ISezary Syndrome: Iimpawu kunye nokuLindelwa koBomi

Umxholo

• Zithini iimpawu?
• Umfanekiso we-erythroderma
• Ngubani osemngciphekweni?
• Ibangelwa yintoni?
• Kufunyaniswa njani?
• I-Sézary syndrome ihlelwe njani?
• Inyangwa njani?
• I-Psoralen kunye ne-UVA (PUVA)
• I-extracorporeal photochemotherapy / photopheresis (ECP)
• Unyango ngemitha
• Unyango ngamayeza
• Immunotherapy (unyango lwebhayoloji)
• Uvavanyo lwezonyango
• Imbonakalo

Yintoni iSezary syndrome?

I-Sézary syndrome luhlobo lwe-T-cell lymphoma. Iiseli zeSézary ziluhlobo oluthile lweeseli ezimhlophe zegazi. Kule meko, iiseli ezinomhlaza zifumaneka kwigazi, kulusu kunye nakwii-lymph node. Umhlaza unganwenwela nakwamanye amalungu.

I-Sézary syndrome ayiqhelekanga kakhulu, kodwa yenza iipesenti ezi-3 ukuya kwezi-5 ze-T-cell lymphomas ezinqamlekileyo. Usenokuyiva ibizwa ngokuba yiSézary erythroderma okanye iSezary's lymphoma.

Zithini iimpawu?

Uphawu oluphawulekayo lwe-Sézary syndrome yi-erythroderma, ebomvu, enqabileyo enokuthi ekugqibeleni igubungele iipesenti ezingama-80 zomzimba. Ezinye iimpawu kunye neempawu zibandakanya:

• ukudumba kolusu
• amacwecwe esikhumba kunye namathumba
• ukwandisa ii-lymph node
• ukuqina kolusu ezintendeni nasezintendeni
• ukungaqheleki kweenzipho kunye neenzwane
• iinkophe ezisezantsi ezijikela ngaphandle
• ukulahleka kweenwele
• ingxaki yokulawula ubushushu bomzimba

I-Sézary syndrome inokubangela ukuba udakada owandisiweyo okanye iingxaki ngemiphunga, isibindi kunye neendlela zesisu. Ukuba nolu hlobo lomhlaza lonyusa umngcipheko wokuphuhliswa kweminye imihlaza.

Umfanekiso we-erythroderma

Ngubani osemngciphekweni?

Nabani na onokuphuhlisa i-Sézary syndrome, kodwa kunokwenzeka ukuba ichaphazele abantu abangaphezulu kweminyaka engama-60.

Ibangelwa yintoni?

Isizathu ngqo asicacanga. Kodwa uninzi lwabantu abane-Sézary syndrome banokuchaphazeleka kwe-chromosomal kwi-DNA yeeseli ezinomhlaza, kodwa hayi kwiiseli ezisempilweni. Ezi azizizo iziphene ezizuzwe njengelifa, kodwa iinguqu ezenzeka kubomi bonke.

Ezona zinto zixhaphakileyo ziilahleko ze-DNA kwii-chromosomes 10 kunye ne-17 okanye ukongezwa kwe-DNA kwii-chromosomes 8 no-17. Okwangoku, akuqinisekanga ukuba oku kuphazamiseka kubangela umhlaza.

Kufunyaniswa njani?

Uvavanyo lomzimba lolusu lwakho lunokwazisa ugqirha malunga nesezary syndrome. Uvavanyo lokuchonga lunokubandakanya uvavanyo lwegazi lokuchonga amanqaku (iantigens) kumphezulu weeseli ezisegazini.

Njengaye neminye imihlaza, i-biopsy yeyona ndlela ifanelekileyo yokufumana isifo. Kwi-biopsy, ugqirha uya kuthatha isampulu encinci yethishu yesikhumba. Ugqirha wezifo uya kuvavanya isampulu phantsi kwemicroscope ukukhangela iiseli zomhlaza.

Iimpawu ze-lymph kunye nomongo wethambo nazo zinokuthi zenziwe i-biopsied. Ukujonga iimvavanyo, ezinje nge-CT, i-MRI, okanye i-PET scan, kunokunceda ekuboneni ukuba umhlaza usasazekile kwii-lymph node okanye kwamanye amalungu.

I-Sézary syndrome ihlelwe njani?

Isiteji sixelela ukuba umhlaza sele usasazeke kangakanani kwaye loluphi unyango olungcono.I-Sézary syndrome ihlelwe ngolu hlobo lulandelayo:

• 1A: Ngaphantsi kweepesenti ezili-10 zolusu zigqunywe ngamabala abomvu okanye ngamacwecwe.
• 1B: Ngaphezulu kwepesenti ye-10 yolusu lubomvu.
• 2A: Naliphi na inani lolusu liyabandakanyeka. Iilymph node zandisiwe, kodwa hayi ngumhlaza.
• 2B: Isilonda esinye okanye nangaphezulu esikhudlwana kunesentimitha enye senze ulusu. Iilymph node zandisiwe, kodwa hayi ngumhlaza.
• 3A: Uninzi lolusu lubomvu kwaye lunokuba namathumba, iipleyiti, okanye amabala. Ii-lymph node ziqhelekile okanye zandisiwe, kodwa hayi ngomhlaza. Igazi linakho okanye lingabinazo iiseli zeSézary ezimbalwa.
• 3B: Kukho izilonda kuninzi lolusu. Iilymph node zinokwandiswa okanye zingangandiswa. Inani leeseli zeSézary egazini liphantsi.
• 4A (1): Izilonda zolusu zigubungela nayiphi na indawo yolusu. Iilymph node zinokwandiswa okanye zingangandiswa. Inani leeseli zeSézary egazini liphezulu.
• 4A (2): Izilonda zolusu zigubungela nayiphi na indawo yolusu. Kukho ii-lymph node ezandisiweyo kwaye iiseli zijongeka zingaqhelekanga kakhulu kuvavanyo oluncinci. Iiseli zeSezari zinokuthi okanye zingabikho egazini.
• 4B: Izilonda zolusu zigubungela nayiphi na indawo yolusu. Ii-lymph node zinokuba yinto eqhelekileyo okanye engaqhelekanga. Iiseli zeSezari zinokuthi okanye zingabikho egazini. Iiseli zeLymphoma zisasazeke kwamanye amalungu okanye izicwili.

Inyangwa njani?

Zininzi izinto ezinefuthe kunyango olulungele wena. Phakathi kwazo kukho:

• isigaba sokuxilongwa
• ubudala
• ezinye iingxaki zempilo

Ezi zilandelayo zonyango lwe-Sézary syndrome.

I-Psoralen kunye ne-UVA (PUVA)

Iyeza elibizwa ngokuba yi-psoralen, elihlala liqokelela kwiiseli zomhlaza, lifakwa emthanjeni. Iyasebenza xa ifunyenwe kukukhanya kwe-ultraviolet A (UVA) ejolise kulusu lwakho. Le nkqubo itshabalalisa iiseli zomhlaza ngengozi encinci kwizicubu ezisempilweni.

I-extracorporeal photochemotherapy / photopheresis (ECP)

Emva kokufumana amayeza akhethekileyo, ezinye iiseli zegazi ziyasuswa emzimbeni wakho. Baphathwa ngokukhanya kwe-UVA ngaphambi kokuba baphinde benziwe kwakhona emzimbeni wakho.

Unyango ngemitha

Amandla eX-reyi asetyenziselwa ukutshabalalisa iiseli zomhlaza. Kwimitha yelanga engaphandle, umatshini uthumela imitha kwiindawo ekujoliswe kuzo emzimbeni wakho. Ukunyanga ngemitha kunokuthomalalisa iintlungu kunye nezinye iimpawu. Unyango olupheleleyo lwe-electron beam (TSEB) unyango lwemitha lusebenzisa umatshini wemitha yangaphandle ukujolisa ii-elektroni kulusu lomzimba wakho uphela.

Unokuba ne-UVA kunye ne-ultraviolet B (UVB) unyango lwe-radiation usebenzisa ukukhanya okhethekileyo okujolise kulusu lwakho.

Unyango ngamayeza

I-Chemotherapy yinkqubo yonyango apho kusetyenziswa iziyobisi ezinamandla ukubulala iiseli zomhlaza okanye ukuyeka ukwahlulahlula. Amanye amachiza e-chemotherapy ayafumaneka kwifom yepilisi, kwaye amanye kufuneka anikwe ngaphakathi.

Immunotherapy (unyango lwebhayoloji)

Iziyobisi ezinje ngee-interferon zisetyenziselwa ukukhuthaza amajoni akho omzimba ukuba alwe nomhlaza.

Iziyobisi ezisetyenziselwa ukunyanga i-Sézary syndrome zibandakanya:

• i-alemtuzumab (iCampath), i-anti-monoclonal antibody
• i-bexarotene (Targretin), i-retinoid
• i-brentuximab vedotin (Adcetris), isiyobisi esidibanisa neziyobisi
• I-chlorambucil (Leukeran), ichiza le-chemotherapy
• corticosteroids ukunciphisa iimpawu zolusu
• cyclophosphamide (Cytoxan), iziyobisi ngamayeza
• i-denileukin difitox (i-Ontak), isilungisi sempendulo ye-biologic
• gemcitabine (Gemzar), i-antimetabolite chemotherapy
• I-interferon alfa okanye i-interleukin-2, izikhuthazi zomzimba
• lenalidomide (Revlimid), i-angiogenesis inhibitor
• liposomal doxorubicin (Doxil), ichiza leyeza
• i-methotrexate (Trexall), i-antimetabolite chemotherapy
• ipentostatin (Nipent), ichiza ye-antimetabolite chemotherapy
• i-romidepsin (Istodax), i-histone deacetylase inhibitor
• vorinostat (Zolinza), histone deacetylase inhibitor

Ugqirha wakho unokukunika indibaniselwano yeziyobisi okanye iziyobisi kunye nezinye iindlela zonyango. Oku kuya kusekelwa kwinqanaba lomhlaza kunye nendlela ophendula ngayo kunyango oluthile.

Unyango lwenqanaba 1 kunye ne-2 kunokwenzeka ukuba lubandakanye:

• Iingqungquthela ze-corticosteroids
• I-retinoids, i-lenalidomide, i-histone deacetylase inhibitors
• IPUVA
• Imitha kunye ne-TSEB okanye i-UVB
• Unyango lwebhayoloji lulodwa okanye ngonyango lolusu
• I-chemotherapy yamacandelo
• ichemotherapy yenkqubo, inokudityaniswa nonyango lolusu

Amanqanaba 3 no-4 anokunyangwa nge:

• Iingqungquthela ze-corticosteroids
• lenalidomide, bexarotene, histone deacetylase inhibitors
• IPUVA
• I-ECP iyodwa okanye ine-TSEB
• Imitha kunye ne-TSEB okanye i-UVB kunye nemitha ye-UVA
• Unyango lwebhayoloji lulodwa okanye ngonyango lolusu
• I-chemotherapy yamacandelo
• ichemotherapy yenkqubo, inokudityaniswa nonyango lolusu

Ukuba unyango alusebenzi, ukufakelwa kweseli ye-stem kunokukhetha.

Uvavanyo lwezonyango

Uphando kunyango lomhlaza luyaqhubeka, kwaye uvavanyo lwezonyango luyinxalenye yenkqubo. Kuvavanyo lweklinikhi, unokufikelela kunyango lokuqhekeka komhlaba olungafumanekiyo kwenye indawo. Ukufumana ulwazi oluthe xaxa kwizilingo zeklinikhi, cela i-oncologist yakho okanye u tyelele iClinicalTrials.gov.

Imbonakalo

I-Sézary syndrome ngumhlaza onobukrakra. Ngonyango, unokukwazi ukucothisa ukuqhubela phambili kwesifo okanye ungene ekuxolelweni. Kodwa amajoni omzimba abuthathaka angakushiya usemngciphekweni wosulelo olungenelelayo kunye neminye imihlaza.

Umyinge wokuphila uneminyaka emi-2 ukuya kwe-4, kodwa eli nqanaba liyaphucuka ngonyango olutsha.

Yiya kugqirha uze uqalise ngonyango ngokukhawuleza kangangoko kunokwenzeka ukuze uqinisekise eyona nto uyithandayo.