Kia ako tātou mō te Anaplastic Large Cell Lymphoma (ALCL) i roto i ngā kupu māmā noa iho.

Kua rongo kē koe i te Anaplastic Large Cell Lymphoma (ALCL)? Tērā pea he ahua uaua tēnei ingoa. Kaua e manukanuka, he momo mate pukupuku tino onge tēnei. I tēnei rā ka kōrero tātou mō te ALCL, me pēhea te whanaketanga, ngā tohumate, ngā maimoatanga, me te maha atu o ngā mea i roto i te huarahi tino māmā ka taea e koe te mārama. Pērā i te kōrero ki tētahi hoa.

He aha te ALCL (Lymphoma Pūtau Nui Anaplastic)?

He whakarāpopototanga noa iho, he momo mate pukupuku onge, he momo onge hoki te ALCL, nō tētahi rōpū nui ake o ngā mate pukupuku e kiia nei ko te Non-Hodgkin Lymphoma . I mōhio koe, pērā i ngā Non-Hodgkin Lymphoma katoa, he momo mate pukupuku anō hoki te ALCL? Ka puta mai ina tipu haere tētahi momo toto mā e kiia nei ko ngā lymphocytes i roto i tō tātou tinana, ā, he tere ake i tōna tere e tika ana.

Whakaarohia ēnei pūtau totohu (limfocyte) he hōia ririki i roto i ō tātou tinana. He wāhanga rātou o tō tātou pūnaha ārai mate, e tiaki ana i a tātou mai i ngā iroriki me ngā mate. E rua ngā momo matua o ngā pūtau totohu: ngā pūtau B me ngā pūtau T. I roto i te ALCL, ka tipu rerekē ō pūtau totohu T, ngā pūtau T rānei.

Nā, kia titiro tātou ki te tikanga o tēnei ingoa "anaplastic large cell lymphoma".

• Anaplastic : Ko te tikanga o tēnei kupu, ina tirohia i raro i te karuārai, he tino rerekē te āhua o ēnei pūtau matepukupuku ki ērā o ngā pūtau T lymphocyte hauora. Me te mea kua whakapōturitia ō rātou āhua me te āhua.
• Pūtau Nui : Ko te tikanga o tēnei, he nui ake te āhua o ngā pūtau-T lymphocytes whanonga rerekē i ngā pūtau-T noa. Ka kitea mārama tēnei rerekētanga i raro i te karuārai.
• Lymphoma : Ko te Lymphoma he mate pukupuku e puta mai ana i te tipu kore mana o ngā pūtau toto mā e kiia nei ko ngā lymphocytes. Ka puta motuhake te ALCL mai i ngā lymphocytes T.

Kei te mārama koe? He kupu ngāwari noa iho, he mate pukupuku te ALCL e nui rawa atu ai ngā pūtau-T, ka whakarerekētia ō rātou āhua, ka tipu kore e taea te whakahaere.

He rerekē ngā momo ALCL?

Ae, he rerekē ngā momo ALCL. Ka whakarōpūtia ēnei i runga i te wāhi kei reira te matepukupuku i roto i tō tinana me ngā āhuatanga o ngā pūtau matepukupuku.

ALCL Pūnaha

Koinei te momo matua. Ko te tikanga o te "pūnaha" ka pā ki te tinana katoa. Ka pā ki ō pona lymph , arā, ki ngā "repe" e kīia nei e tātou, me ngā whekau o roto pērā i tō ate me ō pukahukahu, ā, i ētahi wā ko tō kiri.

E rua ngā momo o te ALCL pūnaha. Ko tētahi e pā ana ki te mea kei roto i tētahi ira te whakarerekētanga ira e kiia nei ko te ira "Anaplastic Lymphoma Kinase (ALK)" . He rite ngā ira ki ngā pukapuka tohutohu iti mō ā tātou pūtau, ā, ka tohutohu ēnei ira ki tētahi pūtau toto-T me pēhea te mahi.

• ALK-pai ALCL: He momo mate pukupuku tēnei he whakarerekētanga ira kei roto i te ira ALK. He mate pukupuku kaha tēnei i te nuinga o te wā. Heoi, he nui ake te kitea i roto i ngā tamariki me ngā taiohi . Waimarie, he pai te urupare a tēnei momo ki te chemotherapy .
• ALCL ALK-kino : Kāore he whakarerekētanga ira ALK i tēnei momo. He mate pukupuku tere tipu (kaha) hoki tēnei. Heoi, ko te nuinga o te wā ka pā ki te hunga kaumātua , ko te hunga neke atu i te 60 tau te pakeke. Tērā pea ka memeha haere tēnei momo i muri i te rongoā chemotherapy, kātahi ka hoki anō. Nō reira, he uaua ake te rongoā i te ALCL ALK-kino, ā, he kino ake te matapae i te ALCL ALK-pai.

Ētahi atu momo ALCL

Kei reira anō ngā momo ALCL e puhoi ake ana te tipu i ngā momo pūnaha katoa.

• ALCL kiri tuatahi : He momo tēnei e pā nui ana ki te kiri. Tērā pea ka puta ngā tohu pēnei i te pupuhi, te pupuhi, me te ekisema. I te nuinga o te wā (tata ki te 90%) kāore e horapa ki tua atu i te kiri.
• ALCL e pā ana ki te whakatō ū (BIA-ALCL) : He momo ALCL tēnei e puta ake ana i te taha o ngā whakatō ū. I te nuinga o te wā ka kitea i muri i te 10 tau i muri i te mahi whakanui ū, te mahi hanga anō rānei i te ū.

Ko wai ka whiwhi i te ALCL?

Ahakoa ka taea e te ALCL te whanake i roto i te tangata o ngā tau katoa, he nui ake te pānga o ētahi momo ki ētahi rōpū.

• ALK-pai ALCL : Ko te nuinga o te wā ka pāngia ngā tamariki kōhungahunga, ngā taiohi, me ngā pakeke taitamariki kei roto i ō rātou tau 20 me 30. He nui ake te kitea i roto i ngā tāne .
• ALK-kino ALCL : Ko te nuinga o tēnei ka pā ki te hunga neke atu i te 60 tau te pakeke. He paku noa ake hoki te kitea i roto i ngā tāne.
• ALCL kiri tuatahi : Ko te nuinga o te wā ka pā tēnei ki ngā pakeke neke atu i te 40 tau te pakeke. He nui ake te kitea i roto i ngā tāne me ngā tāngata mā.
• BIA-ALCL : Ko te tikanga ka kitea tēnei i roto i te hunga kei roto i ō rātou tau 50. Ka taea te whanake i roto i te hunga kua whakauruhia he ū silicone me he ū saline. Kua kitea he hononga nui tēnei ki ngā ū kakano .

He pēhea te noa o te ALCL?

He mate pukupuku tino onge te ALCL. Tata ki te 2% noa iho o ngā lymphoma kore-Hodgkin i roto i ngā pakeke ka kitea he ALCL. He ōrau iti rawa tēnā.

He aha ngā tohu o te ALCL?

He rerekē ngā tohu o te ALCL i runga i te momo.

Ngā tohu o te ALCL pūnaha

I roto i ngā ALCL ALK-pai me ALK-kino, he mea noa ngā pona lymph pupuhi ki ngā wāhi e tipu ana te mate pukupuku. Tērā pea ka rite ki ngā puku i ngā wāhi pēnei i tō kaki, i ō ringaringa, i tō hope rānei . Ko ētahi atu tohu ko:

• Te kirika pinepine.
• Te tino ngenge (‘Te ngenge nui’).
• Te werawera i te pō (‘Night sweats’).
• Te whakahekenga taumaha kāore i te mārama.

Ina kitea te nuinga o te tangata, tera pea kua horapa te mate pukupuku, arā, kei roto i te 'wāhanga whakamua' . Mēnā koinei te āhua, tera pea kua horapa te mate pukupuku ki ngā whekau pēnei i ō pukahukahu, ate, wheua rānei. Kātahi ka puta ngā tohu i runga i te whekau e pāngia ana. Hei tauira, ki te mea he kikī tō uma, he maremare auau rānei, tera pea kua pā te ALCL ki te uma.

Ngā āhuatanga o te ALCL kiri tuatahi

Ka kitea ngā panoni kiri i tēnei momo.

• Ka puta ngā puku whero, parauri-whero rānei, rerekē ki tētahi, ki ētahi wāhi rānei o te kiri, ā, ka nui haere pea i roto i te wā.
• He puku e ara ake ana , he nui ake i te moni. Ka mākūkū pea ēnei i ētahi wā.
• Tērā pea ka mamae ngā puku, kātahi ka huri hei pāpaka .

Ngā Āhuatanga o te BIA-ALCL (Breast Implant-Associated ALCL)

I roto i tēnei, ka kitea ngā huringa mārama o ngā ū.

• Mamae o te ū.
• Te pupuhi o te ū.
• He ponana kiri, he ekisema rānei.
• Te pupuhi, te kohikohi wai rānei e tata ana ki te whakaurunga o tō ū.
• He puranga totoka e tata ana ki te whakaurunga ū.

Ka kitea ēnei huringa i te nuinga o te wā i te ū kotahi, engari i ētahi wā ka kitea ngā huringa i ngā ū e rua.

He aha ngā take o te ALCL?

Ka puta te ALCL ina nui haere ngā pūtau lymphocyte i kōrerotia i mua ake nei, ā, ka tīmata te tipu ki tua atu i ngā kiko hauora e karapoti ana i a rātou. Kāore anō kia mōhiotia e ngā kairangahau he aha i huri ai ēnei pūtau lymphocyte hei matepukupuku (ngā pūtau kino).

Heoi, kua kitea e rātou ētahi whakarerekētanga ira e kitea whānuitia ana me te ALCL. E whakaarohia ana ka taea e ēnei whakarerekētanga ira te huri i ngā pūtau hauora hei pūtau matepukupuku.

Hei tauira, ko te āhuatanga matua o te ALK-positive ALCL ko ngā whakarerekētanga ira i roto i te ira ALK . Waihoki, he mea noa anō hoki ētahi atu whakarerekētanga ira i roto i ētahi atu momo ALCL.

Me pēhea te tautuhi i te ALCL? (Tautuhinga)

Ka tirohia tuatahitia e tō tākuta he tohu o te ALCL, pērā i te pupuhi o ngā kōpū lymph. Mēnā ka whakapaetia tēnei e rātou, ka mahia e rātou ētahi whakamātautau me ngā tikanga rerekē.

• Ngā tikanga whakaahuaKa āwhina ēnei ki te tautuhi i te wāhi kei reira te matepukupuku. Ka rerekē te momo whakamātautau whakaahua e hiahiatia ana e koe i runga i te momo ALCL. Tērā pea ka mahia e tō tākuta he hihi-X o te uma , he matawai CT , he MRI rānei hei rapu i ngā pukupuku. Ka āwhina te matawai PET/CT, te matawai PET rānei ki te whakatau mēnā kua horapa te matepukupuku puta noa i te tinana. Mēnā e whakapaetia ana he BIA-ALCL, ka taea te mahi i tētahi tirotiro ultrasound o te ū .
• Te whakamātautau toto : Ka whakahaerehia e te tākuta ngā whakamātautau toto maha, pērā i te tatau toto katoa (CBC), hei tirotiro i ngā tohu o te ALCL. Mena he rerekē te maha o ngā pūtau toto (ngā pūtau toto whero, ngā pūtau toto mā, ngā pereti toto rānei), he tohu pea tēnei o te ALCL. Ka rapua hoki e rātou ētahi whākōkī me ētahi atu tohu i roto i te toto, nā te mea he tohu pea ēnei o te ALCL.
• Kohikohi pūtau : Ko te huarahi anake hei whakaū i te mate ALCL ko te whai kohikohi pūtau. I roto i te kohikohi pūtau, ka tangohia e te tākuta he tauira iti o te kiko whakapae ka tirohia i raro i te karuārai. Mā te ako i ēnei pūtau, ka taea e te tākuta te whakatau tika i te momo ALCL kei a koe. Ka āwhina hoki tēnei ki te whakamahere i te maimoatanga tino whai hua.

Me pēhea te rongoā mō te ALCL?

Ko te rongoā matū te rongoā e whakamahia whānuitia ana mō te ALCL pūnaha. Ko te pokanga te rongoā pai rawa atu mō te ALCL kiri tuatahi me te BIA-ALCL.

Te maimoatanga o te ALCL pūnaha

Ko ngā lymphoma pūtau nui anaplastic ALK-positive me ALK-negative he pai te urupare ki te chemotherapy pūnaha. Ko te chemotherapy pūnaha ko te hoatu i ngā rongoā whakamate matepukupuku mā roto i te uaua, e haere ana puta noa i te tinana mā roto i te toto ki te whakamate i ngā pūtau matepukupuku.

Ko te rongoā matū mō te ALCL pūnaha ka whakamahia he huinga o ngā rongoā e mahi tahi ana hei patu i ngā pūtau matepukupuku. Ko ētahi tauira ko:

• BV-CHP : Kei roto i tēnei ko ngā rongoā `Brentuximab Vedotin`, `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, me `Prednisone`.
• CHOP : Kei roto i tēnei ko te whakamahinga o ngā rongoā e kiia nei ko te `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, `Vincristine (Oncovin®)`, me te `Prednisone`.
• CHEOP : Kei roto i tēnei ko te whakamahinga o ngā rongoā e kiia nei ko te `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, `Etoposide`, `Vincristine (Oncovin®)`, me te `Prednisone`.

I te nuinga o te wā ka taea e ēnei maimoatanga te whakakore i ngā tohu katoa o te mate pukupuku i roto i te wā poto (e kiia ana tēnei he 'mutunga mate').(Ka kiia tēnei he "hokinga anō") Heoi, ka taea e te mate pukupuku te hoki mai anō. Mena kei te whakaaro tō tākuta ka hoki mai anō te mate pukupuku, ka tūtohu pea rātou i te whakawhitinga pūtau take i a koe e noho ana i te wā e mārire ana. Ka whakakapia ngā pūtau kua whakangaromia e te chemotherapy e te whakawhitinga pūtau take ki ngā pūtau hauora. Kātahi ka whakaputahia e ēnei pūtau hauora ngā pūtau toto hauora (ehara i te mate pukupuku).

Ki te hoki mai te mate pukupuku, ka tūtohu pea tō tākuta i ētahi atu maimoatanga chemotherapy kua hangaia hei rongoā i te lymphoma.

Te maimoatanga o te ALCL kiri tuatahi

Ko te pokanga te maimoatanga tino noa mō tēnei. Mena kua horapa te mate pukupuku ki ngā kōpū lymph, me whakamahi pea koe i te rongoā irahiko . Ko te mahi a te rongoā irahiko mā te tuku i te pūngao ki ngā pūtau mate pukupuku, me te whakangaro i a rātou.

Ka taea e te ALCL kiri tuatahi te hoki mai anō i roto i ngā tau e rima o te maimoatanga. Mēnā ka tupu tēnei, me mahi anō te pokanga me te rongoā irahiko.

Mena he pukupuku ā-rohe tōu e kore e taea te tango mā te pokanga, ki te hoki mai anō rānei te mate pukupuku, me hiahia pea koe ki ētahi atu maimoatanga. Kei roto i ēnei ko:

• Methotrexate ā-waha : He rongoā e whakamahia ana hei rongoā i te lymphoma kore-Hodgkin.
• Bexarotene : He rongoā e whakamahia ana hei rongoā i te lymphoma pūtau-T e pāngia ana te kiri.
• Brentuximab Vedotin : He rongoā e whakamahia ana hei rongoā i te ALCL pūnaha me ētahi atu momo ALCL e hokihoki ana.
• Interferon : He rongoā e āwhina ana i tō pūnaha ārai mate ki te kite me te whawhai ki ngā pūtau matepukupuku, tae atu ki te lymphoma.

Te maimoatanga o te BIA-ALCL

Ko te tikanga, ka taea te rongoā i te BIA-ALCL mā te pokanga hei tango i tō ū tāpiringa me ngā pūtau matepukupuku e karapoti ana. Mēnā kāore e taea te pokanga, ka taea te hoatu he rongoā irahiko hei patu i ngā pūtau matepukupuku.

Mena ka hoki mai te mate pukupuku, ka horapa rānei ki tua atu i te ū, ka tūtohu pea tō tākuta i ngā maimoatanga chemotherapy hei rongoā i te ALK-negative ALCL. Kei roto pea i ēnei ko te chemotherapy CHOP me ngā maimoatanga pēnei i te Brentuximab Vedotin.

He pēhea te kino o te ALCL?

Ahakoa te momo, he mate pukupuku kino te ALCL e hiahia ana kia āta aroturukitia me te maimoatanga tika. Heoi, ko tō matapae me tō mahere maimoatanga e whakawhirinaki ana ki ngā āhuatanga pēnei i te momo ALCL kei a koe, te āhua o tō urupare ki te maimoatanga, me tō kaute International Prognostic Index (IPI) .

I whakawhanakehia te IPI e ngā tākuta mate pukupuku hei āwhina i te matapae i ngā putanga o ngā lymphoma kore-Hodgkin e tere tipu ana, tae atu ki te ALCL pūnaha. Ko te kaute e ahu mai ana i ngā āhuatanga e whai ake nei:

• tō tau.
• Tō kaha ki te mahi i ngā mahi o ia rā.
• Te horapa o tō matepukupuku (te wāhanga o te matepukupuku).
• Te maha o ngā repe hinu e pāngia ana.

Mena kei a koe te ALCL pūnaha, pātai atu ki tō tākuta me pēhea te pānga o tō kaute IPI ki tō tupono ki te ora.

He aha te auau oranga mō te ALCL?

He rerekē ngā reiti oranga mō te ALCL pūnaha. Ko te ALCL ALK-pai e pā ana ki te wā oranga roa ake i te ALCL ALK-kino. I runga i ngā āhuatanga pēnei i tō momo mate pukupuku me te kaute IPI, ko te reiti oranga rima tau mō te ALCL ALK-pai ka taea te horapa mai i te 33% ki te 90%. Waihoki, ko te reiti oranga rima tau mō te ALCL ALK-kino ka taea te horapa mai i te 13% ki te 74%.

He tino pai ngā reiti oranga o te ALCL kiri tuatahi me te BIA-ALCL. Ahakoa ka memeha haere te ALCL kiri tuatahi, ā, ka hoki mai anō i roto i ngā tau e rima, ka taea e ngā maimoatanga whai muri te whakaroa i tō oranga. Ko te reiti oranga e tata ana ki te 80% i muri i te rima tau i muri i te tātaritanga. I roto i te BIA-ALCL, ki te tangohia katoatia te mate pukupuku mā te pokanga, he maha ngā wā kua ngaro katoa te mate pukupuku.

He aha ngā pātai me pātai atu e au ki taku tākuta?

Anei ētahi pātai nui hei pātai atu ki tō tākuta:

• He aha te momo ALCL kei a au?
• Kei te kotahi noa te wāhi e noho ana taku mate pukupuku, kua horapa rānei?
• Ko wai ngā tohunga i roto i taku tīma tiaki?
• He aha te maimoatanga e taunaki ana koe māku?
• Me pēhea taku whakahaere i ngā pānga taha o te maimoatanga?
• He aha taku kaute IPI?
• He aha ngā tūponotanga ka "ora" taku mate pukupuku?
• He aha ngā tūponotanga ka hoki mai anō taku mate pukupuku?
• Kia pēhea te auau o taku haerenga atu ki te tirotiro i tōku āhua?

He mea noa te mataku ina rongo koe he mate pukupuku tōu. Heoi, ko te mea nui kia maumahara ko te ALCL ehara i te momo mate pukupuku kotahi me te putanga ōrite . Ko te momo ALCL kei a koe e whakatau ana mēnā ka horapa tere, ka puhoi rānei. Mā tēnā ka hanga i ō whiringa maimoatanga me tō tirohanga. Ka horapa tere te ALCL o ētahi tāngata. Mō ētahi atu, ka puhoi te tipu, ka nui te wā hei whiwhi maimoatanga hei āwhina i te whakahaere i te mate pukupuku. Kōrero ki tō tākuta mō ngā putanga pea i runga i tō tātaritanga.

Ngā mea tino nui hei maumahara māu (Karere hei kawe ki te kāinga)

Āe, me whakarāpopoto tātou i ētahi o ngā mea tino nui hei maumahara māu mai i ngā mea kua kōrerohia e tātou.

• He momo mate pukupuku onge te ALCL, ā, he rerekē ngā momo.
• Kāore e rite ana ngā momo ALCL katoa. Ka horapa tere ētahi, ka pōturi ētahi.
• Ka rerekē te maimoatanga me ngā tūponotanga o te ora i runga i te momo ALCL kei a koe, tō tau, me tō hauora whānui.
• Ahakoa he maha ake ngā wā ka puta te ALK-positive ALCL i roto i te hunga rangatahi, he pai tonu te urupare ki te maimoatanga.
• He nui ake ngā pakeke e pāngia ana e te ALK-kino ALCL, ā, he uaua ake te rongoā.
• He pai te matapae mō te ALCL kiri me te ALCL e pā ana ki te whakatō ū (BIA-ALCL).
• Kaua e mataku. Ki te mea he ruarua, he tohu rānei āu, rapua tonutia he tohutohu hauora.
• Kōrerotia mārire ki tō tākuta mō tō āhua, tō maimoatanga, me ō pātai katoa. Ko rātou te hunga ka taea te āwhina pai i a koe.

Kia maumahara, ehara i te mea he rite te kino o ngā tātaritanga mate pukupuku katoa. Ahakoa he ALCL, me te tātaritanga tika, te maimoatanga tere, me te aroturukitanga pai, he tokomaha ngā tāngata ka taea te whakatutuki i ngā putanga angitu.

` Lymphoma Pūtau Nui Anaplastic, ALCL, Lymphoma, Matepukupuku, Pūtau T, Ira ALK, Chemotherapy