Kodi Sézary Syndrome ndi chiyani? Kodi imakhudza bwanji khungu lanu?

Kodi munayamba mwaonapo kuti khungu lanu limasanduka lofiira mwadzidzidzi, kuyabwa kwambiri, komanso mdima m'malo ena? Mungaganize kuti ndi vuto lofala pakhungu, koma nthawi zina, lingakhale vuto lalikulu kwambiri. Ndicho chimene tikukamba lero, Sézary Syndrome, khansa yosowa yomwe imakhudza khungu, magazi, ndi ma lymph nodes. Mungasokonezedwe pang'ono ndi dzinalo, koma ndikofunikira kudziwa za izi.

Kodi Sézary Syndrome ndi chiyani?

Mwachidule, Sézary Syndrome ndi khansa ya pakhungu yomwe imafalikira mofulumira kwambiri komanso yosaoneka bwino. Mwachipatala, ili m'gulu la khansa yotchedwa cutaneous T-cell lymphoma. Ma T-lymphocytes, kapena ma T-cell, ndi mtundu wofunikira wa maselo m'thupi lathu. Mu matendawa, mtundu wa selo wotchedwa ma T-cell umakhala khansa ndipo umasonkhana pakhungu lanu, m'magazi, ndi m'malo olumikizirana mafupa.

Taganizirani ngati asilikali omwe amateteza thupi lathu, `(ma T-cell)` awa. Koma asilikaliwa akasochera ndikuyamba kutilimbana nafe, izi ndi zomwe zimachitika. Ichi ndichifukwa chake khungu lanu limasintha mtundu ndikuwoneka lofiira. Nthawi zina khungu limathanso kukhala lakuda. Palinso kuthekera kwa kuyabwa kwakukulu ndi zilonda zopweteka komanso ziphuphu. Choopsa kwambiri ndichakuti maselo a khansa awa m'magazi amatha kufalikira ku ziwalo zina za thupi, kutanthauza, ku ziwalo zamkati. Ngakhale pakadali pano palibe mankhwala athunthu a izi, musadandaule , pali mankhwala ambiri oletsa zizindikiro ndikukupatsani mpumulo.

Kodi vutoli ndi lofala bwanji?

Izi ndi zachilendo kwambiri. Ndiko kuti, ndi matenda omwe sapezeka kawirikawiri. Tangoganizirani, ngakhale m'dziko ngati America, munthu m'modzi pa anthu miliyoni imodzi ndi amene amanenedwa kuti amadwala matendawa chaka chilichonse. Choncho izi sizimapezeka kawirikawiri ku Sri Lanka. Ndicho chifukwa chake ndikofunikira kudziwa izi, chifukwa ngati zizindikiro zikuwonekera, zitha kuzindikirika mwachangu.

Kodi zizindikiro za Sézary Syndrome ndi ziti?

Chizindikiro chachikulu cha matendawa ndi ziphuphu zofiira (erythroderma) . Izi si kuyabwa kokha, zimayabwa kwambiri, zimapweteka, nthawi zina khungu limatuluka ndikufalikira mwachangu kwambiri. Kwa anthu ena, makamaka omwe ali ndi khungu lakuda, zimatha kuonekanso ngati mawanga akuda (omwe amatha kukhala imvi, ofiirira, kapena ofiirira). Poyamba, zizindikirozi zimakhala zovuta kuzizindikira chifukwa zimafanana ndi matenda wamba a pakhungu monga eczema kapena psoriasis. Anthu ambiri amaganiza kuti izi ndi vuto losavuta.

Pamene Sézary Syndrome ikupita patsogolo, khungu lanu lidzayamba kukhala ndi ziphuphu zazing'ono, zokwezeka (mapepala). Malo ena angamveke okhuthala komanso olimba kuposa ena (plaque). Pamapeto pake, khansa ya pakhungu (zotupa) ingayambe. Palinso zizindikiro zina zingapo, zomwe tidzaziyang'ana:

• Ma lymph nodes otupa - m'malo monga khosi, m'khwapa, ndi m'mimba.
• Malungo - Malungo omwe amabwera popanda chifukwa.
• Kutopa nthawi zonse - Kumva kutopa ngakhale mutagona tulo tochuluka bwanji.
• Kuchepetsa thupi kosamveka bwino.
• Kutaya tsitsi - Nthawi zina nsidze ndi tsitsi la thupi zimatha kutayika.
• Ectropion (chikope chopindika).
• Kuchuluka kwa chiwindi (hepatomegaly).
• Kutupa (edema), makamaka m'miyendo ya m'munsi.
• Kusalekerera kuzizira - Izi zikutanthauza kuti mumamva kuzizira kuposa ena, ngakhale kutentha kwa chipinda kumakhala koyenera.

Kodi n’chiyani chimayambitsa matenda a Sézary?

Izi zimachitika chifukwa maselo athanzi otchedwa ma T-cell m'thupi lathu amasinthidwa majini, zomwe zimawasandutsa maselo a khansa osazolowereka. Maselo osazolowerekawa amagawikana mofulumira ndikuchulukana mosalamulirika, pamapeto pake amafalikira pakhungu, m'maselo a lymph, ndi m'magazi. Zili ngati udzu, womwe umafalikira kulikonse popanda kulamulira. Komabe, akatswiri sakudziwabe chomwe chimayambitsa kusintha kwa majini kumeneku. Izi zikutanthauza kuti n'zovuta kunena chifukwa chake.

Kodi ndi zinthu ziti zomwe zimayambitsa izi?

Ngakhale kuti aliyense akhoza kudwala matendawa, zinthu zina zingakuwonjezereni chiopsezo chanu. Mwachitsanzo, ngati mwadwala kachilombo ka T-cell leukemia (HTLV), mukuganiza kuti muli pachiwopsezo chowonjezeka cha matenda a Sézary syndrome. Komabe, ndikofunikira kukumbukira kuti si aliyense amene ali ndi kachilomboka amene angatenge matendawa.

Kodi ndi mavuto otani omwe angayambitse matendawa?

Sézary Syndrome ndi matenda omwe angakhudze kwambiri moyo wa munthu. Tangoganizirani, mungamve bwanji bwino pamene mukukanda khungu lanu nthawi zonse komanso mukumva ululu? Zingayambitse zinthu monga kusowa tulo, nkhawa nthawi zonse, komanso kuvutika maganizo. Palinso mavuto ena angapo:

• Maselo a khansa a T amafalikira m'mapapo, chiwindi, ndulu, ndi m'mafupa.
• Kuwonjezeka kwa chiopsezo chotenga mtundu wina wa lymphoma (khansa ya dongosolo la lymphatic).
• Kuwonjezeka kwa chiopsezo cha matenda pafupipafupi chifukwa chakuti khungu loteteza limawonongeka, zomwe zimapangitsa kuti majeremusi alowe mosavuta.

Kodi madokotala amazindikira bwanji matendawa?

Mukapita kukaonana ndi dokotala, choyamba adzakuyesani thupi ndikukufunsani za zizindikiro zanu. Adzakufunsaninso za mbiri yanu yachipatala. Dokotalayo adzakufunsani za matenda anu akale.Ngati akukayikira kuti Sézary Syndrome ndi yolondola, akhoza kuchita mayeso angapo, monga:

• Kuyezetsa magazi: Izi ndizofunikira kwambiri. Izi zingaphatikizepo "Kuwerengera Magazi Konse (CBC)" (izi zimayang'ana kuchuluka kwa mitundu yosiyanasiyana ya maselo m'magazi, makamaka maselo oyera amagazi), "Kuwerengera kwa magazi a Sézary" (komwe kumayang'ana kuchuluka kwa maselo a Sézary (maselo a khansa omwe ali ndi matendawa) m'magazi, ndi "kuyesedwa kwa magazi m'mbali mwa thupi" (kuyezetsa magazi pogwiritsa ntchito microscope).
• Kufufuza za khungu, lymph node, kapena bone morrow: Mu izi, chidutswa chaching'ono cha minofu chimatengedwa kuchokera pamalo omwe akhudzidwa a khungu kapena chotupa ndikutumizidwa ku labotale. Kumeneko, katswiri wa matenda amayang'ana chitsanzo cha minofu pansi pa maikulosikopu. Izi zitha kuyang'ana zinthu monga zizindikiro za chotupa ndi kusintha kwa maselo.
• Mayeso oti muwone ngati khansa yafalikira: Ngati mayeso a magazi ndi minofu atsimikizira kuti muli ndi matenda a Sézary, mayesowo adzachitika kuti awone ngati khansa yafalikira ku ziwalo zina za thupi. Izi zingaphatikizepo X-ray pachifuwa, CT scans (computerized tomography), ndi Positron Emission Tomography (PET) scan (yomwe imayang'ana momwe maselo amagwirira ntchito m'thupi).

Kodi mankhwala a Sézary Syndrome ndi ati?

Dokotala wanu adzakupatsani chithandizo kutengera zizindikiro zanu komanso momwe khansa yanu yafalikira (momwe yafalikira). Pali njira zosiyanasiyana zochizira izi. Si aliyense amene amalandira chithandizo chofanana.

• Kuchiritsa ndi Kujambula: Izi zimagwiritsa ntchito mitundu yapadera ya kuwala kuti ziwononge maselo a khansa pakhungu. Njira imodzi imatchedwa extracorporeal photopheresis. Mu izi, maselo oyera amachotsedwa m'magazi anu (leukapheresis), amachiritsidwa ndi mankhwala ndikuwunikira kuwala kwa ultraviolet (UV), kenako nkubwezeretsedwa m'thupi lanu. Kapenanso, kuchiritsa ndi kuwala kwa UV kungaperekedwe mwachindunji pakhungu.
• Chithandizo cha radiation: Izi zimaphatikizapo kugwiritsa ntchito kuwala kwamphamvu kwambiri kuti kuwononge maselo a khansa. Izi zitha kuchitika pogwiritsa ntchito chithandizo cha radiation chakunja (pogwiritsa ntchito makina kuti apereke kuwala) kapena chithandizo cha radiation cha electron beam ya khungu lonse (mankhwala a radiation a electron beam ya khungu lonse).
• Chemotherapy: Izi zimaphatikizapo kupereka mankhwala opha maselo a khansa. Pali mitundu yomwe imagwiritsidwa ntchito pakhungu (mankhwala ochizira matenda a m'thupi) ndi mitundu yomwe imakhudza thupi lonse (mankhwala ochizira matenda a m'thupi) (omwe amaperekedwa ngati mapiritsi kapena jakisoni). Vorinostat (Zolinza®) ndi chitsanzo cha mankhwala omwe amaperekedwa ku thupi lonse.
• Chithandizo cholunjika: Mankhwalawa amaukira anthu omwe amapezeka pa maselo a khansa okha. Chithandizo cha monoclonal antibody ndi chimodzi mwa mankhwala olunjika omwe amagwiritsidwa ntchito pa matenda a Sézary.
• Chithandizo cha Immunotherapy: Izi zimaphatikizapo kulimbikitsa chitetezo cha mthupi lanu kuti chilimbane ndi maselo a khansa. Izi zitha kuchitika pogwiritsa ntchito mankhwala monga Interferon (Intron A®, Intron A Multidose Pen.®)).
• Mankhwala ena ochizira: Corticosteroids (mankhwala amtundu wa steroid) ndi mafuta kapena ma gels amtundu wa retinoid (monga bexarotene (Targretin®)) amagwiritsidwanso ntchito poletsa zizindikiro za pakhungu.

"Koma kumbukirani, si mankhwala onsewa omwe ali ofanana kwa aliyense. Dokotala wanu ndiye adzasankha chithandizo chabwino kwambiri cha vuto lanu. Iye adzakufotokozerani zonse."

Kodi zotsatirapo zake ndi ziti?

Mankhwala ambiri a khansa amakhala ndi zotsatirapo zina. Izi ndi zachilendo. Ndikofunikira kudziwa za izi pasadakhale. Kawirikawiri, `(chemotherapy)` ndi `(radiation therapy)` mungayembekezere zinthu monga kutopa, nseru, kusanza, kusowa chilakolako cha chakudya, ndi kutayika kwa tsitsi. `(Phototherapy)` ingakhudze khungu - nthawi zina khungu louma, kufiira. `(Targeted therapy)`, `(immunomodulators)` (mankhwala omwe amasintha chitetezo cha mthupi) ndi `(immunotherapy)` nthawi zina zingayambitse kutupa, kutsegula m'mimba, kutopa, ndi kusintha kwa zigawo za magazi `(kusakhazikika kwa kuchuluka kwa magazi)`. Ndibwino kulankhula ndi dokotala wanu za izi ndikudziwitsa pasadakhale. Mwanjira imeneyi, ngati china chake chachitika, mutha kukhala otsimikiza kuti simuchita mantha.

Kodi izi zingathe kuchiritsidwa kwathunthu?

Ili ndiye vuto lalikulu kwa anthu ambiri. Tsoka ilo, palibe mankhwala a Sézary Syndrome. Koma sizikutanthauza kuti muyenera kutaya chiyembekezo . Pali mankhwala ambiri omwe angathandize kuwongolera zizindikiro, kuletsa maselo osazolowereka kugawikana ndikukula, komanso kuchepetsa kufalikira kwa khansa. Mankhwalawa angathandize kuti moyo wanu ukhale wabwino.

Kodi matendawa amatenga nthawi yayitali bwanji?

Matenda a Sézary ndi matenda osatha. Izi zikutanthauza kuti si matenda omwe amabwera ndi kutha. Mungafunike chithandizo chamankhwala komanso kuyang'aniridwa ndi dokotala kwa moyo wanu wonse.

Kodi mwayi wopulumuka uli bwanji?

Nkhaniyi ndi yovuta kwambiri, ndipo sikhudza aliyense mofanana. Malinga ndi zomwe zapezeka pano, pafupifupi 24% ya anthu omwe ali ndi Sézary Syndrome amakhala ndi moyo kwa zaka zosachepera zisanu pambuyo pa matendawa. Koma kumbukirani, izi ndi kuyerekezera kokha kutengera zomwe ena akumana nazo. Aliyense ndi wosiyana. Izi zitha kusintha pamene mankhwala atsopano komanso ogwira mtima akupezeka. Chifukwa chakuti Sézary Syndrome ndi matenda osowa kwambiri, zimakhala zovuta kupeza zambiri zokwanira. Ngati muli ndi mafunso okhudza matenda anu, ndi bwino kufunsa dokotala wanu. Iye akhoza kufotokoza zoona za matenda anu.

Kodi ndimadzisamalira bwanji?

Kukhala ndi Sézary Syndrome kumatanthauza kuti muyenera kudzisamalira mwapadera pamene mukulandira chithandizo cha moyo wanu wonse. Mungafunike kuchitapo kanthu kuti muteteze khungu lanu.

• Ndikofunikira kwambiri kupewa kukhudzana ndi dzuwa mwachindunji . Mankhwala ena angapangitse khungu kukhala losavuta kukhudzidwa ndi dzuwa.
• Tsatirani malangizo a dokotala molondola. Imwani mankhwala anu pa nthawi yake.
• Sungani khungu lanu loyera ndipo gwiritsani ntchito mafuta odzola (mutakambirana ndi dokotala).
• Idyani zakudya zabwino.
• Yesetsani kukhala osangalala m'maganizo momwe mungathere.

Kodi ndiyenera kupita kwa dokotala liti?

Zizindikiro za Sézary Syndrome zimatha kusintha ndikuipiraipira mwachangu. Choncho dziwani kusintha kulikonse m'thupi lanu. Ngati muwona chilichonse mwa izi, uzani dokotala wanu nthawi yomweyo:

• Ngati muwona kusintha pakhungu lanu - kufiira kapena mdima kufalikira m'malo ambiri pakhungu lanu, ziphuphu zazing'ono zikukulirakulira, kapena ziphuphu zatsopano zikuonekera - izi ndi zizindikiro zakuti vuto lanu likhoza kuipiraipira.
• Ngati mumadwala matenda pafupipafupi ndipo amatenga nthawi yayitali kuti achire. Ngati mumadwala malungo pafupipafupi komanso kupweteka kwa thupi.

Ndi mafunso ati omwe muyenera kufunsa dokotala?

Ngati muli ndi Sézary Syndrome, mungakhale ndi mafunso ambiri. Zingakhale zothandiza kukambirana ndi dokotala wanu. Mutha kufunsa dokotala wanu mafunso monga awa:

• Ndi mankhwala ati omwe ndi abwino kwa ine?
• Kodi ndi zotsatirapo zotani zomwe ndingayembekezere kuchokera ku mankhwala awa? Kodi ndingathane nazo bwanji?
• Kodi pali mayesero aliwonse azachipatala (kafukufuku wa mankhwala atsopano) omwe ndingatenge nawo mbali?
• Kodi khansa ingabwererenso mutalandira chithandizo? Nanga bwanji ngati itabweranso?
• Ndi kusintha kotani komwe ndiyenera kusintha pa moyo wanga?

Kodi pali kusiyana kotani pakati pa Mycosis Fungoides ndi Sézary Syndrome?

Mwina munamvapo mayina awiriwa kale. Mycosis fungoides ndi mtundu wa lymphoma ya khungu ya T-cell yomwe imakhudza khungu. Ndiko kuti, ili m'banja lomwelo la Sézary syndrome. Imayambitsanso ziphuphu zofiira kapena zakuda pakhungu. Komabe, kusiyana kwakukulu ndikuti, mosiyana ndi Sézary syndrome, anthu omwe ali ndi mycosis fungoides alibe maselo ambiri a khansa (maselo a Sézary) omwe amazungulira m'magazi awo. Komabe, nthawi zina mycosis fungoides imatha kukhala yoopsa kwambiri ndikukhala Sézary syndrome. Chifukwa chake pali kulumikizana pakati pa awiriwa.

Pomaliza, zinthu zoti muzikumbukira

Sézary Syndrome ndi khansa yosowa, yomwe imakula mofulumira, yomwe imakhudza khungu ndi magazi. Imathanso kufalikira ku ziwalo zina za thupi. Poyamba, zizindikirozo zingawoneke ngati matenda ofala pakhungu monga eczema, koma zizindikiro za Sézary Syndrome zimakula mofulumira ndipo sizimatha mosavuta. Ngakhale kuti vutoli silingathe kuchiritsidwa kwathunthu, madokotala amatha kuchiza zizindikiro zanu ndikuletsa matendawa kufalikira kuchokera pakhungu ndi magazi kupita ku ziwalo zina.

Choncho, ngati muwona kuyabwa kulikonse kwachilendo, kosalekeza, koopsa, kufiira, mawanga akuda, kapena kukhuthala kwa khungu lanu, ndikofunikira kuonana ndi dokotala, makamaka dokotala wa khungu, mwachangu momwe mungathere. Kukapezeka msanga , chithandizo chimayamba msanga. Zingapangitse kusiyana kwakukulu pa moyo wanu.

Matenda a Sézary , khansa ya pakhungu, T-cell lymphoma, zizindikiro za pakhungu, erythroderma, chithandizo cha khansa, kuyabwa pakhungu