Iyini i-Sézary Syndrome? Iyithinta kanjani isikhumba sakho?

Wake waphawula ukuthi isikhumba sakho siphenduka sibe bomvu ngokuzumayo, silume kakhulu, futhi sibe mnyama kwezinye izindawo? Ungase ucabange ukuthi kuyisimo sesikhumba esivamile nje, kodwa ezimweni ezingavamile, kungaba yinto engathi sína kakhulu. Yilokho esikhuluma ngakho namuhla, i-Sézary Syndrome, umdlavuza ongavamile othinta isikhumba, igazi, kanye nama-lymph node. Ungase udideke kancane ngegama, kodwa kubalulekile ukuqaphela.

Iyini i-Sézary Syndrome?

Kalula nje, i-Sézary Syndrome uhlobo lomdlavuza wesikhumba olungavamile kakhulu futhi olusakazeka ngokushesha. Ngokwezokwelapha, lungolweqembu lomdlavuza olubizwa ngokuthi i-cutaneous T-cell lymphoma. Ama-T-lymphocyte, noma ama-T-cell ngamafuphi, aluhlobo olubalulekile lweseli ohlelweni lokuzivikela lomzimba wethu. Kulesi sifo, uhlobo lweseli olubizwa ngokuthi ama-T-cell luba umdlavuza futhi luqongelela esikhumbeni sakho, egazini, kanye nakuma-lymph node.

Cabanga ngakho njengamasosha avikela imizimba yethu, lawa `(amaseli e-T)`. Kodwa uma la masosha ephambuka futhi eqala ukusebenza ngokumelene nathi, yilokhu okwenzekayo. Yingakho isikhumba sakho sishintsha umbala futhi sibukeke sibomvu. Ngezinye izikhathi isikhumba singaba mnyama futhi. Kukhona futhi ithuba lokulunywa okukhulu kanye nezilonda ezibuhlungu kanye nokuqubuka. Into eyingozi kakhulu ukuthi la mangqamuzana omdlavuza egazini angasakazeka kwezinye izingxenye zomzimba, okungukuthi, ezithweni zangaphakathi. Nakuba okwamanje kungekho ukwelashwa okuphelele kwalokhu, ungakhathazeki , kunezindlela eziningi zokwelapha zokulawula izimpawu nokukunikeza impumuzo.

Sivame kangakanani lesi simo?

Empeleni lokhu akuvamile kakhulu. Okusho ukuthi, yisifo esingajwayelekile ukubonwa. Cabanga nje, ngisho nasezweni elifana neMelika, kubikwa ukuthi umuntu oyedwa kwabayisigidi uthola lesi sifo minyaka yonke. Ngakho-ke lokhu akuyona into evame ukubonwa eSri Lanka. Yingakho kubalulekile ukuqaphela lokhu, ngoba uma izimpawu zivela, zingabonakala ngokushesha.

Ziyini izimpawu ze-Sézary Syndrome?

Uphawu oluyinhloko lwalesi sifo ukuqubuka okubomvu okuhlukile (i-erythroderma) . Lokhu akuyona nje ukulunywa okulula, kuyaluma kakhulu, kuyabuhlungu, ngezinye izikhathi isikhumba siyaphuma futhi sisakazeke ngokushesha okukhulu. Kwabanye abantu, ikakhulukazi labo abanesikhumba esimnyama, kungabonakala futhi njengamabala amnyama (angaba mpunga, onsomi, noma onsundu). Ezigabeni zokuqala, lezi zimpawu kungaba nzima ukuzibona ngoba zifana nezimo zesikhumba ezivamile njenge-eczema noma i-psoriasis. Abantu abaningi bacabanga ukuthi lokhu kuyisifo esilula sokungezwani komzimba.

Njengoba i-Sézary Syndrome iqhubeka, isikhumba sakho sizoqala ukuba namaqhubu amancane aphakanyisiwe (ama-papules). Ezinye izindawo zingase zizwe zijiyile futhi ziqinile kunezinye (i-plaque). Ekugcineni, umdlavuza wesikhumba (amaqhubu) ungavela. Kukhona nezinye izimpawu ezimbalwa, esizozibheka:

• Ama-lymph node avuvukele - ezindaweni ezifana nentamo, emakhwapheni, kanye nasesinqeni.
• Umkhuhlane - Umkhuhlane oqala ngaphandle kwesizathu.
• Ukukhathala okuqhubekayo - Umuzwa wokukhathala kungakhathaliseki ukuthi ulala kangakanani.
• Ukwehlisa isisindo okungachazeki.
• Ukulahlekelwa izinwele - Ngezinye izikhathi amashiya ngisho nezinwele zomzimba zingalahleka.
• I-Ectropion (ijwabu leso eliphendukezelwe).
• Ukwanda kwesibindi (i-hepatomegaly).
• Ukuvuvukala (i-edema), ikakhulukazi emilenzeni engezansi.
• Ukungabekezelelani nokubanda - Lokhu kusho ukuthi uzizwa ubanda kakhulu kunabanye, ngisho noma izinga lokushisa elijwayelekile legumbi.

Yini ebangela i-Sézary Syndrome?

Lokhu kwenzeka ngoba amangqamuzana anempilo abizwa ngokuthi ama-T-cell emzimbeni wethu adlula ezinguqukweni zofuzo, awenze abe amangqamuzana omdlavuza angajwayelekile. Lawa maseli angajwayelekile ahlukana ngokushesha futhi ande ngokungalawuleki, ekugcineni asakazekela esikhumbeni, kuma-lymph node, nasegazini. Kufana nokhula, olusakazeka yonke indawo ngaphandle kokulawulwa. Kodwa-ke, ochwepheshe abasazi kahle ukuthi yini ebangela lezi zinguquko zofuzo. Lokho kusho ukuthi kunzima ukusho ukuthi kungani.

Yiziphi izinto ezibangela lokhu?

Nakuba noma ubani engaba nalesi sifo, ezinye izinto zingandisa ingozi yakho. Isibonelo, uma uke watheleleka ngegciwane le-T-cell leukemia (HTLV) lomuntu, kucatshangwa ukuthi usengozini encane yokuba ne-Sézary syndrome. Kodwa-ke, kubalulekile ukukhumbula ukuthi akuwona wonke umuntu othelelekile ngaleli gciwane ozohlakulela lesi sifo.

Yiziphi izinkinga ezingaba khona ngenxa yalesi sifo?

I-Sézary Syndrome yisifo esingathinta kakhulu izinga lempilo. Cabanga nje, ungazizwa kanjani uphilile uma uhlala uklwebha isikhumba sakho futhi uzwa ubuhlungu? Kungabangela izinto ezifana nokungalali, ukukhathazeka okuqhubekayo, nokucindezeleka. Kukhona nezinye izinkinga eziningana:

• Amaseli e-T anomdlavuza asakazekela emaphashini akho, esibindini, ebhentshini kanye nasemongweni wamathambo.
• Ingozi eyengeziwe yokuthola olunye uhlobo lwe-lymphoma (umdlavuza wesistimu ye-lymphatic).
• Ingozi eyengeziwe yokutheleleka njalo ngoba ungqimba oluvikelayo lwesikhumba lonakele, okuvumela amagciwane ukuthi angene kalula.

Odokotela basithola kanjani lesi sifo?

Uma uya kudokotela, uzoqala ngokukuhlola umzimba bese ekubuza ngezimpawu zakho. Uzobuza nangomlando wakho wezokwelapha odlule. Udokotela uzokwenzaUma kusolwa ukuthi uSézary Syndrome unesifo, angenza izivivinyo eziningana, njengalezi:

• Ukuhlolwa kwegazi: Lokhu kubaluleke kakhulu. Lokhu kungafaka phakathi "Inani Eliphelele Legazi (i-CBC)" (lokhu kuhlola iphesenti lezinhlobo ezahlukene zamaseli egazini, ikakhulukazi amaseli amhlophe egazi), "inani legazi le-Sézary" (elihlola inani lamaseli e-Sézary" (amaseli omdlavuza ajwayelekile kulesi sifo) egazini), kanye "ne-peripheral blood smear" (ukuhlolwa kwesampula yegazi nge-microscope).
• Ukuhlolwa kwesikhumba, i-lymph node, noma i-bone marrow biopsies: Kulokhu, kuthathwa ingxenye encane yezicubu endaweni ethintekile yesikhumba noma iqhubu bese ithunyelwa elabhorethri. Lapho, udokotela wezifo zezokwelapha uhlola isampula yezicubu ngaphansi kwe-microscope. Lokhu kungabheka izinto ezifana nezimpawu zesimila kanye nezinguquko emaseli.
• Ukuhlolwa kokubona ukuthi umdlavuza ususakazekile yini: Uma ukuhlolwa kwegazi nezicubu kuqinisekisa ukuthi unesifo i-Sézary syndrome, kuzokwenziwa ukuhlolwa kokubona ukuthi umdlavuza ususakazekele kwezinye izingxenye zomzimba. Lokhu kungafaka phakathi i-X-ray yesifuba, i-CT scans (computerized tomography), kanye ne-Positron Emission Tomography (PET) scan (ebheka umsebenzi wamaseli emzimbeni).

Yiziphi izindlela zokwelapha i-Sézary Syndrome?

Udokotela wakho uzokunikeza ukwelashwa ngokusekelwe ezimpawini zakho kanye nesigaba somdlavuza wakho (ukuthi ususakazeke kangakanani). Kunezindlela ezahlukene zokwelapha lokhu. Akuwona wonke umuntu othola ukwelashwa okufanayo.

• Ukwelashwa nge-Phototherapy: Lokhu kusebenzisa izinhlobo ezikhethekile zokukhanya ukubhubhisa amangqamuzana omdlavuza esikhumbeni. Enye indlela ibizwa ngokuthi i-extracorporeal photopheresis. Kulokhu, amangqamuzana amhlophe egazi ayasuswa egazini lakho (i-leukapheresis), aphathwe ngomuthi bese evezwa ekukhanyeni kwe-ultraviolet (ukukhanya kwe-UV), bese ebuyiselwa emzimbeni wakho. Ngaphandle kwalokho, ukwelashwa nge-UV phototherapy kunganikezwa ngqo esikhumbeni.
• Ukwelashwa ngemisebe: Lokhu kuhilela ukusebenzisa imisebe enamandla aphezulu ukubhubhisa amangqamuzana omdlavuza. Lokhu kungenziwa ngokusebenzisa ukwelashwa ngemisebe yangaphandle (ukusebenzisa umshini ukuletha imisebe) noma ukwelashwa ngemisebe yesikhumba iyonke (ukwelashwa ngemisebe yesikhumba iyonke).
• Ukwelashwa ngamakhemikhali: Lokhu kuhilela ukunikeza imithi ebulala amangqamuzana omdlavuza. Kunezinhlobo ezisetshenziswa esikhumbeni (i-chemotherapy yangaphandle) kanye nezinhlobo ezithinta umzimba wonke (i-chemotherapy yesistimu) (kungaba ngamaphilisi noma njengomjovo). I-Vorinostat (i-Zolinza®) iyisibonelo somuthi onikezwa umzimba wonke.
• Ukwelashwa okuqondiwe: Lokhu kwelashwa kuhlasela izinhloso ezithile ezitholakala kuphela kumaseli omdlavuza. Ukwelashwa ngama-antibody e-monoclonal kungenye yezindlela zokwelapha eziqondiwe ezisetshenziswa kwi-Sézary syndrome.
• Ukwelashwa nge-immunotherapy: Lokhu kuhilela ukuvuselela amasosha omzimba wakho ukuze alwe namaseli omdlavuza. Lokhu kungenziwa kusetshenziswa imithi efana ne-Interferon (Intron A®, Intron A Multidose Pen.®)).
• Ezinye izindlela zokwelapha ngemithi: Ama-Corticosteroids (imithi yohlobo lwe-steroid) kanye nama-creams noma ama-gel ohlobo lwe-retinoid (isb. i-bexarotene (i-Targretin®)) nawo asetshenziswa ukulawula izimpawu zesikhumba.

"Kodwa khumbula, akuzona zonke lezi zindlela zokwelapha ezifanayo kuwo wonke umuntu. Udokotela wakho uzonquma ukwelashwa okungcono kakhulu kwesimo sakho. Uzokuchazela konke."

Iyini imiphumela emibi yokwelashwa?

Iningi lemithi yokwelapha umdlavuza inemiphumela emibi ethile. Lokhu kuvamile. Kubalulekile ukuzazi kusenesikhathi. Ngokuvamile, `(chemotherapy)` kanye `(radiation therapy)` ungalindela izinto ezifana nokukhathala, isicanucanu, ukuhlanza, ukulahlekelwa yisifiso sokudla, kanye nokulahlekelwa izinwele. `(Phototherapy)` ingathinta isikhumba - ngezinye izikhathi isikhumba esomile, ukubomvu. `(Targeted therapy)`, `(immunomodulators)` (imithi eshintsha isimiso somzimba sokuzivikela) kanye `(immunotherapy)` ngezinye izikhathi kungabangela ukuqunjelwa, uhudo, ukukhathala, kanye nezinguquko ezingxenyeni zegazi `(ukungalingani kwenani legazi)`. Kungumqondo omuhle ukukhuluma nodokotela wakho ngalokhu futhi uqaphele kusenesikhathi. Ngaleyo ndlela, uma kwenzeka okuthile, ungaqiniseka ukuthi awuthuki.

Ingabe lokhu kungelapheka ngokuphelele?

Lena yinkinga enkulu kubantu abaningi. Ngeshwa, ayikho ikhambi le-Sézary Syndrome. Kodwa lokho akusho ukuthi kufanele ulahle ithemba . Kunezindlela eziningi zokwelapha ezingasiza ukulawula izimpawu, ukuvimba amangqamuzana angavamile ukuthi ahlukane futhi akhule, futhi kunciphise ukusabalala komdlavuza. Lezi zindlela zokwelapha zingathuthukisa ikhwalithi yokuphila kwakho.

Lesi sifo sihlala isikhathi esingakanani?

I-Sézary Syndrome iyisifo esingamahlalakhona. Lokhu kusho ukuthi akusona isifo esifika sidlule. Ungase udinge izinga elithile lokwelashwa kanye nokubhekwa kwezokwelapha impilo yakho yonke.

Angakanani amathuba okusinda?

Lena inkinga ebucayi, futhi ayithinti wonke umuntu ngendlela efanayo. Ngokusho kwedatha yamanje, cishe abantu abangu-24% abane-Sézary Syndrome basinda okungenani iminyaka emihlanu ngemva kwalesi sifo. Kodwa khumbula, lokhu kumane kuyisilinganiso esijwayelekile esisekelwe kokuhlangenwe nakho kwabanye. Wonke umuntu uhlukile. Lesi simo singathuthuka njengoba kutholakala ukwelashwa okusha nokusebenza kahle. Ngenxa yokuthi i-Sézary Syndrome iyisifo esingavamile, kunzima ukuqoqa ulwazi olwanele. Uma unemibuzo ngesimo sakho, kungcono ukubuza udokotela wakho. Angachaza amaqiniso ngesimo sakho.

Ngizinakekela kanjani?

Ukuphila ne-Sézary Syndrome kusho ukuthi udinga ukuzinakekela ngokukhethekile ngenkathi uphathwa impilo yakho yonke. Kungase kudingeke uthathe izinyathelo ezengeziwe zokuvikela isikhumba sakho.

• Kubaluleke kakhulu ukugwema ukuchayeka elangeni ngqo . Ezinye izindlela zokwelapha zingenza isikhumba sizwele elangeni.
• Landela imiyalelo kadokotela ngqo. Thatha imithi yakho ngesikhathi.
• Gcina isikhumba sakho sihlanzekile futhi usebenzise i-moisturizer (ngemuva kokubonisana nodokotela).
• Yidla ukudla okunempilo.
• Zama ukujabula ngokwengqondo ngangokunokwenzeka.

Kufanele ngimbone nini udokotela?

Izimpawu ze-Sézary Syndrome zingashintsha futhi zibhebhetheke ngokushesha. Ngakho-ke qaphela noma yiziphi izinguquko emzimbeni wakho. Uma ubona noma yikuphi kwalokhu okulandelayo, tshela udokotela wakho ngokushesha:

• Uma ubona izinguquko esikhumbeni sakho - ukubomvu noma ubumnyama obusakazekela ezindaweni eziningi zesikhumba sakho, amaqhubu amancane ekhula, noma amaqhubu amasha avela - lezi yizimpawu zokuthi isimo sakho singase sibe sibi kakhulu.
• Uma uthola izifo ezithathelwanayo njalo futhi zithatha isikhathi eside ukuphulukiswa. Uma unemfiva evame ukwenzeka kanye nobuhlungu bomzimba.

Yimiphi imibuzo okufanele uyibuze udokotela?

Uma une-Sézary Syndrome, ungase ube nemibuzo eminingi. Kuzoba usizo ukuxoxa ngayo nodokotela wakho. Ungabuza udokotela wakho imibuzo efana nale:

• Yikuphi ukwelashwa okungcono kakhulu kimi?
• Yimiphi imiphumela emibi engingayilindela kulezi zindlela zokwelapha? Ngingabhekana kanjani nayo?
• Ingabe kukhona izivivinyo zemitholampilo (ucwaningo lwezokwelapha ezintsha) engingahlanganyela kuzo?
• Ingabe umdlavuza ungabuya ngemva kokwelashwa? Kuthiwani uma ubuye?
• Yiziphi izinguquko okudingeka ngizenze endleleni yami yokuphila?

Uyini umehluko phakathi kwe-Mycosis Fungoides kanye ne-Sézary Syndrome?

Kungenzeka ukuthi uke wezwa la magama amabili ngaphambili. I-Mycosis fungoides nayo uhlobo lwe-cutaneous T-cell lymphoma ethinta isikhumba. Okusho ukuthi, itholakala emndenini ofanayo ne-Sézary syndrome. Iphinde ibangele ukuqubuka okubomvu noma okumnyama esikhumbeni. Kodwa-ke, umehluko omkhulu ukuthi, ngokungafani ne-Sézary syndrome, abantu abane-mycosis fungoides abanawo amangqamuzana omdlavuza asakazeka egazini labo. Kodwa-ke, ngezinye izikhathi i-mycosis fungoides ingaba yingozi kakhulu futhi ikhule ibe yi-Sézary syndrome. Ngakho-ke kukhona ukuxhumana phakathi kwalezi ezimbili.

Okokugcina, izinto okufanele uzikhumbule

I-Sézary Syndrome umdlavuza ongavamile, okhula ngokushesha, othinta isikhumba negazi. Ungasakazeka nakwezinye izingxenye zomzimba. Ekuqaleni, izimpawu zingase zibukeke njengesifo sesikhumba esivamile njenge-eczema, kodwa izimpawu ze-Sézary Syndrome ziba zimbi ngokushesha futhi azipheli kalula. Nakuba lesi simo singelapheki ngokuphelele, odokotela bangelapha izimpawu zakho futhi bavimbele lesi sifo ukuthi singasabalali sisuka esikhumbeni nasegazini siye kwezinye izitho zomzimba.

Ngakho-ke, uma ubona noma yikuphi ukulunywa okungavamile, okuqhubekayo, okunamandla, ukubomvu, amabala amnyama, noma ukuqina kwesikhumba sakho, kubalulekile ukubona udokotela, ikakhulukazi udokotela wesikhumba, ngokushesha okukhulu. Uma kutholakala kusenesikhathi , ukwelashwa kungashesha ukuqala. Kungenza umehluko omkhulu empilweni yakho.

I- Sézary syndrome, umdlavuza wesikhumba, i-T-cell lymphoma, izimpawu zesikhumba, i-erythroderma, ukwelashwa komdlavuza, ukulunywa kwesikhumba