He aha te Mate Castleman? Me kōrero māmā noa iho!

Kua kite koe i ēnei puku iti kei tō kaki, kei ō ringaringa rānei? I ētahi wā ka puta mai ēnei puku ina pāngia koe e te makariri, te rewharewha rānei. Engari, ehara tēnei i te mea ngāwari noa iho. He mate onge tēnei engari he mea nui kia mōhio koe. Ka kiia tēnei ko te Mate Castleman .

Ki te kī māmā noa, ko te Mate Castleman he āhuatanga e kaha rawa ai te pūnaha ārai mate o te tinana . Whakaarohia, ina uru mai he iroriki e mate ai te tinana, ka whawhai tō tātou pūnaha ārai mate ki a ia, pērā i te hōia e haere ana ki te pakanga. Ina ora te mate, ka noho puku anō taua hōia. Engari i roto i te tangata e pāngia ana e te Mate Castleman, kei te 'whakahaere' tonu tēnei pūnaha ārai mate. Ka puta te mumura roa i roto i te tinana, ka kino pea ō tātou whekau.

I tēnei wā, ko ō tātou pona lymph – ko aua puku ririki te ingoa – he rite ki te tātari. Ka tātarihia ngā iroriki me ngā pūtau ārai mate katoa mā roto i a rātou. Nō reira, nā te mea kei te mahi tonu te pūnaha ārai mate, kei te mahi tonu hoki ēnei pona lymph i te wā roa. Kātahi ka nui haere te tipu o ngā pūtau i roto i a rātou, ā, ka pupuhi ngā pona lymph. Ehara i te mea ko tēnei pupuhi anake, engari i roto i te Mate Castleman, ka puta he huringa ki te kiko o ēnei pona lymph.

He mate tino onge tēnei. Hei tauira, i te Hononga o Amerika, e 4,300 ki te 5,200 noa iho ngā kēhi hou ka kitea i ia tau. Nō reira, kei te ako tonu ngā tākuta i te nui o ngā mea mō tēnei. Heoi, ki te kitea tēnei mate i a koe, ka whiwhi koe i ngā maimoatanga motuhake e hāngai ana ki a koe.

He aha ngā momo matua o te mate Castleman?

E rua ngā momo matua o tēnei mate.

Mate Castleman Unicentric (UCD)

Ko te mate Unicentric Castleman (UCD) ko te wā ka nui haere te kotahi, te torutoru rānei o ngā pona lymph i tētahi wāhi o tō tinana . Whakaarohia tō kaki anake, ō kēkē rānei. Tata ki te toru hauwhā o ngā tūroro Castleman ka taka ki tēnei wāhanga.

Mate Castleman maha-pokapū (MCD)

Ko te mate Castleman maha-pokapū (MCD) he āhua e pupuhi ai ngā pona lymph i ngā wāhi maha o te tinana, kaua i te kotahi anake. Hei whakamārama ake, ka wehea anō e ngā tākuta tēnei momo MCD ki:

• MCD e pā ana ki te POEMS: He mate toto tino onge te POEMS. I ētahi wā, ka puta tahi te POEMS me te MCD.
• MCD e pā ana ki te HHV-8: Ka puta tēnei momo i te mate urutā e kiia nei ko te Human Herpes Virus-8 (HHV-8) . Mena kei a koe te mate HIV, kei te ngoikore rānei tō pūnaha ārai mate, ka nui ake te tūponotanga ka pāngia koe e tēnei momo MCD.
• MCD kāore i te mōhiotia te take (MCD Idiopathic - iMCD):Koinei te momo MCD tino noa. Ko te tikanga o te 'idiopathic' ko te kore e mōhiotia te tino take.

Inaianei, e toru ngā wāhanga iti o tēnei take kāore i te mōhiotia o te iMCD:

• Te hononga o te iMCD ki te mate TAFRO: I ētahi wā ka haere tahi te iMCD me tētahi āhuatanga e kiia nei ko te mate TAFRO. Ko te ingoa TAFRO i ahu mai i ngā reta tuatahi o ōna tohu. Ko ēnei: ​​Thrombocytopenia (iti te nui o ngā pereti toto), Anasarca (pupuhi o te tinana i te wai), Kirika , te ngoikore o te whatukuhu (tākihi), me te Organomegaly (te whakanui ake i te whēkau, i te ate rānei).
• iMCD me te Idiopathic Plasmacytic Lymphadenopathy (iMCD-IPL): I tēnei momo, ka piki pea te nui o ngā pereti toto. Tērā pea ka nui rawa ngā paturopi e whakaputaina ana e ō pūtau toto mā.
• iMCD kāore i te whakahuatia i tētahi atu huarahi (iMCD-NOS): Kāore hoki i te mōhiotia te tino take o tēnei momo, e kiia nei ko te iMCD-NOS, ā, kāore he hononga ki te TAFRO.

He aha ngā tohu o te mate Castleman?

He rerekē ngā tohu o te mate Castleman i runga i te momo e pāngia ana koe. I te nuinga o te wā, ki te mea he mate Castleman kotahi-pokapū tōu (UCD), kāore pea koe e kite i tētahi tohu nui. Ko ngā kōpū waikura pupuhi pea te tohu anake ka kitea e koe. Heoi, i ētahi wā ka puta ngā tohu mena ka pēhia e te kōpū waikura pupuhi tētahi atu okana tata.

Heoi, ki te mea he MCD tōu, he nui ake te tūponotanga ka kitea ngā tohu. Haunga ngā kōpū lymph e pupuhi ana, tera pea ka kitea e koe ngā tohu pēnei i:

• Kirika
• Te ngenge (he tohu pea tēnei o te anemia)
• Te werawera i te pō
• Whakapairuaki me te ruaki
• Te ngaronga taumaha mō te kore take
• Te pupuhi i ō waewae, i ō pona waewae, i tō puku rānei
• Te whēkau nui (splenomegaly) te ate nui rānei (hepatomegaly)
• Te ngoikore o ngā ringa me ngā waewae (peripheral neuropathy)

He aha te take o te mate Castleman?

Kāore anō kia mōhiotia te tino pūtake o te mate Castleman kotahi-pokapū (UCD), engari kua kitea ko te pangia e te huaketo e kiia nei ko te HHV-8 tētahi o ngā take o te whanaketanga o te mate Castleman maha-pokapū (MCD).

Kei te rangahau tonu ngā kaipūtaiao i ētahi atu take pea o te UCD me te iMCD. Hei tauira, kei te tirotirohia e rātou ngā mate urutā ehara i te HHV-8, ngā whakarerekētanga ira , me ngā urupare ārai mate .

Ko wai te hunga tino mōrearea ki te pangia e tēnei mate?

Kāore anō kia tautuhia ngā āhuatanga mōrearea motuhake mō te UCD, te iMCD rānei. Heoi, ki te mea kei a koe te mate HIV , tetahi atu mate rānei e ngoikore ai tō pūnaha ārai mate, ka nui ake tō tūponotanga ki te whakawhanake i te MCD e pā ana ki te HHV-8.

Ka taea te mate Castleman i ngā tau katoa, engari ko te nuinga o te wā ka kitea i waenga i te hunga i waenga i te 30 me te 60 tau te pakeke .

He aha ngā raruraru pea ka pā mai i te mate Castleman?

Ka whakanui ake te mate Castleman i tō tupono ki te pāngia e te mate pukupuku, inā koa te lymphoma .

Ahakoa he tino onge, ka tūpono te hunga whai UCD ki te whakawhanake i tētahi mate kino e kiia nei ko te Paraneoplastic Pemphigus (PNP) . Ka whakanui ake hoki te MCD i tō tupono ki te whakawhanake i ngā mate ka kino pea ō whekau. Ka taea e tēnei kino te whakararu i te ora mēnā kāore e rongoatia tika.

Kaua e manukanuka, ka aroturuki tonu tō tākuta i tō āhua, ā, ka āwhina i a koe ki te ārai i ēnei raruraru i ngā wā katoa e taea ai.

Me pēhea te tautuhi a ngā tākuta i te mate Castleman?

He rite tonu ngā tohu o te Mate Castleman ki ērā o ētahi atu mate noa, pērā i te rewharewha, te makariri rānei. Nō reira, ka whakakorea tuatahitia e tō tākuta ēnei mate. Kātahi ka whakahaerehia he whakamātautau hei tātaritanga i te Mate Castleman me te whakatau i te momo.

Ko ngā whakamātautau me ngā tikanga pea ko:

• Ngā whakamātautau taiwhanga: Ka tirohia e tō tākuta te maha o ngā pūtau toto mō ngā āhuatanga rerekē me ētahi atu tohu moroiti o te mate Castleman. Tērā pea ka tonoa he whakamātautau HIV . Mēnā he MCD e pā ana ki te HHV-8 tōu, tera pea he pai tō whakamātautau HIV.
• Ngā whakamātautau whakaahua: Ka taea e ngā whakamātautau pēnei i ngā karapa CT me ngā karapa PET te āwhina i te kimi i ngā pona lymph pupuhi i roto i tō tinana, me te tirotiro hoki mō ētahi atu tohu o te mate Castleman, pēnei i te ate, te whēkau rānei kua rahi.
• Te tirotiro i ngā kōpū lymph: Ko te huarahi anake hei mōhio māu mēnā kei a koe te mate Castleman ko te tirotiro i ngā kōpū lymph. Ko te tikanga o tēnei ko te tango i tētahi wahi iti o te kiko mai i te kōpū lymph, ka tirotirohia i raro i te karu hiko. Mā tēnei ka kitea mēnā he tohu kei te mate.

Me pēhea te rongoā i te mate Castleman?

He rerekē te maimoatanga mō te mate Castleman i runga i te momo e pāngia ana koe.

Te Maimoatanga o te Mate Castleman Unicentric (UCD)

Ko te maimoatanga matua mō te UCD ko te tango pokanga i ngā kōpū lymph e pāngia ana. I ētahi wā, me whakamahi pea koe i te rongoā irahiko , i te rongoā ārai mate rānei i mua i te pokanga. Ka whakaitihia e ēnei maimoatanga ngā tipu i roto i ngā kōpū lymph, kia māmā ake ai te tango.

Engari ki te kore e taea e ngā taote pokanga te tango i te kōpū lymph e pāngia ana, ā, kāore he tohu mōu, ka aroturuki pea tō tākuta i a ia, kaua e rongoā. Ki te mea he tohu tōu, ā, kāore e taea te pokanga, ka tūtohu pea rātou i ngā maimoatanga mō te MCD.

Te rongoā i te mate Castleman maha-pokapū (MCD)

He uaua ake te rongoā i te MCD i te UCD. Nā te mea kua horapa puta noa i te tinana, kāore i te whakamahia te pokanga, te rongoā irahiko rānei. Engari, ko te rongoā kei runga i te mate HHV-8 me te kaha o te mate.

Ka whakamahia pea e tō tākuta ngā maimoatanga pēnei i:

• Corticosteroids: Ka āwhina ēnei rongoā ki te whakaiti i te mumura me te whakaora i ngā tohu.
• Ngā rongoā chemotherapy: Ka whakahaere ngā rongoā chemotherapy i te tipu nui o ngā pūtau i roto i tō pūnaha lymphatic. Ko te Rituximab he rongoā e tohua ana mō te MCD e pā ana ki te HHV-8.
• Te rongoā ārai mate: Kei roto i tēnei maimoatanga te whakamahi i ngā mea pēnei i ngā paturopi monoclonal . Ka āwhina ēnei ki te whakamarie i te urupare ārai mate o tō tinana. Ko Siltuximab (Sylvant®) anake te maimoatanga kua whakaaetia e te U.S. Food and Drug Administration (FDA) mō te iMCD.
• Ngā rongoā ārai huaketo: Mena kei a koe te MCD e pā ana ki te HHV-8, ka hiahia pea koe ki ngā rongoā ārai huaketo hei rongoā i te mate HIV, i te mate HHV-8 rānei.

Āhea ahau me toro atu ki te tākuta?

Ki te kite koe i tētahi puku, i tētahi tipu rānei i roto i tō kaki, i tō kēkē, i tō hope rānei, me toro atu ki te tākuta tonu . Waihoki, ki te pā mai ētahi atu tohu o te Mate Castleman kua whakahuatia ake nei, ā, kāore e pai ake i muri i ētahi wiki, me kōrero anō hoki ki tō tākuta.

Kia pēhea te roa ka taea e koe te ora me tēnei mate?

He tino pai te āhua o te mate mō te nuinga o te hunga e pāngia ana e te UCD. Mā te tango i ngā kōpū lymph e pāngia ana ka taea te whakaora i te mate. Mēnā ka rongoatia, kāore te UCD e pā ki te roanga o te oranga.

Engari ko te tirohanga mō te hunga whai MCD e whakawhirinaki ana ki te momo MCD kei a rātou me te kaha o te mate. Mō ētahi tāngata, ka puta ohorere ngā tohu, ā, ka noho hei mea mōrearea ki te ora. Mō ētahi atu, ka roa pea ngā tohu. I waenga i te 65% me te 75% o te hunga kua tātaritatia he MCD kei te ora tonu i muri i ngā tau e rima . Mā ngā maimoatanga hou, kei te tumanakohia ka pai ake tēnei āhuatanga.

Kāore he whakautu ngāwari mō te mate Castleman. Ka whakawhirinaki tō wheako ki te momo mate Castleman kei a koe. Ka pā pea ki tō wheako ētahi atu mate, pērā i te HIV, ngā mate e pā ana rānei, pērā i te TAFRO me te POEMS. I ētahi wā, ko te pokanga me te tirotiro tata pea ngā mea e hiahiatia ana. Mēnā rānei, me whai momo maimoatanga koe puta noa i tō oranga hei ārai i ngā raruraru. Pātai atu ki tō tākuta me pēhea te pānga o tō tātaritanga ki tō maimoatanga me tō tirohanga.

Nā, he aha ngā mea tino nui hei maumahara mā tātou i tēnei?

• He mate pūnaha ārai mate onge engari he mate kino pea te mate Castleman, ā, ka kaha rawa te mahi a te pūnaha ārai mate.
• E rua ngā momo matua: ko te UCD me te MCD. I te UCD, ka pupuhi ngā pona lymph i te wāhi kotahi, i te MCD ia, ka pupuhi i ngā wāhi maha.
• He rerekē ngā tohu. I ētahi wā, kāore he tohu, he puku pupuhi noa iho. Tērā pea he tohu pēnei i te kirika, te ngenge, me te hekenga o te taumaha.
• Kāore i te mārama tonu ngā pūtake, engari kua honoa te huaketo HHV-8 ki te MCD. He nui ake te mōrearea o te hunga whai HIV.
• Ka taea e te kitenga wawe me te maimoatanga tika te arahi ki ngā hua pai. Nō reira, ki te kite koe i tētahi puku rerekē, pupuhi, ētahi atu tohu rerekē rānei i tō tinana, rapua tonutia he tohutohu hauora. Ko te mea nui ko te noho mōhio, kaua hoki e mataku.

` Te Mate Castleman, Ngā Pūnaha Lymph, Lymphadenopathy, Pūnaha Ārai Mate, UCD, MCD, HHV-8, Ngā Tohu, Te Maimoatanga