Yintoni iSézary Syndrome? Ichaphazela njani ulusu lwakho?

Ngaba wakha waphawula ukuba ulusu lwakho lujika lube bomvu ngequbuliso, lurhawuzelele kakhulu, luze lube mnyama kwezinye iindawo? Usenokucinga ukuba yimeko eqhelekileyo yolusu, kodwa kwiimeko ezingaqhelekanga, inokuba yinto enzulu ngakumbi. Yiloo nto sithetha ngayo namhlanje, iSézary Syndrome, umhlaza ongaqhelekanga ochaphazela ulusu, igazi, kunye ne-lymph nodes. Usenokudideka kancinci ngeli gama, kodwa kubalulekile ukuliqaphela.

Yintoni iSézary Syndrome?

Ngamafutshane, iSézary Syndrome luhlobo lomhlaza wesikhumba olungaqhelekanga nolusasazeka ngokukhawuleza. Ngokwezonyango, lukwiqela lomhlaza olubizwa ngokuba yi-cutaneous T-cell lymphoma. Ii-T-lymphocytes, okanye ii-T-cells ngamafutshane, luhlobo olubalulekileyo lweeseli kwinkqubo yomzimba yethu yokuzikhusela. Kwesi sifo, uhlobo lweseli olubizwa ngokuba zii-T-cells luba ngumhlaza kwaye luqokelelane eluswini lwakho, egazini, nakwii-lymph nodes.

Cinga njengamadoda akhusela imizimba yethu, la `(ii-T-cells)`. Kodwa xa la majoni ephambuka aze aqale ukusebenza nxamnye nathi, nantsi into eyenzekayo. Yiyo loo nto ulusu lwakho lutshintsha umbala luze lubonakale lubomvu. Ngamanye amaxesha ulusu lunokuba mnyama. Kukwakho nethuba lokuba kubekho ukurhawuzelela okukhulu kunye nezilonda ezibuhlungu kunye namaqhuqhuva. Eyona nto iyingozi kukuba la maseli omhlaza egazini anokusasazeka kwezinye iindawo zomzimba, oko kukuthi, kwizitho zangaphakathi. Nangona okwangoku kungekho nyango lupheleleyo loku, ungakhathazeki , kukho unyango oluninzi lokulawula iimpawu kunye nokukunika isiqabu.

Ixhaphake kangakanani le meko?

Oku kunqabile kakhulu. Oko kukuthi, sisifo esingafane sibonwe. Khawuthelekelele, nakwilizwe elifana neMelika, ngumntu omnye kwisigidi kuphela oxelwa ukuba unesifo minyaka le. Ngoko ke oku akusiyonto ibonwa rhoqo eSri Lanka. Yiyo loo nto kubalulekile ukuqaphela oku, kuba ukuba iimpawu ziyavela, zinokubonwa ngokukhawuleza.

Zithini iimpawu zeSézary Syndrome?

Uphawu oluphambili lwesi sifo kukurhawuzelelwa okubomvu okwahlukileyo (i-erythroderma) . Oku akukokurhawuzelelwa nje okulula, kuyarhawuzelela kakhulu, kuyabuhlungu, ngamanye amaxesha ulusu luyaxobuka luze lusasazeke ngokukhawuleza. Kwabanye abantu, ingakumbi abo banesikhumba esimnyama, kunokubonakala njengamabala amnyama (anokuba ngwevu, mfusa, okanye mdaka). Kwinqanaba lokuqala, ezi mpawu zinokuba nzima ukuzibona kuba zifana neemeko eziqhelekileyo zesikhumba ezifana ne-eczema okanye i-psoriasis. Abantu abaninzi bacinga ukuba oku kukunganyamezelani nje.

Njengoko iSézary Syndrome iqhubeka, ulusu lwakho luya kuqala ukuba namaqhuma amancinci aphakanyisiweyo (iipapules). Ezinye iindawo zinokuziva zityebile kwaye ziqinile kunezinye (i-plaque). Ekugqibeleni, umhlaza wolusu (ii-tumor) unokuvela. Kukwakho nezinye iimpawu ezimbalwa, esiza kuzijonga:

• Ii-lymph nodes ezivuvukileyo - kwiindawo ezifana nentamo, emakhwapheni, nasemqolo.
• Umkhuhlane - Umkhuhlane oqala ngaphandle kwesizathu.
• Ukudinwa rhoqo - Ukuziva udiniwe nokuba ulala kangakanani na.
• Ukunciphisa umzimba ngendlela engachazekiyo.
• Ukulahleka kweenwele - Ngamanye amaxesha amashiya kunye neenwele zomzimba zinokulahleka.
• I-Ectropion (ijwabu leso elijijekileyo).
• Ukwanda kwesibindi (i-hepatomegaly).
• Ukudumba (ukudumba), ingakumbi kwimilenze engezantsi.
• Ukunganyamezelani nokubanda - Oku kuthetha ukuba uziva ubanda kunabanye, nokuba kubushushu obuqhelekileyo begumbi.

Yintoni ebangela iSézary Syndrome?

Oku kwenzeka kuba iiseli ezisempilweni ezibizwa ngokuba zii-T-cells emzimbeni wethu zifumana utshintsho lwezakhi zofuzo, zizenze zibe ziiseli zomhlaza ezingaqhelekanga. Ezi seli ezingaqhelekanga zahlukana ngokukhawuleza kwaye zande ngokungalawulekiyo, ekugqibeleni zisasazeke eluswini, kwii-lymph nodes, nasegazini. Kufana nokhula, olusasazeka kuyo yonke indawo ngaphandle kokulawulwa. Nangona kunjalo, iingcali azikazi ukuba yintoni kanye kanye ebangela olu tshintsho lwezakhi zofuzo. Oko kuthetha ukuba kunzima ukutsho kanye kanye ukuba kutheni.

Zithini izinto ezibangela oku?

Nangona nabani na enokuba nesi sifo, ezinye izinto zinokunyusa umngcipheko wakho. Umzekelo, ukuba ukhe wosulelwa yintsholongwane ye-T-cell leukemia (HTLV), kucingelwa ukuba usemngciphekweni okhulayo wokuba ne-Sézary syndrome. Nangona kunjalo, kubalulekile ukukhumbula ukuba ayinguye wonke umntu osulelwe yile ntsholongwane oza kuba nesi sifo.

Ziziphi iingxaki ezinokubakho zesi sifo?

I-Sézary Syndrome sisifo esinokuchaphazela kakhulu umgangatho wobomi. Khawucinge nje, ungaziva njani uphilile xa uhlala ukrwempa ulusu lwakho kwaye uneentlungu? Inokubangela izinto ezifana nokungalali, ixhala elingapheliyo, kunye nokudakumba. Kukwakho nezinye iingxaki ezininzi:

• Ii-T-cells ezinesifo somhlaza zisasazeka kwimiphunga yakho, esibindini, kwi-spleen, nakumongo wethambo.
• Umngcipheko okhulayo wokufumana olunye uhlobo lwe-lymphoma (umhlaza wenkqubo ye-lymphatic).
• Umngcipheko ophezulu wokosuleleka rhoqo ngenxa yokuba umaleko wokhuseleko lolusu wonakele, nto leyo evumela iintsholongwane ukuba zingene lula.

Oogqirha bayixilonga njani le sifo?

Xa usiya kugqirha, uza kuqala akuhlole aze akubuze ngeempawu zakho. Uza kubuza nangembali yakho yezonyango yangaphambili. Ugqirha uzaUkuba kurhanelwa ukuba uSézary Syndrome unesifo, unokwenza iimvavanyo ezininzi, ezifana nezi:

• Uvavanyo lwegazi: Oku kubaluleke kakhulu. Oku kungabandakanya "uBalo olupheleleyo lwegazi (i-CBC)" (oku kujonga ipesenti yeentlobo ezahlukeneyo zeeseli egazini, ingakumbi iiseli ezimhlophe zegazi), "ubalo lwegazi lwe-Sézary" (olujonga inani leeseli ze-Sézary" (iiseli zomhlaza eziqhelekileyo kwesi sifo) egazini, kunye "novavanyo lwegazi oluphuma ecaleni" (uvavanyo lwesampulu yegazi oluqhutywa ngemicroscope).
• Iibhayopsy zesikhumba, i-lymph node, okanye i-bone marrow: Kule nto, kuthathwa iqhekeza elincinci lezicubu kwindawo echaphazelekayo yesikhumba okanye iqhuma lize lithunyelwe kwilebhu. Apho, ingcali yezonyango ihlola isampuli yezicubu phantsi kwe-microscope. Oku kunokukhangela izinto ezifana neempawu ze-tumor kunye notshintsho kwiiseli.
• Uvavanyo lokujonga ukuba umhlaza usasazekile na: Ukuba uvavanyo lwegazi kunye nezicubu luqinisekisa ukuba unesifo se-Sézary, uvavanyo luya kwenziwa ukuze kubonwe ukuba umhlaza usasazeke na kwezinye iindawo zomzimba. Ezi zinokubandakanya i-X-ray yesifuba, ii-CT scans (computerized tomography), kunye ne-Positron Emission Tomography (PET) scan (ejonga umsebenzi weeseli emzimbeni).

Ziziphi iindlela zonyango lweSézary Syndrome?

Ugqirha wakho uza kukunika unyango olusekelwe kwiimpawu zakho kunye nenqanaba lomhlaza wakho (ukuba usasazeke kangakanani). Kukho unyango olwahlukeneyo loku. Asinguye wonke umntu ofumana unyango olufanayo.

• I-Phototherapy: Oku kusebenzisa iintlobo ezikhethekileyo zokukhanya ukutshabalalisa iiseli zomhlaza eluswini. Enye indlela ibizwa ngokuba yi-extracorporeal photopheresis. Kule ndlela, iiseli ezimhlophe zegazi ziyasuswa egazini lakho (i-leukapheresis), zinyangwe ngeyeza kwaye zibekwe kukukhanya kwe-ultraviolet (ukukhanya kwe-UV), zize zibuyiselwe emzimbeni wakho. Kungenjalo, i-UV phototherapy inokunikwa ngqo eluswini.
• Unyango ngemitha: Oku kubandakanya ukusebenzisa imitha enamandla aphezulu ukutshabalalisa iiseli zomhlaza. Oku kungenziwa ngokusebenzisa unyango lwemitha lwangaphandle (kusetyenziswa umatshini ukuhambisa imitha) okanye unyango lwemitha yemitha ye-electron yesikhumba iyonke (unyango lwemitha ye-electron yesikhumba iyonke).
• I-Chemotherapy: Oku kubandakanya ukunika amayeza abulala iiseli zomhlaza. Kukho iintlobo ezisetyenziswa eluswini (i-topical chemotherapy) kunye neentlobo ezichaphazela umzimba wonke (i-systemic chemotherapy) (nokuba zinikwa njengeepilisi okanye njengenaliti). I-Vorinostat (Zolinza®) ngumzekelo weyeza elinikwa umzimba wonke.
• Unyango olujoliswe kuko: Olu nyango luhlasela iinjongo ezithile ezifumaneka kuphela kwiiseli zomhlaza. Unyango lwe-Monoclonal antibody lolunye lolo nyango lujoliswe kuko olusetyenziselwa i-Sézary syndrome.
• Unyango lwe-immunotherapy: Oku kuquka ukuvuselela amajoni omzimba wakho ukuze alwe neeseli zomhlaza. Oku kungenziwa kusetyenziswa amayeza anjenge-Interferon (Intron A®, Intron A Multidose Pen.®)).
• Ezinye iindlela zonyango zamayeza: IiCorticosteroids (amayeza anjenge-steroid) kunye neekhrimu okanye iijeli zohlobo lwe-retinoid (umz. i-bexarotene (Targretin®)) nazo zisetyenziselwa ukulawula iimpawu zolusu.

"Kodwa khumbula, ayizizo zonke ezi ndlela zonyango ezifanayo kuye wonke umntu. Ugqirha wakho uya kugqiba ngolona nyango lufanelekileyo kwimeko yakho. Uza kukuchazela yonke into."

Zithini iziphumo ebezingalindelekanga zonyango?

Uninzi lwezonyango zomhlaza lunemiphumo emibi. Oku kuqhelekile. Kubalulekile ukuba uzazi kwangaphambili. Ngokubanzi, `(chemotherapy)` kunye `(radiation therapy)` ungalindela izinto ezifana nokudinwa, isicaphucaphu, ukuhlanza, ukuphelelwa ngumdla wokutya, kunye nokulahleka kweenwele. `(Phototherapy)` inokuchaphazela ulusu - ngamanye amaxesha ulusu olomileyo, ukubomvu. `(Targeted therapy)`, `(immunomodulators)` (amayeza atshintsha inkqubo yomzimba yokuzikhusela) kunye `(immunotherapy)` ngamanye amaxesha zinokubangela ukudumba, urhudo, ukudinwa, kunye notshintsho kwiinxalenye zegazi `(ukungalingani kwenani legazi)`. Yingcinga elungileyo ukuthetha nogqirha wakho ngale nto kwaye uyiqaphele kwangaphambili. Ngale ndlela, ukuba kukho into eyenzekayo, unokuqiniseka ukuba awuphazami.

Ngaba oku kunganyangwa ngokupheleleyo?

Le yeyona ngxaki inkulu kubantu abaninzi. Ngelishwa, akukho nyango lweSézary Syndrome. Kodwa oko akuthethi ukuba ufanele ulahle ithemba . Kukho unyango oluninzi olunokunceda ukulawula iimpawu, ukuthintela iiseli ezingaqhelekanga ukuba zingahlukani kwaye zikhule, kwaye unciphise ukusasazeka komhlaza. Olu nyango lunokuphucula umgangatho wobomi bakho.

Ihlala ixesha elingakanani le sifo?

I-Sézary Syndrome yimeko engapheliyo. Oku kuthetha ukuba ayisosifo esifika sidlule. Ungadinga unyango oluthile kunye nolawulo lwezonyango ubomi bakho bonke.

Angakanani amathuba okusinda?

Le yingxaki ebucayi, kwaye ayichaphazeli wonke umntu ngendlela efanayo. Ngokwedatha yangoku, malunga nama-24% abantu abaneSézary Syndrome baphila ubuncinane iminyaka emihlanu emva kwesi sifo. Kodwa khumbula, olu luqikelelo nje oluqhelekileyo olusekelwe kumava abanye. Wonke umntu wahlukile. Le meko inokuphucula njengoko unyango olutsha nolusebenzayo lufumaneka. Ngenxa yokuba iSézary Syndrome sisifo esingaqhelekanga, kunzima ukuqokelela ulwazi olwaneleyo. Ukuba unemibuzo malunga nemeko yakho, kungcono ubuze ugqirha wakho. Angachaza iinyani malunga nemeko yakho.

Ndingazinyamekela njani?

Ukuphila neSézary Syndrome kuthetha ukuba kufuneka uzikhathalele ngokukodwa ngelixa usenza unyango ubomi bakho bonke. Kusenokufuneka uthathe amanyathelo ongezelelweyo okukhusela ulusu lwakho.

• Kubaluleke kakhulu ukuphepha ukukhanya kwelanga ngqo . Ezinye iindlela zonyango zingenza ulusu lube buthathaka kwilanga.
• Landela imiyalelo kagqirha ngokuchanekileyo. Sela amayeza akho ngexesha.
• Gcina ulusu lwakho lucocekile kwaye usebenzise i-moisturizer (emva kokubonisana nogqirha).
• Yitya ukutya okunempilo.
• Zama ukuba nolonwabo lwengqondo kangangoko unako.

Ndifanele ndimbone nini ugqirha?

Iimpawu zeSézary Syndrome zinokutshintsha kwaye zibe mandundu ngokukhawuleza. Ngoko ke lumka naluphi na utshintsho emzimbeni wakho. Ukuba ubona nayiphi na kwezi zilandelayo, xelela ugqirha wakho ngoko nangoko:

• Ukuba ubona utshintsho eluswini lwakho - ukuba bomvu okanye ukuba mnyama kusasazeka kwiindawo ezininzi zolusu lwakho, amaqhuma amancinci ekhula, okanye kuvela amaqhuma amatsha - ezi ziimpawu zokuba imeko yakho isenokuba mandundu.
• Ukuba ufumana usulelo rhoqo kwaye luthatha ixesha elide ukuphola. Ukuba unefiva rhoqo kunye neentlungu zomzimba.

Yeyiphi imibuzo omele uyibuze ugqirha?

Ukuba uneSézary Syndrome, usenokuba nemibuzo emininzi. Kuya kuba luncedo ukuyixoxa nogqirha wakho. Ungabuza ugqirha wakho imibuzo efana nale:

• Zeziphi iindlela zonyango ezilungele mna?
• Zeziphi iziphumo ebezingalindelekanga endinokuzilindela kwezi ndlela zonyango? Ndingajongana njani nazo?
• Ngaba kukho naziphi na iimvavanyo zeklinikhi (uphando malunga nonyango olutsha) endinokuthi ndithathe inxaxheba kuzo?
• Ngaba umhlaza ungabuya emva konyango? Kuthekani ukuba uyabuya?
• Zeziphi iinguqu ekufuneka ndizenze kwindlela endiphila ngayo?

Yintoni umahluko phakathi kweMycosis Fungoides kunye neSézary Syndrome?

Usenokuba ukhe waweva la magama mabini ngaphambili. I-Mycosis fungoides ikwaluhlobo lwe-cutaneous T-cell lymphoma echaphazela ulusu. Oko kukuthi, ikwintsapho enye ne-Sézary syndrome. Ikwabangela ukurhawuzelela okubomvu okanye okumnyama eluswini. Nangona kunjalo, umahluko ophambili kukuba, ngokungafaniyo ne-Sézary syndrome, abantu abane-mycosis fungoides abanazo iiseli zomhlaza ezijikeleza egazini labo. Nangona kunjalo, ngamanye amaxesha i-mycosis fungoides inokuba nzima kwaye ikhule ibe yi-Sézary syndrome. Ngoko ke kukho unxibelelwano phakathi kwezi zimbini.

Okokugqibela, izinto ekufuneka uzikhumbule

I-Sézary Syndrome ngumhlaza ongaqhelekanga, okhula ngokukhawuleza, ochaphazela ulusu negazi. Ingasasazeka nakwamanye amalungu omzimba. Ekuqaleni, iimpawu zinokubonakala ngathi yimeko eqhelekileyo yesikhumba efana ne-eczema, kodwa iimpawu ze-Sézary Syndrome ziba mandundu ngokukhawuleza kwaye azipheli lula. Nangona le meko ingenakunyangwa ngokupheleleyo, oogqirha banokunyanga iimpawu zakho kwaye bathintele ukuba isifo singasasazeki ukusuka eluswini nasegazini siye kwamanye amalungu omzimba.

Ngoko ke, ukuba ubona naluphi na uhlobo lokurhawuzelela okungaqhelekanga, okungapheliyo, okuqatha, ukuba bomvu, amabala amnyama, okanye ukuqina kolusu lwakho, kubalulekile ukubona ugqirha, ingakumbi ingcali yesikhumba, ngokukhawuleza. Okukhona kubonwa kwangoko , kokukhona unyango lunokuqala ngokukhawuleza. Kunokwenza umahluko omkhulu ebomini bakho.

Isifo sikaSézary , umhlaza wolusu, i-T-cell lymphoma, iimpawu zolusu, i-erythroderma, unyango lomhlaza, ukurhawuzelelwa lusu