Ake sifunde nge-Anaplastic Large Cell Lymphoma (ALCL) ngamagama alula.

Wake wezwa nge-Anaplastic Large Cell Lymphoma (ALCL)? Mhlawumbe leli gama lizwakala liyinkimbinkimbi kancane. Kodwa ungakhathazeki, lolu uhlobo lomdlavuza olungavamile kakhulu. Namuhla sizoxoxa ngokuthi iyini i-ALCL, ukuthi iqala kanjani, yiziphi izimpawu, yiziphi izindlela zokwelapha, kanye nezinye izinto eziningi ngendlela elula kakhulu ongayiqonda. Njengokukhuluma nomngane.

Kuyini i-ALCL (i-Anaplastic Large Cell Lymphoma)?

Kalula nje, i-ALCL uhlobo lomdlavuza olungavamile nolungajwayelekile oluyingxenye yeqembu elikhulu lomdlavuza olubizwa ngokuthi i-Non-Hodgkin Lymphoma . Ubuwazi ukuthi njengazo zonke i-Non-Hodgkin Lymphoma, i-ALCL nayo uhlobo lomdlavuza? Kwenzeka lapho uhlobo lweseli elimhlophe legazi elibizwa ngokuthi ama-lymphocyte emzimbeni wethu lukhula ngendlela engalawuleki futhi ngesivinini esikhulu kunalokho okufanele.

Cabanga ngala ma-lymphocyte njengamasosha amancane emizimbeni yethu. Ayingxenye yesimiso sethu sokuzivikela, esivikela kumagciwane nezifo. Kunezinhlobo ezimbili eziyinhloko zama-lymphocyte: amaseli e-B namaseli e-T . Ku-ALCL, ama-lymphocyte akho e-T, noma amaseli e-T, akhula ngendlela engavamile.

Manje ake sibone ukuthi leli gama elithi "i-anaplastic large cell lymphoma" lisho ukuthini.

• I-Anaplastic : Leli gama lisho ukuthi uma libhekwa ngesibonakhulu, lawa maseli omdlavuza abukeka engavamile futhi ehlukile uma eqhathaniswa namaseli e-T lymphocyte anempilo. Kunjengokungathi isimo sawo nokubukeka kwawo kuphambukile.
• Iseli Elikhulu : Lokhu kusho ukuthi ama-T lymphocyte aziphatha ngendlela engavamile abonakala emakhulu kunama-T cell ajwayelekile. Lo mehluko ubonakala ngokucacile ngaphansi kwe-microscope.
• I-Lymphoma : I-Lymphoma umdlavuza obangelwa ukukhula okungalawuleki kwamaseli amhlophe egazi abizwa ngokuthi ama-lymphocyte. I-ALCL ikhula ngqo kuma-T lymphocyte.

Uyaqonda? Kalula nje, i-ALCL ingumdlavuza lapho amaseli e-T eba makhulu ngendlela engavamile, aguquke, futhi akhule ngendlela engalawuleki.

Ingabe kunezinhlobo ezahlukene ze-ALCL?

Yebo, kunezinhlobo ezahlukene ze-ALCL. Lezi zihlukaniswa ngokususelwa endaweni yomdlavuza emzimbeni wakho kanye nezici zamaseli omdlavuza.

I-ALCL ehlelekile

Lolu uhlobo oluyinhloko. Igama elithi "systemic" lisho ukuthi lingathinta wonke umzimba. Lingathinta ama-lymph node akho, noma "izindlala" njengoba sizibiza kanjalo, kanye nezitho zangaphakathi njengesibindi namaphaphu akho, futhi ngezinye izikhathi isikhumba sakho.

I-ALCL yesistimu ihlukaniswe yaba izinhlobo ezimbili. Eyokuqala isekelwe ekutheni kukhona yini ukuguquka kwezakhi zofuzo okubizwa ngokuthi i- "Anaplastic Lymphoma Kinase (ALK)" gene . Izakhi zofuzo zifana nezincwadi ezincane zemiyalelo yamaseli ethu, futhi lezi zakhi zofuzo zitshela ngisho ne-T lymphocyte ukuthi isebenze kanjani.

• I-ALCL ene-ALK: Lolu uhlobo lomdlavuza olunezinguquko ku-ALK gene. Lokhu kuvame ukuba umdlavuza oyingozi. Kodwa-ke, kuvame kakhulu ezinganeni nakubantu abadala abasebasha . Ngenhlanhla, lolu hlobo lusabela kahle ku -chemotherapy .
• I-ALK-negative ALCL : Lolu hlobo alunayo i-ALK gene mutation. Lona futhi umdlavuza okhula ngokushesha (onamandla). Kodwa-ke, uthinta kakhulu abantu asebekhulile , ngokuvamile labo abaneminyaka engaphezu kwengu-60 ubudala. Lolu hlobo lungase luphumule ngemva kokwelashwa ngamakhemikhali bese luphinde lubuye. Ngakho-ke, i-ALCL-negative ALCL kunzima kakhulu ukwelapha, futhi isibikezelo sibi kakhulu kune-ALCL-negative ALCL.

Ezinye izinhlobo ze-ALCL

Kukhona nezinhlobo ze-ALCL ezikhula kancane kunezinhlobo ze-systemic.

• I-ALCL yesikhumba eyinhloko : Lolu uhlobo oluthinta kakhulu isikhumba. Izimpawu ezifana nama-blister, amaqhubu, kanye ne-eczema zingase zivele. Ezimweni eziningi (cishe ama-90%) azisabalali ngale kwesikhumba.
• I-ALCL ehlobene nokufakelwa kwebele (BIA-ALCL) : Lolu uhlobo lwe-ALCL oluvela eduze nokufakelwa kwebele. Ngokuvamile lutholakala cishe eminyakeni eyi-10 ngemva kwenqubo yokukhulisa ibele noma yokuvuselela ibele.

Ubani othola i-ALCL?

Nakuba i-ALCL ingakhula kubantu banoma yimuphi ubudala, ezinye izinhlobo zithinta kakhulu amaqembu athile.

• I-ALCL ene-ALK : Lokhu kuvame ukuthinta izingane ezisencane, intsha, kanye nabantu abadala abasebasha abaseminyakeni yabo yama-20 nama-30 . Kuvame kakhulu kwabesilisa .
• I-ALCL engena-ALK : Lokhu kuthinta kakhulu abantu abangaphezu kweminyaka engama-60. Kuvamile futhi kwabesilisa.
• I-ALCL yesikhumba eyinhloko : Lokhu kwenzeka kakhulu kubantu abadala abangaphezu kweminyaka engama-40. Kuvame kakhulu kwabesilisa nakubantu abamhlophe.
• I-BIA-ALCL : Lokhu kuvame ukutholakala kubantu abaseminyakeni yabo yo-50 . Kungavela kubantu abaye bafakelwa kokubili i-silicone kanye ne-saline breast implants. Kutholakale ukuthi kuhlotshaniswa kakhulu ne -textured breast implants .

Ivame kangakanani i-ALCL?

Empeleni i-ALCL ingumdlavuza ongavamile kakhulu . Cishe ama-2% kuphela ama-non-Hodgkin lymphomas kubantu abadala atholakala njenge-ALCL. Lelo yiphesenti elincane kakhulu.

Ziyini izimpawu ze-ALCL?

Izimpawu ze-ALCL ziyahlukahluka kuye ngohlobo.

Izimpawu ze-ALCL ehlelekile

Kuzo zombili i-ALCL ene-ALK kanye ne-ALK-negative, ama-lymph node avuvukele avamile lapho umdlavuza ukhula khona. Angase azwakale njengezigaxa ezindaweni ezifana nentamo yakho, emakhwapheni, noma embotsheni . Ezinye izimpawu zifaka:

• Umkhuhlane ovamile.
• Ukuzizwa ukhathele kakhulu (`Ukukhathala`).
• Ukujuluka ebusuku (`Ukujuluka ebusuku`).
• Ukwehlisa isisindo okungachazeki.

Uma abantu abaningi bethola ukuthi umdlavuza ususakazeke kancane, okusho ukuthi usesigabeni esithuthukile . Uma kunjalo, umdlavuza ungase ususakazeke ezithweni ezifana namaphaphu akho, isibindi, noma amathambo. Ngemuva kwalokho, izimpawu zingavela kuye ngesitho esithintekile. Isibonelo, uma uqinile esifubeni noma ukhwehlela njalo, i-ALCL kungenzeka ukuthi isithinte isifuba.

Izici ze-ALCL eyinhloko yesikhumba

Izinguquko zesikhumba zingabonakala kulolu hlobo.

• Amaqhubu angavamile abomvu noma abomvu ngokunsundu avela endaweni eyodwa noma ngaphezulu yesikhumba, okungaba makhulu ngokuhamba kwesikhathi.
• Amaqhubu aphakanyisiwe , amakhulu kunohlamvu lwemali. Lokhu kungaba buhlungu ngezinye izikhathi.
• Amaqhubu angase abe buhlungu bese ekhula abe uqweqwe .

Izici ze-BIA-ALCL (i-ALCL ehlobene nokufakelwa kwebele)

Kulokhu, izinguquko ezicacile emabeleni zingabonakala.

• Ubuhlungu bebele.
• Ukuvuvukala kwebele.
• Ukuqubuka kwesikhumba noma i-eczema.
• Ukuvuvukala noma ukunqwabelana koketshezi eduze kwesitho sakho sesifuba.
• Isisindo esiqinile eduze kwe-implant yebele.

Lezi zinguquko zivame ukubonakala ebeleni elilodwa, kodwa ngezinye izikhathi izinguquko zingabonakala kuwo womabili amabele.

Yini ebangela i-ALCL?

I-ALCL yenzeka lapho amangqamuzana e-lymphocyte esikhulume ngawo ngaphambili ephindaphindeka ngokungalawuleki futhi eqala ukukhula ngale kwezicubu ezinempilo ezizungezile. Abacwaningi abasazi kahle ukuthi kungani lawa maseli e-lymphocyte eba nomdlavuza (amangqamuzana abulalayo).

Kodwa-ke, bathole izinguquko eziningana zofuzo ezivame ukubonakala nge-ALCL. Lezi zinguquko kucatshangwa ukuthi zibangela amangqamuzana aphilile ukuba aguquke abe amangqamuzana omdlavuza.

Isibonelo, isici esiyinhloko se-ALK-positive ALCL ukuguqulwa kwezakhi zofuzo ku- ALK gene . Ngokufanayo, okunye ukuguqulwa kwezakhi zofuzo kuvamile nakwezinye izinhlobo ze-ALCL.

I-ALCL ihlolwa kanjani? (Ukuxilongwa)

Udokotela wakho uzoqala ngokukwenza ukuhlolwa ngokomzimba ukuze ahlole izimpawu ze-ALCL, njengezindlala ze-lymph ezivuvukele. Uma besola lokhu, bazokwenza izivivinyo nezinqubo eziningana ezahlukene.

• Izinqubo zokuthwebula izithombe: Lokhu kungasiza ekuboneni ukuthi umdlavuza ukuphi. Uhlobo lokuhlolwa kwezithombe oludingayo luzohluka kuye ngohlobo lwe-ALCL. Udokotela wakho angenza i -X-ray yesifuba , i-CT scan , noma i-MRI ukuze abheke izimila. I -PET/CT scan noma i-PET scan ingasiza ekuboneni ukuthi umdlavuza ususakazeke emzimbeni wonke. Uma kusolwa ukuthi i-BIA-ALCL, i-ultrasound yebele ingenziwa.
• Umsebenzi Wegazi : Udokotela uzokwenza ukuhlolwa kwegazi okuhlukahlukene, njengokuhlolwa kwegazi okuphelele (i-CBC), ukuhlola izimpawu ze-ALCL. Uma inani lamaseli egazi (amaseli abomvu egazi, amaseli amhlophe egazi, noma ama-platelet) lingajwayelekile, kungaba uphawu lwe-ALCL. Bazophinde babheke ama-enzyme athile kanye nezinye izimpawu egazini, njengoba lokhu kungaba izimpawu ze-ALCL.
• I-Biopsy : Okuwukuphela kwendlela yokuqinisekisa ukuxilongwa kwe-ALCL ukwenza i-biopsy. Ku-biopsy, udokotela uthatha isampula encane yezicubu ezisolisayo bese ezihlola ngaphansi kwe-microscope. Ngokutadisha la maseli, udokotela angakwazi ukunquma ukuthi hlobo luni lwe-ALCL onalo. Lokhu kusiza futhi ukuhlela ukwelashwa okuphumelelayo kakhulu.

Iphathwa kanjani i-ALCL?

Ukwelashwa okuvamile kwe-ALCL ehlelekile yi -chemotherapy . Ukuhlinzwa kuyindlela yokwelapha ekhethwayo ye-ALCL eyinhloko yesikhumba kanye ne-BIA-ALCL.

Ukwelashwa kwe-ALCL ehlelekile

Ama-lymphoma e-anaplastic large cell ane-ALK-positive kanye ne-ALK-negative avame ukusabela kahle ku-systemic chemotherapy. I-systemic chemotherapy ihilela ukunikeza imithi ebulala umdlavuza ngemithambo yegazi, ehamba emzimbeni wonke ngegazi ukuze ibulale amangqamuzana omdlavuza.

Ukwelashwa ngamakhemikhali kwe-ALCL ehlelekile kuvame ukusebenzisa inhlanganisela yemithi esebenza ndawonye ukubulala amangqamuzana omdlavuza. Ezinye izibonelo zifaka:

• I-BV-CHP : Lokhu kufaka phakathi imithi ethi `Brentuximab Vedotin`, `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, kanye nethi `Prednisone`.
• I-CHOP : Lokhu kuhilela ukusetshenziswa kwemithi ebizwa ngokuthi i-`Cyclophosphamide`, i-`Doxorubicin (Hydroxydaunorubicin)`, i-`Vincristine (Oncovin®)`, kanye ne-`Prednisone`.
• I-CHEOP : Lokhu kuhilela ukusetshenziswa kwemithi ebizwa ngokuthi i-'Cyclophosphamide', i-'Doxorubicin (Hydroxydaunorubicin)', i-'Etoposide', i-'Vincristine (Oncovin®)', kanye ne-'Prednisone'.

Lokhu kwelashwa ngokuvamile kungabangela ukuba zonke izimpawu zomdlavuza zinyamalale esikhathini esifushane (lokhu sikubiza ngokuthi 'ukuxolelwa').(Kubizwa ngokuthi "ukubuyela emuva") Kodwa-ke, umdlavuza ungabuya. Uma udokotela wakho ecabanga ukuthi umdlavuza ungase ubuye, angase akuncome ukufakelwa i-stem cell ngenkathi usesimweni esihle. Ukufakelwa i-stem cell kuthatha indawo yamaseli abhujiswe yi-chemotherapy ngamaseli anempilo. Lawa maseli anempilo abe esekhiqiza amaseli egazi anempilo (angenawo umdlavuza).

Uma umdlavuza ubuya, udokotela wakho angase ancome izindlela ezahlukene zokwelapha ngamakhemikhali ezenzelwe ukwelapha i-lymphoma.

Ukwelashwa kwe-ALCL eyinhloko yesikhumba

Ukwelashwa okuvame kakhulu kwalokhu ukuhlinzwa . Uma umdlavuza ususakazekele kuma-lymph node, ungase udinge nokwelashwa ngemisebe . Ukwelashwa ngemisebe kusebenza ngokuqondisa amandla kumaseli omdlavuza nokuwabhubhisa.

I-ALCL yesikhumba eyinhloko ingaphinde ivele phakathi neminyaka emihlanu yokwelashwa. Uma lokhu kwenzeka, kuzodingeka ukuhlinzwa kanye nokwelashwa ngemisebe futhi.

Uma unezimila ezisendaweni ezingenakukwazi ukususwa ngokuhlinzwa, noma uma umdlavuza uqhubeka nokubuya, ungadinga ezinye izindlela zokwelapha. Lokhu kungafaka:

• I-Methotrexate Yomlomo : Umuthi osetshenziselwa ukwelapha i-non-Hodgkin lymphoma.
• I-Bexarotene : Umuthi osetshenziselwa ukwelapha i-T-cell lymphoma ethinta isikhumba.
• I-Brentuximab Vedotin : Umuthi osetshenziselwa ukwelapha i-ALCL ehlelekile kanye nezinye izinhlobo ze-ALCL eziphindaphindayo.
• I-Interferon : Umuthi osiza amasosha omzimba wakho ukuthi aqaphele futhi alwe namaseli omdlavuza, okuhlanganisa ne-lymphoma.

Ukwelashwa kwe-BIA-ALCL

I-BIA-ALCL ivame ukwelapheka ngokuhlinzwa ukuze kususwe i-implant yebele lakho kanye nanoma yimaphi amangqamuzana omdlavuza azungezile. Uma ukuhlinzwa kungenzeki, ukwelashwa ngemisebe kunganikezwa ukuze kubulawe amangqamuzana omdlavuza.

Uma umdlavuza ubuya noma usakazekela ngale kwebele, udokotela wakho angase ancome ukwelashwa ngamakhemikhali asetshenziswa ekwelapheni i-ALCL engena-ALK. Lokhu kungafaka phakathi ukwelashwa ngamakhemikhali kwe-CHOP kanye nokwelashwa okufana ne-Brentuximab Vedotin.

I-ALCL ibaluleke kangakanani?

Kungakhathaliseki ukuthi hlobo luni, i-ALCL ingumdlavuza onzima odinga ukubhekwa ngokucophelela kanye nokwelashwa okufanele. Kodwa-ke, ukubikezela kwakho kanye nohlelo lokwelashwa kuncike ezintweni ezifana nohlobo lwe-ALCL onayo, indlela osabela ngayo ekwelashweni, kanye nesilinganiso sakho se-International Prognostic Index (IPI) .

I-IPI yasungulwa odokotela bezifo zomdlavuza ukusiza ukubikezela umphumela wama-non-Hodgkin lymphomas akhula ngokushesha, okuhlanganisa ne-ALCL ehlelekile. Amaphuzu asekelwe kulezi zici ezilandelayo:

• iminyaka yakho.
• Ikhono lakho lokwenza imisebenzi yansuku zonke.
• Ukuthi umdlavuza wakho ususabalale kangakanani (isigaba somdlavuza).
• Inani lezindlala ze-sebaceous ezithintekile.

Uma une-ALCL ehlelekile, buza udokotela wakho ukuthi amaphuzu akho e-IPI athinta kanjani amathuba akho okululama.

Lingakanani izinga lokusinda kwe-ALCL?

Amazinga okusinda kwe-ALCL ehlelekile ayahlukahluka kakhulu. I-ALCL ene-ALK ihlotshaniswa nesikhathi eside sokusinda kune-ALCL engena-ALK. Kuye ngezici ezifana nohlobo lwakho lomdlavuza kanye nesilinganiso se-IPI, izinga lokusinda leminyaka emihlanu le-ALCL ene-ALK lingasukela ku-33% kuya ku-90%. Ngokufanayo, izinga lokusinda kweminyaka emihlanu le-ALCL engena-ALK lingasukela ku-13% kuya ku-74%.

I-ALCL yesikhumba eyinhloko kanye ne-BIA-ALCL zinamazinga okusinda amahle kakhulu . Nakuba i-ALCL yesikhumba eyinhloko ivame ukuphulukiswa futhi ibuye ingakapheli iminyaka emihlanu, ukwelashwa okulandelayo kungandisa impilo yakho. Izinga lokusinda licishe libe ngu-80% eminyakeni emihlanu ngemva kokuxilongwa. Ku-BIA-ALCL, uma umdlavuza ususwe ngokuphelele ngokuhlinzwa, umdlavuza uvame ukuphela ngokuphelele.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Nazi ezinye zemibuzo ebalulekile okufanele uyibuze udokotela wakho:

• Nginaluphi uhlobo lwe-ALCL?
• Ingabe umdlavuza wami utholakala endaweni eyodwa kuphela, noma usakazeke?
• Obani ochwepheshe eqenjini lami lokunakekela?
• Yikuphi ukwelashwa ongincomayo?
• Ngingabhekana kanjani nemiphumela emibi yokwelashwa?
• Iyini i-IPI score yami?
• Angakanani amathuba okuba umdlavuza wami 'uphele'?
• Angakanani amathuba okuthi umdlavuza wami uzobuya?
• Kukangaki ngidinga ukuza ukuzolandela ukuze ngiqaphe isimo sami?

Kuvamile ukuzizwa wesaba uma uthola ukuthi unomdlavuza. Kodwa-ke, into ebaluleke kakhulu okufanele uyikhumbule ukuthi i-ALCL akuyona uhlobo olulodwa lomdlavuza olunomphumela ofanayo . Uhlobo lwe-ALCL onalo lunquma ukuthi lusakazeka ngokushesha noma kancane. Lokho kuzokwakha izinketho zakho zokwelashwa kanye nombono wakho. I-ALCL yabanye abantu isakazeka ngokushesha. Kwabanye, ikhula kancane, ikunike isikhathi esanele sokuthola ukwelashwa okungasiza ekulawuleni umdlavuza. Khuluma nodokotela wakho ngemiphumela engaba khona ngokusekelwe ekuxilongweni kwakho.

Izinto ezibaluleke kakhulu okudingeka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ake sifingqe ezinye zezinto ezibaluleke kakhulu okudingeka uzikhumbule kulokho esikhulume ngakho.

• I-ALCL uhlobo lomdlavuza olungavamile, futhi kunezinhlobo ezahlukene zalo.
• Akuzona zonke izinhlobo ze-ALCL ezifanayo. Ezinye zisakazeka ngokushesha, ezinye kancane kancane.
• Ukwelashwa kanye namathuba okululama kuyahlukahluka kuye ngohlobo lwe-ALCL onayo, iminyaka yakho, kanye nempilo yakho iyonke.
• Nakuba i-ALCL ene-ALK ivame kakhulu kubantu abasebasha, isabela kahle ekwelashweni.
• I-ALCL engena-ALK ithinta abantu abadala abaningi futhi kungaba nzima kakhulu ukwelapha.
• Isibikezelo se-ALCL yesikhumba kanye ne-ALCL ehlobene nokufakelwa kwebele (BIA-ALCL) ngokuvamile sihle.
• Ungesabi. Uma unokungabaza noma izimpawu, funa iseluleko sezokwelapha ngokushesha.
• Khuluma ngokukhululekile nodokotela wakho ngesimo sakho, ukwelashwa kwakho, nanoma yimiphi imibuzo ongase ube nayo. Yibo abangakusiza kangcono.

Khumbula, akuzona zonke izifo zomdlavuza ezingathi sína ngokulinganayo. Ngisho noma une-ALCL, ngokuxilongwa okunembile, ukwelashwa okusheshayo, kanye nokuqapha okuhle, abantu abaningi bangafinyelela imiphumela ephumelelayo.

I- Anaplastic Large Cell Lymphoma, i-ALCL, i-Lymphoma, umdlavuza, amaseli e-T, i-ALK Gene, i-Chemotherapy