Ngā mea e hiahia ana koe kia mōhio mō te mate Castleman

Kua pāngia koe e tētahi puku, e kiia nei e mātou he "puku," i tō kaki, i tō kēkē, i tō hope rānei? I ētahi wā, ka pupuhi ngā puku pēnei i te wā e pāngia ana koe e te makariri noa, e tētahi momo mate pāwera rānei. He mea noa tēnā. Engari ki te mea he puku pupuhi pēnei āu mō te wā roa, kāore he take, ā, kei te ngenge hoki koe, kei te heke hoki tō taumaha, me āwangawanga koe. I tēnei rā, ka kōrero tātou mō tētahi mate onge ka taea te whakapae i ēnei wā. Ko te Mate Castleman tēnā.

He aha te mate Castleman?

Whakaarohia he rite tō tātou tinana ki tētahi whenua. Kātahi ko tō tātou pūnaha ārai mate te ope taua e tiaki ana i taua whenua. Ina uru mai tētahi iroriki (hoariri) ki roto i te tinana mai i waho, ka haere tēnei ope ki te pakanga ka hinga i taua hoariri. Kia mutu te pakanga, ka hoki te ope ki te pā, ka noho puku. Koinei te mea e tupu ana i roto i te tinana o te tangata hauora.

Engari he rerekē te āhua mō te tangata e pangia ana e te mate a Castleman. Ko te mea e tupu ana i konei, ko te ope taua i haere ki te pakanga, arā, te pūnaha ārai mate, kāore e marino ahakoa kua mutu te pakanga. Kei te noho pukumahi tonu rātou. Me te mea kei te rite tonu rātou mō te pakanga. Ka puta te mumura roa i roto i te tinana. Ka taea e tēnei te kino i ō tātou whekau.

I tēnei wā, ko ō tātou pona lymph, aua mea ririki, he rite ki ngā turanga hōia. He nui ā rātou mahi, pērā i te tātari i ngā iroriki me te penapena i ngā pūtau ārai mate. Ināianei, nā te mea kei te mahi tonu te pūnaha ārai mate, kei te mahi tonu hoki ēnei pona lymph. Nō reira, ka tīmata te tipu nui o ngā pūtau i roto i a rātou. Koia te take e pupuhi ai ngā pona lymph i te mate a Castleman. Ehara i te mea he pupuhi noa iho, engari ka puta he huringa ki te kiko o aua pona lymph.

He aha ngā momo matua o te mate Castleman?

E rua ngā momo matua o te mate Casselman. He rerekē rawa ngā tohu me ngā maimoatanga mō ia momo. Nō reira, he mea nui kia mārama pai ki ēnei momo e rua.

He momo āhua anō kei te mate Castleman maha-pokapū (MCD)?

Āe, kua wehea anō te MCD ki ētahi momo momo. Ka mahia tēnei whakarōpūtanga i runga i te take me ētahi atu āhuatanga e pā ana ki tēnei.

• MCD e pā ana ki te POEMS: He mate toto tino onge te POEMS. I ētahi wā ka puta tahi te MCD me te POEMS.
• MCD e pā ana ki te HHV-8: Ka puta tēnei i te mate urutā e kiia nei ko te Human Herpes Virus-8 (HHV-8). He tino noa tēnei momo i roto i te hunga kua pangia e te HIV , kua ngoikore rānei te pūnaha ārai mate mō ētahi atu take.
• MCD Idiopathic (iMCD): Ko te tikanga o te kupu hauora " idiopathic " ko "kāore anō kia kitea he take." Ko te nuinga o ngā tūroro MCD he momo MCD tēnei. Ko te tikanga kāore anō kia mōhiotia te take tuturu.

Kua wehea anō tēnei momo iMCD ki ngā wāhanga iti e toru:

1. iMCD e pā ana ki te TAFRO: Ko te ingoa TAFRO i ahu mai i te huinga o ētahi tohu. Arā, ko te Thrombocytopenia (ngā pereti toto iti), te Anasarca (te pupuhi o te tinana i te wai), te Kirika (te kirika), te ngoikore o te whatukuhu (te ngoikore o te mahi whatukuhu) me te Organomegaly (te whakanui o te ate, te whēkau rānei) ngā tohu ka kitea i roto i tēnei.

2. iMCD me te idiopathic plasmacytic lymphadenopathy (iMCD-IPL): I tēnei momo, ka piki ake pea te maha o ngā pereti toto. Tērā pea ka nui rawa te hanga o ngā paturopi e ngā pūtau toto mā.

3. iMCD, kāore i te whakahuatia i tētahi atu wāhi (iMCD-NOS): Ka taka ngā tūroro me te iMCD kāore i te whanaunga ki a TAFRO, ā, kāore hoki e kitea he take motuhake mō rātou ki tēnei wāhanga.

He aha pea ngā tohu o tēnei mate?

Ka rerekē ngā tohu i runga i te momo mate a Castleman kei a koe.

Ko te mate Unicentric Casselman (UCD) kāore he tohu . Ko te tohu anake he pupuhi o te kōpū lymph (adenoid). I ētahi wā, ka puta noa ngā tohu (hei tauira, te mamae, te poto o te manawa) ina pēhia te kōpū pupuhi ki tētahi atu okana.

Heoi, i roto i te mate Castleman maha-pokapū (MCD), ka kitea ake ngā tohu. Haunga ngā repe pupuhi, ka pāngia pea koe e:

• Te kirika auau
• Te tino ngenge me te ngenge, kāore he take (tērā pea nā te anemia tēnei)
• Te werawera nui i te pō
• Whakapairuaki me te ruaki
• Te whakahekenga taumaha ohorere
• Te pupuhi o ngā waewae, ngā pona, te puku rānei
• Te whēkau nui (splenomegaly) te ate nui rānei (hepatomegaly)
• Te ngoikore o ngā ringa me ngā waewae (peripheral neuropathy)

He aha i puta ai tēnei mate? He āhuatanga mōrearea kei reira?

Ina hoki, kāore anō kia kitea e ngā kaipūtaiao tētahi tino take mō te mate Castleman kotahi-pokapū (UCD) .

E pā ana te mate Castleman maha-pokapū (MCD) ki te huaketo HHV-8 kua whakahuatia ake nei. Heoi, kei te rangahau tonu ngā kairangahau mēnā ko ētahi atu mate, ngā whakarerekētanga ira, ngā urupare ārai mate rānei te take o te momo iMCD kāore i te mōhiotia.

Mō ngā āhuatanga mōrearea, kāore he āhuatanga mōrearea e mōhiotia ana mō te UCD me te iMCD. Heoi, ki te mea kei a koe te mate HIV, te ngoikore rānei o tētahi atu pūnaha ārai mate, he nui ake tō tūponotanga ki te whakawhanake i te MCD e pā ana ki te HHV-8.

Ahakoa ka taea e tēnei mate te pā ki ngā tāngata o ngā tau katoa, ko te nuinga o te wā ka pā ki te hunga i waenga i te 30 me te 60 tau te pakeke .

He aha ngā raruraru pea ka pā mai i tēnei mate?

Tērā pea ka paku whakanuia e te mate Castleman tō tupono ki te whakawhanake i ētahi atu āhuatanga, inā koa ko ngā mate pukupuku , pērā i te lymphoma, he mate pukupuku o te pūnaha lymphatic.

Hei tāpiritanga, ko te hunga whai UCD kei te mōrearea ki te whakawhanake i tētahi mate kiri onge engari he kino e kiia nei ko te paraneoplastic pemphigus (PNP). He nui ake te mōrearea o te hunga whai MCD ki te whakawhanake i ngā momo mate. Mēnā kāore e rongoatia, ka taea e ēnei mate te whakararu i ngā okana, tae atu ki te mate.

Kaua e manukanuka, ka aroturukihia tonutia tēnei āhuatanga e tō tākuta, ā, ka mahi i ngā mahi e tika ana hei āwhina i te aukati i ēnei raruraru i ngā wā katoa e taea ana.

Me pēhea te tātaritanga tika a te tākuta i tēnei mate?

Nā te mea he rite ngā tohu o te mate a Castleman ki ērā o ētahi atu mate, pērā i te makariri noa, te rewharewha rānei, ka tirohia tuatahitia e te tākuta mēnā he mate noa. Kātahi ka whakapaehia te mate a Castleman, ā, ka whakahaerehia he whakamātautau motuhake.

Ko ngā whakamātautau e whai ake nei ka mahia i te nuinga o te wā:

• Ngā whakamātautau taiwhanga: Ka tirohia tō toto i raro i te karuārai hei tirotiro i ngā hapa o te maha o ngā pūtau toto me ētahi atu hapa. Mena kei te whakapaetia he MCD e pā ana ki te HHV-8, ka taea hoki te mahi i tētahi whakamātautau HIV.
• Ngā whakamātautau whakaahua: Ka taea e ngā karapa CT me ngā karapa PET te āwhina ki te tautuhi i ngā wāhi kei reira ngā pona lymph pupuhi i roto i te tinana. Ka taea hoki e rātou te tirotiro i te nui haere o ngā whekau pēnei i te ate me te whēkau.
• Ko te koiora o ngā kōpū lymph: Koinei anake te huarahi tino whai hua hei whakaū i te mate . Ko te tikanga o tēnei ko te tango i tētahi wāhanga iti rawa o te kiko mai i te kōpū lymph pupuhi, ka tirotirohia i raro i te karuārai. Mā tēnei ka taea te tino mōhio mēnā he huringa pūtau motuhake e pā ana ki te mate Casselman.

He aha ngā maimoatanga mō tēnei?

He rerekē rawa ngā tikanga maimoatanga i runga i te momo mate.

Te Maimoatanga o te Mate Casselman Unicentric (UCD)

Ko te maimoatanga matua me te tino angitu mō te UCD ko te tango pokanga i te kōpuku lymph e pāngia ana . I te nuinga o te wā, ka ora katoa te mate i muri i tēnei pokanga.

I ētahi wā, mēnā he nui te puku, ka hoatu te rongoā irahiko, te rongoā ārai mate rānei i mua i te pokanga hei whakaiti, hei whakangāwari hoki i te tango.

Mena kei tētahi wāhi e kore e taea te tango mā te pokanga te puku, ā, kāore he tohu, ka whakatau pea tō tākuta kia kaua e rongoātia, engari kia mātakitaki noa iho. Heoi, mō tētahi kāore e pai ki te pokanga, ā, kei te mau tonu ngā tohu, ka taea te hoatu i te maimoatanga kotahi ki te MCD.

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

Nā te mea he mate te MCD e horapa ana ki ngā wāhanga maha o te tinana, kāore e taea te whakaora mā te pokanga, mā te rongoā irahiko rānei. Nō reira, ka whakamahia ētahi atu maimoatanga. Ko te maimoatanga e whakawhirinaki ana ki te HHV-8 kei a koe me te kaha o te mate.

Ko ngā maimoatanga e whai ake nei ka whakaratohia te nuinga:

• Corticosteroids: Ka āwhina ēnei rongoā ki te whakahaere i ngā tohu mā te whakaiti i te mumura i roto i te tinana.
• Ngā rongoā chemotherapy: Ka mahi ēnei mā te whakahaere i te tere o te tipu o ngā pūtau i roto i te pūnaha lymphatic. He mea noa te whakamahi i te Rituximab mō te MCD e pā ana ki te HHV-8.
• Te rongoā ārai mate: Ka mahi ēnei maimoatanga mā te whakamarie i te pūnaha ārai mate o tō tinana e kaha rawa ana te mahi. Ko Siltuximab (Sylvant®) anake te rongoā kua whakaaetia mō te iMCD.
• Ngā rongoā ārai huaketo: Mena kei a koe te MCD e pā ana ki te HHV-8, ka hoatu pea ēnei rongoā ki a koe hei whakahaere i te mate HIV, i te mate HHV-8 rānei.

Kia pēhea te wā me toro atu ahau ki te tākuta?

Ki te kite koe i tētahi puku, i tētahi puku rānei i tō kaki, i tō kēkē, i tō hope rānei, me kōrero ki tō tākuta .

Waihoki, ki te roa tonu ngā tohu kua whakahuatia i roto i tēnei tuhinga, pērā i te kirika kore e taea te whakamārama, te whakahekenga taumaha, me te ngenge nui, mō ētahi wiki, me rapu tohutohu hauora.

Ka taea te ora me tēnei mate?

Ko te whakautu ki tēnei pātai e whakawhirinaki ana ki te momo Casselman kei a koe.

He tino pai ngā hua ka puta mai i te tangata whai UCD . Ina tangohia te pukupuku mā te pokanga, ka ora katoa te mate. Whai muri i te maimoatanga, kāore tēnei e pā ki tō roanga o te oranga.

He ahua uaua te matapae mō te hunga whai MCD . He rerekē i runga i te momo me te kaha o te mate. Mō ētahi, ka taea e ngā tohu te huri ohorere hei kino, ā, ka mōrearea te ora. Mō ētahi atu, ka roa pea ngā tohu, engari he tumanako anō kei reira. I waenganui i te 65% me te 75% o te hunga kua tautuhia he MCD ka ora mō te neke atu i te rima tau . Kei te pai haere tēnei me ngā maimoatanga hou.

Kāore he whakautu māmā, kotahi te rahi e tika ana mō te mate Casselman. Ka whakawhirinaki ki tō wheako, ki te momo mate kei a koe, me te mea kei a koe ētahi atu mate, pēnei i te HIV, te TAFRO, te POEMS rānei. I ētahi wā, ka ranea te pokanga me te aroturuki hauora tonu. Mēnā rānei, me whiwhi pea koe i ngā maimoatanga maha puta noa i tō oranga. Nō reira, he pai ake te kōrero tuwhera ki tō tākuta mō tō mate, tō maimoatanga, me tō heke mai.