ISézary Syndrome: Izimpawu Nokulindelwa Kwempilo

-Delile

• Ziyini izimpawu nezimpawu?
• Isithombe se-erythroderma
• Ubani osengozini?
• Kubangelwa yini?
• Kutholakala kanjani?
• Ihlelwe kanjani iSézary syndrome?
• Ilashwa kanjani?
• I-Psoralen ne-UVA (PUVA)
• I-Extracorporeal photochemotherapy / photopheresis (ECP)
• Ukwelashwa ngemisebe
• Ukwelashwa ngamakhemikhali
• I-Immunotherapy (ukwelashwa kwe-biologic)
• Ukuhlolwa kwemitholampilo
• Umbono

Iyini i-Sézary syndrome?

I-Sézary syndrome iyindlela ye-T-cell lymphoma ehlukanisiwe. Amaseli weSézary awuhlobo oluthile lwamaseli amhlophe egazi. Kulesi simo, amangqamuzana anomdlavuza angatholakala egazini, esikhunjeni nakuma-lymph node. Umdlavuza ungadlulela nakwezinye izitho.

I-Sézary syndrome ayijwayelekile kakhulu, kodwa yakha amaphesenti ama-3 kuya kwangu-5 ama-T-cell lymphomas anciphisiwe. Ungase uzwe futhi kuthiwa yi-Sézary erythroderma noma i-Sézary's lymphoma.

Ziyini izimpawu nezimpawu?

Uphawu oluphawulekayo lwe-Sézary syndrome yi-erythroderma, ukuqubuka okubomvu, okunukayo okungagcina kumboze amaphesenti angama-80 omzimba. Ezinye izimpawu nezimpawu zifaka:

• ukuvuvukala kwesikhumba
• izingcwecwe zesikhumba nezicubu
• ama-lymph node akhulisiwe
• ukuqina kwesikhumba ezintendeni zezandla nasematheni
• ukungajwayelekile kwezinzipho nezinzwane
• izinkophe eziphansi eziphendukela ngaphandle
• ukulahleka kwezinwele
• Inkinga yokulawula ukushisa komzimba

I-Sézary syndrome nayo ingadala ubende obukhulisiwe noma izinkinga ngamaphaphu, isibindi kanye nomgudu wamathumbu. Ukuba nale fomu yomdlavuza enobudlova kwandisa ingozi yokuhlaselwa eminye imidlavuza.

Isithombe se-erythroderma

Ubani osengozini?

Noma ngubani angaba ne-Sézary syndrome, kodwa kungenzeka ukuthi ithinte abantu abaneminyaka engaphezu kwengu-60.

Kubangelwa yini?

Imbangela ngqo ayicaci. Kepha abantu abaningi abane-Sézary syndrome banokungajwayelekile kwe-chromosomal ku-DNA yamaseli anomdlavuza, kepha hhayi kumaseli anempilo. Lokhu akukhona ukukhubazeka okuzuzwe njengefa, kodwa izinguquko ezenzeka esikhathini sokuphila konke.

Okungajwayelekile okujwayelekile ukulahleka kwe-DNA kusuka kuma-chromosomes 10 no-17 noma izengezo ze-DNA kuma-chromosomes 8 no-17.

Kutholakala kanjani?

Ukuhlolwa kwesikhumba sakho ngokomzimba kungaxwayisa udokotela ukuthi kungenzeka yini ukuthi kune-Sézary syndrome. Ukuhlola okuxilongayo kungafaka ukuhlolwa kwegazi ukukhomba izimpawu (ama-antigen) ebusweni bamaseli egazini.

Njengakwezinye izinhlobo zomdlavuza, i-biopsy iyindlela engcono kakhulu yokuthola ukuxilongwa. Nge-biopsy, udokotela uzothatha isampula elincane lezicubu zesikhumba. Isazi sezifo sizohlola isampula ngaphansi kwesibonakhulu ukubheka amangqamuzana omdlavuza.

Ama-lymph node nomnkantsha wamathambo nawo angaphanjaniswa. Ukuhlola ukuhlolwa, okufana nokuhlolwa kwe-CT, i-MRI, noma i-PET, kungasiza ekunqumeni ukuthi umdlavuza ususabalele kuma-lymph node noma ezinye izitho.

Ihlelwe kanjani iSézary syndrome?

Isiteji sitshela ukuthi umdlavuza ususabalale kangakanani nokuthi yiziphi izindlela zokwelashwa ezingcono kakhulu. I-Sézary syndrome ihlelwe kanjena:

• 1A: Amaphesenti angaphansi kweshumi esikhumba ambozwe ngamachashazi abomvu noma amapuleti.
• 1B: Amaphesenti angaphezu kweshumi esikhumba abomvu.
• 2A: Noma yiliphi inani lesikhumba liyabandakanyeka. Ama-lymph node akhulisiwe, kepha hhayi umdlavuza.
• 2B: Kwakheke isikhumba esisodwa noma ngaphezulu esikhumbeni esisodwa. Ama-lymph node akhulisiwe, kepha hhayi umdlavuza.
• 3A: Iningi lesikhumba libomvu futhi lingaba nezicubu, izingcwecwe noma amabala. Ama-lymph node ajwayelekile noma akhulisiwe, kepha hhayi umdlavuza. Igazi kungenzeka noma liqukethe amaseli ambalwa eSézary.
• 3B: Kukhona izilonda ngaphezu kwesikhumba esiningi. Ama-lymph node angakhuliswa noma angakhuliswa. Inani lamaseli we-Sézary egazini liphansi.
• 4A (1): Izilonda zesikhumba zimboza noma iyiphi ingxenye yesikhumba. Ama-lymph node angakhuliswa noma angakhuliswa. Isibalo samaseli we-Sézary egazini siphezulu.
• 4A (2): Izilonda zesikhumba zimboza noma iyiphi ingxenye yesikhumba. Kunama-lymph node akhulisiwe futhi amaseli abukeka angajwayelekile kakhulu ngaphansi kokuhlolwa okuncane. Amaseli weSézary angahle noma angabi segazini.
• 4B: Izilonda zesikhumba zimboza noma iyiphi ingxenye yesikhumba. Ama-lymph node angajwayelekile noma angajwayelekile. Amaseli weSézary angahle noma angabi segazini. Amaseli e-lymphoma asabalalele kwezinye izitho noma izicubu.

Ilashwa kanjani?

Kunezici eziningi ezinomthelela wokuthi yikuphi ukwelashwa okungaba ngcono kuwe. Phakathi kwazo kukhona:

• isigaba sokuxilongwa
• ubudala
• ezinye izinkinga zempilo

Okulandelayo ngezinye izindlela zokwelapha i-Sézary syndrome.

I-Psoralen ne-UVA (PUVA)

Umuthi obizwa nge- psoralen, ovame ukuqoqeka kumaseli womdlavuza, ufakwa emthanjeni. Iyasebenza uma ivezwa ekukhanyeni kwe-ultraviolet A (UVA) ebhekiswe esikhumbeni sakho. Le nqubo ibhubhisa amangqamuzana omdlavuza ngokulimaza okuncane kuphela kwizicubu ezinempilo.

I-Extracorporeal photochemotherapy / photopheresis (ECP)

Ngemuva kokuthola izidakamizwa ezikhethekile, amanye amangqamuzana egazi ayasuswa emzimbeni wakho. Ziphathwa ngokukhanya kwe-UVA ngaphambi kokuba ziphinde zifakwe emzimbeni wakho.

Ukwelashwa ngemisebe

Kusetshenziswa i-X-ray enamandla amakhulu ukubhubhisa amangqamuzana omdlavuza. Kumisebe yangaphandle yesigxobo, umshini uthumela imisebe ezindaweni ezihlosiwe zomzimba wakho. Ukwelashwa ngemisebe nakho kungabudambisa ubuhlungu nezinye izimpawu. Ukwelashwa kwemisebe ye-total electron beam (TSEB) isebenzisa umshini wemisebe wangaphandle ukukhomba ama-electron esikhunjeni somzimba wakho wonke.

Ungaba futhi ne-UVA ne-ultraviolet B (UVB) therapy radiation usebenzisa isibani esikhethekile esibhekiswe esikhunjeni sakho.

Ukwelashwa ngamakhemikhali

I-Chemotherapy yindlela yokwelashwa okuhlelekile lapho kusetshenziswa khona izidakamizwa ezinamandla ukubulala amangqamuzana omdlavuza noma ukumisa ukwahlukana kwawo. Eminye imishanguzo yokwelashwa ngamakhemikhali iyatholakala ngefomu lamaphilisi, kanti eminye kufanele inikezwe ngemithambo yegazi.

I-Immunotherapy (ukwelashwa kwe-biologic)

Izidakamizwa ezinjenge-interferon zisetshenziselwa ukukhuthaza amasosha akho omzimba ukuba alwe nomdlavuza.

Izidakamizwa ezisetshenziselwa ukwelapha iSézary syndrome zifaka:

• i-alemtuzumab (iCampath), i-antibody eyodwa
• i-bexarotene (iTargretin), i-retinoid
• i-brentuximab vedotin (i-Adcetris), i-anti-drug conjugate
• I-chlorambucil (Leukeran), umuthi we-chemotherapy
• corticosteroids ukuqeda izimpawu zesikhumba
• i-cyclophosphamide (i-Cytoxan), umuthi we-chemotherapy
• denileukin difitox (Ontak), isilungisi sempendulo se-biologic
• gemcitabine (Gemzar), i-antimetabolite chemotherapy
• i-interferon alfa noma i-interleukin-2, izivikeli mzimba
• lenalidomide (Revlimid), i-angiogenesis inhibitor
• liposomal doxorubicin (Doxil), umuthi wokwelapha ngamakhemikhali
• i-methotrexate (Trexall), i-antimetabolite chemotherapy
• i-pentostatin (Nipent), i-antimetabolite chemotherapy
• i-romidepsin (Istodax), i-histone deacetylase inhibitor
• i-vorinostat (i-Zolinza), i-histone deacetylase inhibitor

Udokotela wakho angakunika inhlanganisela yezidakamizwa noma izidakamizwa kanye nezinye izindlela zokwelapha.Lokhu kuzosuselwa esigabeni somdlavuza nokuthi uphendula kahle kangakanani ekwelashweni okuthile.

Ukwelashwa kwesigaba 1 no-2 kungenzeka kufake phakathi:

• ama-corticosteroids aphezulu
• ama-retinoids, i-lenalidomide, i-histone deacetylase inhibitors
• PUVA
• imisebe ene-TSEB noma i-UVB
• ukwelashwa kwe-biologic kukodwa noma ngokwelashwa kwesikhumba
• i-topical chemotherapy
• i-systemic chemotherapy, mhlawumbe ihlanganiswe nokwelashwa kwesikhumba

Izigaba 3 no-4 zingelashwa nge:

• ama-corticosteroids aphezulu
• lenalidomide, bexarotene, histone deacetylase inhibitors
• PUVA
• I-ECP iyodwa noma ne-TSEB
• imisebe ene-TSEB noma i-UVB nemisebe ye-UVA
• ukwelashwa kwe-biologic kukodwa noma ngokwelashwa kwesikhumba
• i-topical chemotherapy
• i-chemic systemic, mhlawumbe ihlanganiswe nokwelashwa kwesikhumba

Uma ukwelashwa kungasasebenzi, ukufakelwa kwe-stem cell kungaba yindlela.

Ukuhlolwa kwemitholampilo

Ucwaningo ngokwelashwa komdlavuza luyaqhubeka, futhi izivivinyo zokwelashwa ziyingxenye yaleyo nqubo. Esivivinyweni somtholampilo, ungahle ukwazi ukufinyelela ekwelashweni okuqhekezayo okutholakala ngaphandle kwenye indawo. Ukuthola eminye imininingwane ngokuhlolwa komtholampilo, cela i-oncologist yakho noma uvakashele iClinicalTrials.gov.

Umbono

ISézary syndrome iwumdlavuza onolaka ikakhulukazi. Ngokwelashwa, ungakwazi ukunciphisa ukukhula kwesifo noma ungene ekuxolelweni. Kepha amasosha omzimba abuthakathaka angakushiya usengozini yokutheleleka kosomathuba neminye imidlavuza.

Isilinganiso sokusinda besiyiminyaka emi-2 kuye kwengu-4, kepha leli zinga liyathuthuka ngezindlela zokwelashwa ezintsha.

Bona udokotela wakho bese uqala ukwelashwa ngokushesha okukhulu ukuze uqiniseke ngombono omuhle kakhulu.