Masifunde nge-Anaplastic Large Cell Lymphoma (ALCL) ngokulula.

Ngaba wakha weva nge-Anaplastic Large Cell Lymphoma (ALCL)? Mhlawumbi eli gama livakala liyinkimbinkimbi kancinci. Kodwa ungakhathazeki, olu luhlobo lomhlaza olungaqhelekanga kakhulu. Namhlanje siza kuthetha ngokuba yintoni i-ALCL, indlela ekhula ngayo, zithini iimpawu, zithini iindlela zonyango, kunye nezinye izinto ezininzi ngendlela elula kakhulu onokuyiqonda. Njengokuthetha nomhlobo.

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

Ngamafutshane, i-ALCL luhlobo lomhlaza olungaqhelekanga nolungaqhelekanga olukwiqela elikhulu lomhlaza olubizwa ngokuba yi -Non-Hodgkin Lymphoma . Ubusazi ukuba njengazo zonke ii-Non-Hodgkin Lymphoma, i-ALCL ikwaluhlobo lomhlaza? Kwenzeka xa uhlobo lweseli emhlophe yegazi ebizwa ngokuba yi-lymphocytes emzimbeni wethu lukhula ngaphandle kolawulo kwaye ngesantya esiphezulu kunokuba kufanelekile.

Cinga ngala ma-lymphocyte njengamajoni amancinci emizimbeni yethu. Ayinxalenye yenkqubo yethu yokuzikhusela, esikhusela kwiintsholongwane nezifo. Kukho iintlobo ezimbini eziphambili ze-lymphocyte: ii-B cells kunye nee-T cells . Kwi-ALCL, ii-T lymphocyte zakho, okanye ii-T cells, zikhula ngendlela engaqhelekanga.

Ngoku makhe sibone ukuba eli gama lithi "i-anaplastic large cell lymphoma" lithetha ukuthini.

• I-Anaplastic : Eli gama lithetha ukuba xa zijongwa nge-microscope, ezi seli zomhlaza zibonakala zingaqhelekanga kwaye zahlukile xa zithelekiswa neeseli ze-T lymphocyte eziphilileyo. Kungathi imo yazo kunye nenkangeleko yazo zigqwethekile.
• Iseli Enkulu : Oku kuthetha ukuba ii-T lymphocytes eziziphatha ngendlela engaqhelekanga zibonakala zinkulu kunee-T cells eziqhelekileyo. Lo mahluko ubonakala ngokucacileyo phantsi kwe-microscope.
• I-Lymphoma : I-Lymphoma ngumhlaza obangelwa kukukhula okungalawulekiyo kweeseli ezimhlophe zegazi ezibizwa ngokuba zii-lymphocytes. I-ALCL ikhula ngokukodwa kwi-T lymphocytes.

Uyaqonda? Ngamafutshane, i-ALCL ngumhlaza apho iiseli ze-T ziba nkulu ngokungaqhelekanga, zitshintshatshintsha, kwaye zikhula ngendlela engalawulekiyo.

Ngaba kukho iintlobo ezahlukeneyo ze-ALCL?

Ewe, kukho iintlobo ezahlukeneyo ze-ALCL. Ezi zahlulwe ngokwendawo yomhlaza emzimbeni wakho kunye neempawu zeeseli zomhlaza.

I-ALCL ecwangcisiweyo

Olu lolona hlobo luphambili. Igama elithi "Systemic" lithetha ukuba linokuchaphazela umzimba wonke. Linokuchaphazela ama-lymph nodes akho, okanye "ii-glands" njengoko sizibiza njalo, kunye nezitho zangaphakathi ezifana nesibindi sakho kunye nemiphunga, kwaye ngamanye amaxesha ulusu lwakho.

I-ALCL yenkqubo yahlulwe yaba ziintlobo ezimbini. Enye isekelwe ekubeni kukho utshintsho kwi-gene ebizwa ngokuba yi -``Anaplastic Lymphoma Kinase (ALK)'' gene . Ii-gene zifana neencwadi ezincinci zemiyalelo yeeseli zethu, kwaye ezi gene zixelela ne-T lymphocyte indlela yokusebenza.

• I-ALCL ene-ALK: Olu luhlobo lomhlaza olunotshintsho kwi-ALK gene. Olu luhlobo lomhlaza oludla ngokuba luhlaselo olunamandla. Nangona kunjalo, luxhaphake kakhulu ebantwaneni nakubantu abadala abancinci . Ngethamsanqa, olu hlobo lusabela kakuhle kwi -chemotherapy .
• I-ALK-negative ALCL : Olu hlobo alunazo iinguqu ze-ALK gene. Olu lukwangumhlaza okhula ngokukhawuleza (ohlaselayo). Nangona kunjalo, luchaphazela kakhulu abantu abadala , ngokuqhelekileyo abo baneminyaka engaphezu kwama-60 ubudala. Olu hlobo lunokubuyela esimeni emva kwe-chemotherapy luze lubuye kwakhona. Ke ngoko, i-ALCL-negative ALCL kunzima ukuyinyanga, kwaye i-prognosis imbi kakhulu kune-ALCL-negative ALCL.

Ezinye iintlobo ze-ALCL

Kukwakho neentlobo ze-ALCL ezikhula kancinci kuneentlobo ze-systemic.

• I-ALCL yesikhumba esiphambili : Olu luhlobo oluchaphazela kakhulu ulusu. Iimpawu ezinje ngamaqhuqhuva, amaqhuqhuva, kunye ne-eczema zinokubakho. Kwiimeko ezininzi (malunga ne-90%) ayisasazeki ngaphaya kolusu.
• I-ALCL enxulumene nokufakelwa kwamabele (BIA-ALCL) : Olu luhlobo lwe-ALCL oluvela malunga nokufakelwa kwamabele. Idla ngokuchongwa malunga neminyaka eli-10 emva kwenkqubo yokwandiswa kwamabele okanye yokwakhiwa kwakhona kwamabele.

Ngubani ofumana i-ALCL?

Nangona i-ALCL inokukhula kubantu bayo nayiphi na iminyaka, ezinye iintlobo zichaphazela amaqela athile ngakumbi.

• I-ALCL ene-ALK : Oku kudla ngokuchaphazela abantwana abakwiminyaka yokuqala ukuya esikolweni, abakwishumi elivisayo, kunye nabantu abadala abaselula abakwiminyaka yabo yama-20 nama-30 . Kuqheleke kakhulu kumadoda .
• I-ALCL engena-ALK : Oku kuchaphazela kakhulu abantu abangaphezu kweminyaka engama-60 ubudala. Ikwaxhaphake kancinci kumadoda.
• I-ALCL yesikhumba esiphambili : Oku kwenzeka rhoqo kubantu abadala abangaphezu kweminyaka engama-40 ubudala. Kuqheleke kakhulu kumadoda nakubantu abamhlophe.
• I-BIA-ALCL : Oku kudla ngokufunyaniswa kubantu abakwiminyaka yabo yama-50 . Kungavela kubantu abaye bafakelwa zombini ii-silicone kunye ne-saline breast implants. Kufunyaniswe ukuba kunxulunyaniswa ngokukodwa nee -textured breast implants .

Ixhaphake kangakanani i-ALCL?

I-ALCL ngokwenene ngumhlaza ongaqhelekanga . Kuphela yi-2% yee-non-Hodgkin lymphomas kubantu abadala ezifunyaniswa ziyi-ALCL. Lipesenti elincinci kakhulu elo.

Zithini iimpawu ze-ALCL?

Iimpawu ze-ALCL ziyahluka ngokuxhomekeke kuhlobo.

Iimpawu ze-ALCL yesiqhelo

Kwi-ALCL ene-ALK kunye ne-ALK-negative, ii-lymph nodes ezivuvukileyo ziqhelekile apho umhlaza ukhula khona. Zisenokuvakala ngathi ziqhuma kwiindawo ezifana nentamo yakho, emakhwapheni, okanye emqolo . Ezinye iimpawu ziquka:

• Umkhuhlane oqhelekileyo.
• Ukuziva udiniwe kakhulu (`Ukudinwa`).
• Ukubila ebusuku (`Ukubila ebusuku`).
• Ukunciphisa umzimba ngendlela engachazekiyo.

Xa uninzi lwabantu lufunyaniswa ukuba lunesifo, umhlaza usenokuba usasazeke kancinci, oko kuthetha ukuba 'ukwinqanaba eliphezulu' . Kwimeko enjalo, umhlaza usenokuba usasazeke kwizitho ezifana nemiphunga yakho, isibindi, okanye amathambo. Emva koko, iimpawu zinokuvela ngokuxhomekeke kwisitho esichaphazelekileyo. Umzekelo, ukuba uxinezelekile esifubeni okanye ukhwehlela rhoqo, i-ALCL isenokuba ichaphazele isifuba.

Iimpawu ze-ALCL yesikhumba esiphambili

Utshintsho lolusu lunokubonwa kolu hlobo.

• Amaqhuqhuva angaqhelekanga abomvu okanye abomvu-mdaka avela kwindawo enye okanye ezingaphezulu zesikhumba, anokukhula abe makhulu ngokuhamba kwexesha.
• Amaqhuma aphakanyisiweyo , amakhulu kunengqekembe. Ngamanye amaxesha anokurhawuzelela.
• Amaqhuma anokuba buhlungu aze emva koko abe yirhashalala .

Iimpawu zeBIA-ALCL (i-ALCL enxulumene neBreast Implant)

Kule nto, utshintsho olucacileyo emabeleni lunokubonwa.

• Intlungu yebele.
• Ukudumba kwamabele.
• Ukuqhambuka kwesikhumba okanye i-eczema.
• Ukudumba okanye ulwelo oluqokelelene kufutshane ne-implant yakho yebele.
• Ingqimba eqinileyo kufutshane ne-implant yebele.

Olu tshintsho ludla ngokubonakala kwibele elinye, kodwa ngamanye amaxesha utshintsho lunokubonwa kuzo zombini iibele.

Yintoni ebangela i-ALCL?

I-ALCL yenzeka xa iiseli ze-lymphocyte ebesithethe ngazo ngaphambili zanda ngokungalawulekiyo kwaye ziqala ukukhula ngaphaya kwezicubu eziphilileyo ezizijikelezileyo. Abaphandi abasazi ukuba kutheni ezi seli ze-lymphocyte ziba ngumhlaza (iiseli ezinobungozi).

Nangona kunjalo, bachonge utshintsho oluninzi lwezakhi zofuzo oluqhele ukubonwa kwi-ALCL. Kukholelwa ukuba olu tshintsho lwezakhi zofuzo lunokubangela ukuba iiseli eziphilileyo ziguquke zibe ziiseli zomhlaza.

Umzekelo, uphawu oluphambili lwe-ALK-positive ALCL kukuguquka kwezakhi zofuzo kwi- ALK gene . Ngokufanayo, ezinye iinguqulelo zezakhi zofuzo ziqhelekile nakwezinye iintlobo ze-ALCL.

Ifunyaniswa njani i-ALCL? (Ukuxilongwa)

Ugqirha wakho uza kuqala enze uvavanyo lomzimba ukujonga iimpawu ze-ALCL, ezifana nee-lymph nodes ezivuvukileyo. Ukuba bayakukrokrela oku, baza kwenza uvavanyo kunye neenkqubo ezahlukeneyo.

• Iinkqubo zomfanekiso: Ezi zinokunceda ekuchongeni apho umhlaza ukhoyo. Uhlobo lovavanyo lwemifanekiso olufunayo luya kwahluka ngokuxhomekeke kuhlobo lwe-ALCL. Ugqirha wakho unokwenza i -X-ray yesifuba , i-CT scan , okanye i-MRI ukujonga iithumba. I -PET/CT scan okanye i-PET scan inokunceda ekuchongeni ukuba umhlaza usasazeke emzimbeni wonke. Ukuba kurhanelwa ukuba i-BIA-ALCL iyasebenza, kunokwenziwa i-ultrasound yebele .
• Umsebenzi wegazi : Ugqirha uza kwenza uvavanyo lwegazi olwahlukeneyo, njengovavanyo lwegazi olupheleleyo (CBC), ukujonga iimpawu ze-ALCL. Ukuba inani leeseli zegazi (iiseli ezibomvu zegazi, iiseli ezimhlophe zegazi, okanye iiplatelets) aliqhelekanga, isenokuba luphawu lwe-ALCL. Baza kukhangela nee -enzymes ezithile kunye nezinye iimpawu egazini, njengoko ezi zinokuba ziimpawu ze-ALCL.
• I-Biopsy : Eyona ndlela yokuqinisekisa ukuxilongwa kwe-ALCL kukwenza i-biopsy. Kwi-biopsy, ugqirha uthatha isampuli encinci yezicubu ezikrokrelekayo aze azihlole phantsi kwe-microscope. Ngokufunda ezi seli, ugqirha unokufumanisa ukuba loluphi uhlobo lwe-ALCL onayo. Oku kukwanceda ekucwangciseni unyango olusebenzayo.

Iphathwa njani i-ALCL?

Unyango oluqhelekileyo lwe-ALCL esemgangathweni yi -chemotherapy . Utyando lolona nyango lukhethwayo kwi-ALCL yokuqala yesikhumba kunye ne-BIA-ALCL.

Unyango lwe-ALCL esisiseko

Ii-lymphomas ze-anaplastic large cell lymphomas ezine-ALK-positive kunye ne-ALK-negative zihlala zisabela kakuhle kwi-systemic chemotherapy. I-systemic chemotherapy ibandakanya ukunika amayeza okubulala umhlaza ngemithambo, ehamba emzimbeni wonke ngegazi ukuze abulale iiseli zomhlaza.

I-chemotherapy ye-ALCL esetyenziswa rhoqo isebenzisa indibaniselwano yamayeza asebenzisanayo ukubulala iiseli zomhlaza. Eminye imizekelo ibandakanya:

• I-BV-CHP : Oku kuquka amayeza athi `Brentuximab Vedotin`, `Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, kunye nethi `Prednisone`.
• I-CHOP : Oku kuquka ukusetyenziswa kwamayeza abizwa ngokuba yi-`Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, `Vincristine (Oncovin®)`, kunye ne-`Prednisone`.
• I-CHEOP : Oku kuquka ukusetyenziswa kwamayeza abizwa ngokuba yi-`Cyclophosphamide`, `Doxorubicin (Hydroxydaunorubicin)`, `Etoposide`, `Vincristine (Oncovin®)`, kunye ne-`Prednisone`.

Olu nyango lunokubangela ukuba zonke iimpawu zomhlaza ziphele ngexesha elifutshane (sikubiza oku ngokuthi 'ukuxolelwa').(Kubizwa ngokuba yi-"relapse") Nangona kunjalo, umhlaza ungabuya. Ukuba ugqirha wakho ucinga ukuba umhlaza ungabuya, banokucebisa ukuba utyandwe utyando lwe-stem cell ngelixa uphilile. Utyando lwe-stem cell luthatha indawo yeeseli ezitshatyalaliswe yi-chemotherapy ngeeseli eziphilileyo. Ezi seli ziphilileyo emva koko zivelisa iiseli zegazi eziphilileyo (ezingengomhlaza).

Ukuba umhlaza ubuya, ugqirha wakho unokucebisa ezinye iindlela zonyango ze-chemotherapy ezenzelwe ukunyanga i-lymphoma.

Unyango lwe-ALCL yesikhumba esiphambili

Unyango oluqhelekileyo loku lutyando . Ukuba umhlaza usasazeke kwiindawo ze-lymph nodes, ungadinga nonyango lwe-radiation . Unyango lwe-radiation lusebenza ngokuyalela amandla kwiiseli zomhlaza nokuzitshabalalisa.

I-ALCL yesikhumba esiphambili inokuphinda ivele emva kweminyaka emihlanu yonyango. Ukuba oku kuyenzeka, kuya kufuneka utyando kunye nonyango lwemitha kwakhona.

Ukuba unezimila ezikwindawo ethile ezingenakususwa ngotyando, okanye ukuba umhlaza uyaqhubeka nokubuya, ungadinga olunye unyango. Ezi zingabandakanya:

• I-Methotrexate yomlomo : Iyeza elisetyenziselwa ukunyanga i-non-Hodgkin lymphoma.
• I-Bexarotene : Liyeza elisetyenziselwa ukunyanga i-T-cell lymphoma echaphazela ulusu.
• I-Brentuximab Vedotin : Iyeza elisetyenziselwa ukunyanga i-ALCL ephilayo kunye nezinye iintlobo ze-ALCL eziphindaphindayo.
• I-Interferon : Iyeza elinceda inkqubo yakho yomzimba ukuba iqaphele kwaye ilwe neeseli zomhlaza, kuquka ne-lymphoma.

Unyango lwe-BIA-ALCL

I-BIA-ALCL idla ngokunyangwa ngotyando lokususa i-implant yebele lakho kunye nazo naziphi na iiseli zomhlaza ezijikeleze lona. Ukuba utyando alunakwenzeka, unyango lwemitha lunokunikezelwa ukubulala iiseli zomhlaza.

Ukuba umhlaza ubuya okanye usasazeka ngaphaya kwebele, ugqirha wakho unokucebisa unyango lwe-chemotherapy olusetyenziselwa ukunyanga i-ALK-negative ALCL. Oku kungabandakanya i-CHOP chemotherapy kunye nonyango olufana neBrentuximab Vedotin.

Ibaluleke kangakanani i-ALCL?

Nokuba loluphi na uhlobo, i-ALCL ngumhlaza onzima ofuna ukujongwa ngononophelo kunye nonyango olufanelekileyo. Nangona kunjalo, ukuqikelela kwakho isifo kunye nesicwangciso sonyango kuxhomekeke kwizinto ezifana nohlobo lwe-ALCL onayo, indlela osabela ngayo kunyango, kunye namanqaku akho e-International Prognostic Index (IPI) .

I-IPI yaphuhliswa ngoogqirha be-oncologists ukunceda ekuqikeleleni iziphumo ze-non-Hodgkin lymphomas ezikhula ngokukhawuleza, kuquka ne-ALCL yenkqubo. Amanqaku asekelwe kwezi zinto zilandelayo:

• iminyaka yakho.
• Ukukwazi kwakho ukwenza imisebenzi yemihla ngemihla.
• Ukuba umhlaza wakho usasazeke kangakanani (inqanaba lomhlaza).
• Inani lee-sebaceous glands ezichaphazelekileyo.

Ukuba une-ALCL esempilweni, buza ugqirha wakho ukuba amanqaku akho e-IPI achaphazela njani amathuba akho okuchacha.

Ingakanani izinga lokusinda kwi-ALCL?

Amanqanaba okusinda kwi-ALCL eqhelekileyo ayahluka kakhulu. I-ALCL ene-ALK inxulunyaniswa nexesha elide lokusinda kune-ALK-negative ALCL. Ngokuxhomekeke kwizinto ezifana nohlobo lwakho lomhlaza kunye namanqaku e-IPI, izinga lokusinda leminyaka emihlanu kwi-ALK-positive ALCL linokuqala ukusuka kwi-33% ukuya kwi-90%. Ngokufanayo, izinga lokusinda kweminyaka emihlanu kwi-ALK-negative ALCL linokuqala ukusuka kwi-13% ukuya kwi-74%.

I-ALCL yesikhumba esisiseko kunye ne-BIA-ALCL zinezinga elihle kakhulu lokusinda. Nangona i-ALCL yesikhumba esisiseko idla ngokubuyela esimeni sayo kwaye ibuye emva kweminyaka emihlanu, unyango olulandelayo lunokwandisa ubomi bakho. Izinga lokusinda limalunga ne-80% kwiminyaka emihlanu emva kokufunyaniswa. Kwi-BIA-ALCL, ukuba umhlaza ususwe ngokupheleleyo ngotyando, umhlaza uhlala uphelile ngokupheleleyo.

Ndingayibuza yiphi imibuzo ugqirha wam?

Nazi ezinye zemibuzo ebalulekileyo ekufuneka uyibuze ugqirha wakho:

• Luhlobo luni lwe-ALCL endinalo?
• Ingaba umhlaza wam uphelela kwindawo enye, okanye usasazeke?
• Ngoobani iingcali kwiqela lam lokhathalelo?
• Loluphi unyango olundicebisayo?
• Ndingazilawula njani iziphumo ebezingalindelekanga zonyango?
• Ithini inqaku lam le-IPI?
• Zithini amathuba okuba umhlaza wam 'uphinde uphile'?
• Zithini amathuba okuba umhlaza wam ubuye?
• Kufuneka ndize kangaphi ukuze ndilandelele unyango lwam ukuze ndijonge imeko yam?

Kuqhelekile ukuziva usoyika xa ufumanisa ukuba unomhlaza. Nangona kunjalo, eyona nto ibalulekileyo ekufuneka uyikhumbule kukuba i-ALCL ayilohlobo olunye lomhlaza olunesiphumo esifanayo . Uhlobo lwe-ALCL onalo luchaza ukuba isasazeka ngokukhawuleza okanye kancinci. Oko kuya kuyila iindlela zakho zonyango kunye nendlela ocinga ngayo. I-ALCL yabanye abantu isasazeka ngokukhawuleza. Kwabanye, ikhula kancinci, ikunika ixesha elaneleyo lokufumana unyango olunokunceda ukulawula umhlaza. Thetha nogqirha wakho malunga neziphumo ezinokubakho ngokusekelwe kwisifo sakho.

Izinto ezibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, masishwankathele ezinye zezona zinto zibalulekileyo ekufuneka uzikhumbule koko sithethe ngako.

• I-ALCL luhlobo olungaqhelekanga lomhlaza, kwaye kukho iintlobo ezahlukeneyo zalo.
• Ayizizo zonke iintlobo ze-ALCL ezifanayo. Ezinye zisasazeka ngokukhawuleza, ezinye zisasazeka kancinci.
• Unyango kunye namathuba okuchacha ayahluka ngokuxhomekeke kuhlobo lwe-ALCL onayo, ubudala bakho, kunye nempilo yakho iyonke.
• Nangona i-ALCL ene-ALK ixhaphake kakhulu kubantu abancinci, isabela kakuhle kunyango.
• I-ALCL engena-ALK ichaphazela abantu abadala abaninzi kwaye kunokuba nzima ukunyanga.
• Ingqikelelo ye-ALCL yesikhumba kunye ne-ALCL enxulumene nokufakelwa kwebele (BIA-ALCL) ilungile ngokubanzi.
• Musa ukoyika. Ukuba unamathandabuzo okanye iimpawu, funa ingcebiso kagqirha ngokukhawuleza.
• Thetha ngokukhululekileyo nogqirha wakho malunga nemeko yakho, unyango lwakho, kunye nayo nayiphi na imibuzo onokuba nayo. Ngabo abanokukunceda ngcono.

Khumbula, ayizizo zonke izifo ezifunyaniswe ngumhlaza ezinzulu ngokulinganayo. Nokuba kukho i-ALCL, xa kukho ukuxilongwa ngokuchanekileyo, unyango olukhawulezileyo, kunye nokubekwa esweni kakuhle, abantu abaninzi banokufikelela kwiziphumo eziphumelelayo.

I- Anaplastic Large Cell Lymphoma, i-ALCL, i-Lymphoma, umhlaza, ii-T Cells, i-ALK Gene, i-Chemotherapy