki taku tākuta?
Kaua e wareware ki te kōrero ki tō tākuta mō ēnei mea, he mea nui hoki kia mārama koe ki tō āhua:
- "E te tākuta, ko ēhea ngā wāhanga o tōku tinana e pāngia ana e te mate RDD?"
- "Me whai atu anō ētahi atu whakamātautau whakaahua (pēnei i ngā karapa) me ētahi atu whakamātautau toto?"
- "Me rongoā ahau, kāore rānei e taea e taku mate RDD te memeha noa iho?"
- "Kia pēhea te nui o tōku manukanuka mō tēnei āhuatanga RDD?"
- "He aha te maimoatanga pai rawa atu e taunaki ana te tākuta mōku?"
- "He aha ngā pānga taha ka pā mai i muri i te maimoatanga?"
- "He aha ngā tūponotanga ka hoki anō taku mate RDD i muri i te maimoatanga?"
- "Kia pēhea te auau o taku haerenga mai ki te tirotiro hauora hei aroturuki i tōku āhua?"
He mate pukupuku te mate Ross-Dorffman (RDD)?
Kāo. He pātai tēnei e pātaihia ana e te tini tāngata. Ehara te mate Rose-Dorffman i te mate pukupuku (pai) . Arā, kāore e horapa mai i tētahi wāhanga o te tinana ki tētahi atu pērā i te mate pukupuku (mate pukupuku). Heoi, ko ngā puku e puta mai ana i te RDD ka pā ki ngā okana, ka whakararuraru i tā rātou mahi, ā, ka puta he raruraru. Ko tō tākuta te tangata pai rawa atu ki te mōhio ki te pānga o tō mate RDD ki tō tinana, me te mea me āwangawanga koe mōna.
Nā wai i kite te mate Ross-Dorffman (RDD)?
He mea pai hoki kia mōhio ki tēnei. I te tau 1965, i kitea e tētahi kaipūtaiao ko Pierre Paul Louis Lucien Destombes ngā tohumate o te RDD i roto i ngā tūroro tokowhā. Kātahi, i te tau 1969, i rangahauhia e ngā kaipūtaiao tokorua ko Juan Rosai rāua ko Ronald Dorfman tētahi atu rōpū tūroro he rite ngā tohumate. E mōhiotia whānuitia ana te mate nei ko te mate Rosai-Dorfman, e whakakotahi ana i ō rāua ingoa. He onge te wā ka kiia hoki ko te mate Rosai-Dorfman-Destombes, hei whakanui i ngā kaipūtaiao tokotoru.
Hei whakamutunga, ngā mea hei maumahara (Karere hei tango ki te kāinga)
He uaua te tautuhi i te mate Ross-Dorffmann (RDD) nā te mea he tino onge, ā, he rerekē te pānga ki ia tangata . Kāore he aratohu maimoatanga kotahi mō te RDD. Koia te mea nui kia kōrero noa koe ki tō tākuta, kia mārama ki tō tautuhi, me te mōhio ki ngā kōwhiringa maimoatanga e wātea ana ki a koe.
Tērā pea he RDD ngawari tōu, ā, ka memeha noa iho. Tērā pea me whakamahi koe i ētahi maimoatanga. Pātai atu ki tō tākuta me pēhea te whakarite i tō mahere maimoatanga me ngā hua e tumanakohia ana kia rite ki tō āhua.
Kia maumahara, ehara i te mea ko koe anake . Mēnā kei te pāngia koe e tēnei mate onge, he mea nui kia whai i ngā tohutohu hauora, kia kaha hoki te hinengaro. Mēnā he pātai, he ruarua rānei āu, kōrerohia ki tō tākuta. Kaua e mataku, nā te mea ko te mōhio te patu pai rawa atu hei whawhai ki ēnei momo āhuatanga.
Mate Rosai -Dorfman, RDD, histiocytes, lymphadenopathy, puku kaki, mate kiri, CRDD, mate onge, pūnaha ārai mate











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