Sézary Syndrome: Zizindikiro ndi Chiyembekezo cha Moyo

Zamkati

• Zizindikiro zake ndi ziti?
• Chithunzi cha erythroderma
• Ndani ali pachiwopsezo?
• Zimayambitsa chiyani?
• Kodi amapezeka bwanji?
• Kodi matenda a Sézary amapezeka bwanji?
• Amachizidwa bwanji?
• Psoralen ndi UVA (PUVA)
• Zowonjezera photochemotherapy / photopheresis (ECP)
• Thandizo la radiation
• Chemotherapy
• Immunotherapy (mankhwala a biologic)
• Mayesero azachipatala
• Chiwonetsero

Kodi Sézary syndrome ndi chiyani?

Matenda a Sézary ndi mawonekedwe a T-cell lymphoma. Maselo a Sézary ndi mtundu wina wama cell oyera. Momwemonso, maselo a khansa amapezeka m'magazi, khungu, ndi ma lymph node. Khansara imatha kufalikira ku ziwalo zina.

Matenda a Sézary siofala kwambiri, koma amapanga 3 mpaka 5 peresenti ya T-cell lymphomas yodulira. Mutha kumvanso kuti Sézary erythroderma kapena Sézary's lymphoma.

Zizindikiro zake ndi ziti?

Chizindikiro chodziwika bwino cha matenda a Sézary ndi erythroderma, zotupa zofiira, zoyipa zomwe zimatha kuphimba pafupifupi 80 peresenti ya thupi. Zizindikiro zina ndi monga:

• kutupa kwa khungu
• zikopa za khungu ndi zotupa
• ma lymph node owonjezera
• khungu lakuda pamanja ndi zidendene
• zovuta za zikhadabo ndi zikhadabo
• zikope zapansi zomwe zimayang'ana kunjaku
• kutayika tsitsi
• zovuta zowongolera kutentha kwa thupi

Matenda a Sézary amathanso kuyambitsa nthenda yotupa kapena mavuto m'mapapu, chiwindi, komanso m'mimba. Kukhala ndi khansa yamtunduwu kumawonjezera chiopsezo chokhala ndi khansa zina.

Chithunzi cha erythroderma

Ndani ali pachiwopsezo?

Aliyense atha kudwala matenda a Sézary, koma mwina amakhudza anthu azaka zopitilira 60.

Zimayambitsa chiyani?

Zomwe zimayambitsa sizimveka bwino. Koma anthu ambiri omwe ali ndi matenda a Sézary ali ndi vuto la chromosomal mu DNA yama cell a khansa, koma osati m'maselo athanzi. Izi si zolakwika zobadwa nazo, koma zosintha zomwe zimachitika kwanthawi yayitali.

Zovuta zodziwika bwino ndikutaya kwa DNA kuchokera kuma chromosomes 10 ndi 17 kapena kuwonjezera kwa DNA kuma chromosomes 8 ndi 17. Komabe, sizikudziwika kuti zovuta izi zimayambitsa khansa.

Kodi amapezeka bwanji?

Kuunika khungu lanu kumatha kudziwitsa dokotala za matenda a Sézary. Kuyezetsa matenda kumatha kuphatikizira kuyesa magazi kuti azindikire zolembera (ma antigen) pamwamba pamaselo m'magazi.

Mofanana ndi khansa zina, biopsy ndiyo njira yabwino kwambiri yodziwira matenda. Pofufuza, adotolo amatenga pang'ono khungu. Katswiri wazachipatala adzaunika zitsanzozo pogwiritsa ntchito microscope kuti ayang'ane maselo a khansa.

Mafupa am'mimba ndi mafupa amatha kupangidwanso. Kuyesa kuyerekezera, monga CT, MRI, kapena PET scan, kungathandize kudziwa ngati khansa yafalikira ku ma lymph node kapena ziwalo zina.

Kodi matenda a Sézary amapezeka bwanji?

Staging imanena momwe khansara yafalikira komanso momwe njira zabwino zochiritsira ziliri.Matenda a Sézary adapangidwa motere:

• 1A: Pafupifupi 10 peresenti ya khungu limakutidwa ndi zigamba zofiira kapena zikwangwani.
• 1B: Oposa 10 peresenti ya khungu ndi lofiira.
• 2A: Kuchuluka kulikonse kwa khungu kumakhudzidwa. Mafupa am'mimba amakula, koma osati khansa.
• 2B: Chotupa chimodzi kapena zingapo zokulirapo kuposa 1 sentimita zapanga pakhungu. Mafupa am'mimba amakula, koma osati khansa.
• 3A: Khungu lambiri limakhala lofiira ndipo limakhala ndi zotupa, zikwangwani, kapena zigamba. Ma lymph lymph ndi abwinobwino kapena kukulitsidwa, koma osati khansa. Magazi atha kukhala kapena sangakhale ndi maselo ochepa a Sézary.
• 3B: Pali zotupa zambiri pakhungu. Ma lymph lymph atha kukulitsidwa kapena sangakulitsidwe. Chiwerengero cha maselo a Sézary m'magazi ndi ochepa.
• 4A (1): Zilonda za khungu zimaphimba mbali iliyonse yakhungu. Ma lymph lymph atha kukulitsidwa kapena sangakulitsidwe. Maselo a Sézary m'magazi ndi okwera.
• 4A (2): Zilonda za khungu zimaphimba mbali iliyonse yakhungu. Pali ma lymph node owonjezera ndipo ma cell amawoneka osazolowereka poyesedwa pang'ono. Maselo a Sézary atha kukhala kapena sangakhale m'magazi.
• 4B: Zilonda za khungu zimaphimba mbali iliyonse ya khungu. Ma lymph node amatha kukhala abwinobwino kapena osazolowereka. Maselo a Sézary atha kukhala kapena sangakhale m'magazi. Maselo a Lymphoma afalikira ku ziwalo zina kapena minofu.

Amachizidwa bwanji?

Pali zifukwa zingapo zomwe zimakhudza chithandizo chomwe chingakhale chabwino kwa inu. Zina mwa izo ndi izi:

• siteji pakuzindikira
• zaka
• mavuto ena azaumoyo

Otsatirawa ndi ena amachiritso a Sézary syndrome.

Psoralen ndi UVA (PUVA)

Mankhwala otchedwa psoralen, omwe amakonda kusonkhanitsa m'maselo a khansa, amalowetsedwa mumtsempha. Amayiyika ikayatsidwa kuwala kwa ultraviolet A (UVA) yolunjika pakhungu lanu. Izi zimawononga maselo a khansa osavulaza kwenikweni minofu yabwinobwino.

Zowonjezera photochemotherapy / photopheresis (ECP)

Mukalandira mankhwala apadera, maselo ena amwazi amachotsedwa mthupi lanu. Amathandizidwa ndi kuwala kwa UVA asanabwezeretsedwe m'thupi lanu.

Thandizo la radiation

Mphamvu zazikulu za X-ray zimagwiritsidwa ntchito kuwononga maselo a khansa. Pozungulira ma radiation a kunja, makina amatumiza cheza m'malo olunjika a thupi lanu. Thandizo la radiation lingathetsenso ululu ndi zisonyezo zina. Mankhwala onse a khungu la electron beam (TSEB) amagwiritsa ntchito makina amtundu wakunja kuti apange ma electron pakhungu la thupi lanu lonse.

Muthanso kukhala ndi mankhwala a radiation a UVA ndi ultraviolet B (UVB) pogwiritsa ntchito kuwala kwapadera komwe kumakhudza khungu lanu.

Chemotherapy

Chemotherapy ndi mankhwala amachitidwe momwe mankhwala amphamvu amagwiritsidwira ntchito kupha ma cell a khansa kapena kusiya magawano awo. Mankhwala ena a chemotherapy amapezeka pamapiritsi, ndipo ena ayenera kupatsidwa kudzera m'mitsempha.

Immunotherapy (mankhwala a biologic)

Mankhwala monga ma interferon amagwiritsidwa ntchito kupangitsa chitetezo chamthupi chanu kulimbana ndi khansa.

Mankhwala omwe amagwiritsidwa ntchito pochiza matenda a Sézary ndi awa:

• alemtuzumab (Campath), antioclonal antibody
• bexarotene (Targretin), retinoid
• brentuximab vedotin (Adcetris), mankhwala osokoneza bongo
• chlorambucil (Leukeran), mankhwala a chemotherapy
• corticosteroids kuti athetse mawonekedwe akhungu
• cyclophosphamide (Cytoxan), mankhwala a chemotherapy
• denileukin difitox (Ontak), wosintha poyankha biologic
• gemcitabine (Gemzar), mankhwala a antimetabolite chemotherapy
• interferon alfa kapena interleukin-2, zoteteza ku chitetezo cha mthupi
• lenalidomide (Revlimid), choletsa angiogenesis
• liposomal doxorubicin (Doxil), mankhwala a chemotherapy
• methotrexate (Trexall), mankhwala a antimetabolite chemotherapy
• pentostatin (Nipent), mankhwala a antimetabolite chemotherapy
• romidepsin (Istodax), histone deacetylase inhibitor
• vorinostat (Zolinza), histone deacetylase inhibitor

Dokotala wanu akhoza kukupatsani mankhwala osakanikirana kapena mankhwala ena. Izi zitengera gawo la khansa komanso momwe mungayankhire mankhwala ena.

Chithandizo cha gawo 1 ndi 2 chikuyenera kuphatikizira:

• apakhungu corticosteroids
• retinoids, lenalidomide, histone deacetylase zoletsa
• PUVA
• radiation ndi TSEB kapena UVB
• mankhwala a biologic palokha kapena ndi khungu
• apakhungu chemotherapy
• systemic chemotherapy, mwina kuphatikiza ndi khungu

Magawo 3 ndi 4 atha kulandira chithandizo ndi:

• apakhungu corticosteroids
• lenalidomide, bexarotene, histone deacetylase inhibitors
• PUVA
• ECP yokha kapena ndi TSEB
• radiation ndi TSEB kapena UVB ndi UVA radiation
• mankhwala a biologic palokha kapena ndi chithandizo cha khungu
• apakhungu chemotherapy
• systemic chemotherapy, mwina kuphatikiza ndi khungu

Ngati mankhwala sakugwiranso ntchito, kupatula ma cell a stem kungakhale kosankha.

Mayesero azachipatala

Kafukufuku wazithandizo za khansa akupitilirabe, ndipo mayesero azachipatala ndi gawo la njirayi. Pakuyesa kwamankhwala, mutha kukhala ndi mwayi wopeza zochiritsira zapadera zomwe sizipezeka kwina kulikonse. Kuti mumve zambiri zamayeso azachipatala, funsani oncologist wanu kapena pitani ku ClinicalTrials.gov.

Chiwonetsero

Matenda a Sézary ndi khansa yoopsa kwambiri. Ndi chithandizo, mutha kuchepetsa kukula kwa matenda kapena ngakhale kukhululukidwa. Koma chitetezo chamthupi chofooka chimatha kukusiyani pachiwopsezo cha matenda opatsirana komanso khansa zina.

Avereji ya kupulumuka yakhala zaka 2 mpaka 4, koma mlingowu ukukulira ndi mankhwala atsopano.

Onani dokotala wanu ndipo yambani kuchipatala posachedwa kuti muwone bwino.