Sézary Syndrome: Zviratidzo uye Hupenyu Husingatarisiri

Zvemukati

• Ndezvipi zviratidzo nezviratidzo?
• Mufananidzo we erythroderma
• Ndiani ari panjodzi?
• Chii chinokonzera?
• Inoonekwa sei?
• Sézary syndrome inorongwa sei?
• Inorapwa sei?
• Psoralen uye UVA (PUVA)
• Yakawedzera photochemotherapy / photopheresis (ECP)
• Radiation kurapa
• Chemotherapy
• Immunotherapy (biologic kurapa)
• Kliniki miedzo
• Outlook

Chii chinonzi Sézary syndrome?

Sézary syndrome chimiro chechepfu T-cell lymphoma. Sézary masero ndeimwe mhando yerudzi rweropa. Mune mamiriro aya, maseru ane cancer anogona kuwanikwa muropa, ganda, uye lymph node. Gomarara rinogona kutapukirawo kune dzimwe nhengo.

Sézary syndrome haina kujairika, asi inoumba 3 kusvika ku5 muzana yekucheka T-cell lymphomas. Iwe unogona zvakare kuinzwa ichinzi Sézary erythroderma kana Sézary's lymphoma.

Ndezvipi zviratidzo nezviratidzo?

Chiratidzo chinozivikanwa cheSézary syndrome ierythroderma, tsvuku, inopenya iyo inogona kupedzisira yavhara kusvika makumi masere muzana emuviri. Zvimwe zviratidzo nezviratidzo zvinosanganisira:

• kuzvimba kweganda
• ganda matanda uye mamota
• akawedzera lymph node
• kuomarara kweganda pazvanza nematsoka
• kukanganisika kwenzara nezvigunwe
• maziso ezasi anotendeuka kunze
• kurasikirwa nevhudzi
• dambudziko kudzora tembiricha yemuviri

Sézary syndrome inogona zvakare kukonzera yakawedzera spleen kana matambudziko nemapapu, chiropa, uye gastrointestinal turakiti. Kuva neichi chimiro chine hukasha chekenza kunowedzera mukana wekubata imwe kanzira.

Mufananidzo we erythroderma

Ndiani ari panjodzi?

Chero ani zvake anogona kuvandudza Sézary syndrome, asi zvinonyanya kukanganisa vanhu vanopfuura makore makumi matanhatu.

Chii chinokonzera?

Chikonzero chaicho hachina kujeka. Asi vanhu vazhinji vane Sézary syndrome vane chromosomal zvisizvo muDNA yemasero ane cancer, asi kwete mumasero ane hutano. Izvi hazvisi zvikanganiso zvakagarwa nhaka, asi shanduko dzinoitika pamusoro pehupenyu hwese.

Izvo zvakajairika zvakajairika kurasikirwa kweDNA kubva kuma chromosomes 10 uye 17 kana DNA kuwedzerwa kune chromosomes 8 uye 17. Zvakadaro, hazvisi chokwadi kuti izvi zvisizvo zvinokonzeresa cancer.

Inoonekwa sei?

Kuongorora kwepanyama kweganda rako kunogona kuzivisa chiremba nezve mukana weSézary syndrome. Kuongororwa kuongororwa kunogona kusanganisira kuongororwa kweropa kuti uone mamaki (maantigen) pamusoro pemasero muropa.

Sezvimwe nemamwe kanzira, biopsy ndiyo nzira yakanakisa yekusvika pakuongororwa. Kune biopsy, chiremba achatora svumbunuro diki yeganda. Nyanzvi yezvirwere anoongorora muyenzaniso pasi pe microscope kutsvaga masero ekenza.

Lymph nodes uye mapfupa emapfupa anogona zvakare kuve biopsied. Kufungidzira bvunzo, senge CT, MRI, kana PET scans, zvinogona kubatsira kuona kana kenza yapararira kune ma lymph node kana zvimwe nhengo.

Sézary syndrome inorongwa sei?

Staging inotaura kuti kenza yapararira zvakadii uye ndeipi nzira dzakanakisa dzekurapa.Sézary syndrome yakarongedzwa seinotevera:

• 1A: Asingasviki gumi muzana yeganda rakafukidzwa nezvitsvuku kana mabara.
• 1B: Kupfuura gumi muzana yeganda rakatsvuka.
• 2A: Chero huwandu hweganda hunobatanidzwa. Lymph node yakawedzerwa, asi kwete cancer.
• 2B: Rimwe kana mamwe mamota akakura kupfuura 1 sentimita akagadzirwa paganda. Lymph node yakawedzerwa, asi kwete cancer.
• 3A: Mazhinji eganda akatsvuka uye anogona kunge aine mamota, mabara, kana zvigamba. Lymph node akajairwa kana kuwedzerwa, asi kwete cancer. Ropa rinogona kana kunge risina mashoma maseru eSézary.
• 3B: Kune maronda pamusoro peganda rakawanda. Lymph node inogona kana kusakwanisa kuwedzerwa. Huwandu hweSézary masero muropa ishoma.
• 4A (1): Mahombekombe eganda anofukidza chero chikamu cheganda pamusoro. Lymph node inogona kana kusakwanisa kuwedzerwa. Huwandu hweSézary masero muropa hwakakwira.
• 4A (2): Mahombekombe eganda anofukidza chero chikamu cheganda pamusoro. Kune ma lymph node akawedzeredzwa uye maseru anotaridzika kunge asina kujairika pasi peongororo diki. Sézary masero anogona kana kunge asiri muropa.
• 4B: Mahombekombe eganda anofukidza chero chikamu cheganda pamusoro. Iyo lymph node inogona kuve yakajairika kana isina kujairika. Sézary masero anogona kana kunge asiri muropa. Lymphoma masero akapararira kune dzimwe nhengo kana zvinyama.

Inorapwa sei?

Zvimwe zvinhu zvinokanganisa kurapwa kupi kungave kwakanakira iwe. Pakati pavo pane:

• danho pakuongororwa
• zera
• mamwe matambudziko ehutano

Izvi zvinotevera mamwe marapirwo eSézary syndrome.

Psoralen uye UVA (PUVA)

Mushonga unonzi psoralen, uyo unowanzounganidza mumasero egomarara, unopinzwa mutsinga. Inoita kuti ishandiswe painoratidzwa kune ultraviolet A (UVA) mwenje inotungamirwa paganda rako. Maitiro aya anoparadza maseru ekenza nekukuvara kushoma kune zvinyama zvine hutano.

Yakawedzera photochemotherapy / photopheresis (ECP)

Mushure mekugamuchira akakosha madhiragi, mamwe masero eropa anobviswa kubva mumuviri wako. Vanorapwa neUVA mwenje vasati vadzoreredzwa kumuviri wako.

Radiation kurapa

Yakakwira-simba X-ray inoshandiswa kuparadza cancer maseru. Mune yekunze beam radiation, muchina unotumira mwaranzi kunzvimbo dzakanangwa dzemuviri wako. Radiation kurapa kunogona kubvisa marwadzo uye zvimwe zviratidzo futi. Yese ganda electron beam (TSEB) radiation kurapwa inoshandisa yekunze radiation michina kunongedza maerekitironi paganda remuviri wako wese.

Iwe unogona zvakare kuve neUVA uye ultraviolet B (UVB) radiation kurapwa uchishandisa mwenje wakanangana neganda rako.

Chemotherapy

Chemotherapy inzira yekurapa iyo mishonga ine simba inoshandiswa kuuraya cancer maseru kana kumisa kupatsanurwa kwavo. Mimwe mishonga yekemotherapy inowanikwa mupiritsi fomu, uye vamwe vanofanirwa kupihwa mukati.

Immunotherapy (biologic kurapa)

Zvinodhaka zvakaita sema interferon zvinoshandiswa kukurudzira immune system yako kurwisa gomarara.

Mishonga inoshandiswa kurapa Sézary syndrome inosanganisira:

• alemtuzumab (Campath), monoclonal antibody
• bexarotene (Targretin), iro retinoid
• brentuximab vedotin (Adcetris), inorwisa-antibody zvinodhaka
• chlorambucil (Leukeran), chemotherapy mushonga
• corticosteroids kubvisa zviratidzo zveganda
• cyclophosphamide (Cytoxan), chemotherapy mushonga
• denileukin difitox (Ontak), biologic mhinduro modifier
• gemcitabine (Gemzar), antimetabolite chemotherapy
• interferon alfa kana interleukin-2, immune zvinokurudzira
• lenalidomide (Revlimid), angiogenesis inhibitor
• liposomal doxorubicin (Doxil), chemotherapy mushonga
• methotrexate (Trexall), antimetabolite chemotherapy
• pentostatin (Nipent), antimetabolite chemotherapy
• romidepsin (Istodax), histone deacetylase inhibitor
• vorinostat (Zolinza), histone deacetylase inhibitor

Chiremba wako anogona kuraira kusanganiswa kwemishonga kana madhiragi pamwe nemamwe marapirwo. Izvi zvichave zvinoenderana nedanho rekenza uye nemabatiro aunoita kune kumwe kurapwa.

Kurapa kwechitsauko 1 ne2 kunogona kusanganisira:

• yemusoro corticosteroids
• retinoids, lenalidomide, histone deacetylase inhibitors
• PUVA
• radiation ine TSEB kana UVB
• biologic kurapa pachayo kana nerapi reganda
• topical chemotherapy
• systemic chemotherapy, pamwe inosanganiswa neyekurapa kweganda

Matanho 3 ne4 anogona kurapwa ne:

• yemusoro corticosteroids
• lenalidomide, bexarotene, histone deacetylase inhibitors
• PUVA
• ECP yoga kana neTSSEB
• radiation ine TSEB kana UVB uye UVA radiation
• biologic kurapa pachayo kana nerapi reganda
• topical chemotherapy
• systemic chemotherapy, pamwe inosanganiswa neyekurapa kweganda

Kana kurapwa kusisiri kushanda, iyo stem cell kuisirwa inogona kunge iri sarudzo.

Kliniki miedzo

Kutsvagisa kwekurapa kenza kuri kuenderera, uye kiriniki miedzo chikamu cheizvozvo. Mune kiriniki yekuyedzwa, iwe unogona kunge uine mukana wekuputsa kwepasi kurapwa kusingawanikwe chero kumwe kunhu. Kuti uwane rumwe ruzivo nezve kiriniki miedzo, bvunza wako oncologist kana shanyira ClinicalTrials.gov.

Outlook

Sézary syndrome inonyanya kuita gomarara rine hukasha. Nokurapwa, iwe unogona kukwanisa kunonoka kukura kwechirwere kana kutopinda mukuregererwa. Asi immune system isina kusimba inogona kukusiya uchinetseka kutapukirwa nenjodzi uye mimwe kanzira.

Avhareji kupona kwave kuri maviri kusvika mana makore, asi chiyero ichi chiri kuvandudzika nemishonga mitsva.

Ona chiremba wako uye tanga kurapwa nekukurumidza sezvinobvira kuti uve nechokwadi cheakanyanya kunaka maonero.