Sézary Mob: Cov tsos mob thiab lub neej cia siab

Zoo Siab

• Cov cim thiab tsos mob zoo li cas?
• Daim duab ntawm erythroderma
• Leej twg yuav muaj kev pheej hmoo?
• Dab tsi ua rau nws?
• Nws kuaj tau li cas?
• Tus mob Sézary mob li cas?
• Yuav kho nws li cas?
• Psoralen thiab UVA (PUVA)
• Kev Saib Xyuas Kho Mob Tshaj Plaws / Photopheresis (ECP)
• Kev siv hluav taws xob
• Kws khomob
• Kev Siv Tshuaj Tiv Thaiv (biologic therapy)
• Cov chaw kuaj mob
• Outlook

Tus Mob Sézary yog dab tsi?

Sézary syndrome yog ib daim ntawv uas txiav qhov mob T-cell lymphoma. Sézary cell yog ib hom qe ntshav dawb. Hauv cov mob no, cov qog nqaij hlav ntshav muaj peev xwm nrhiav tau nyob hauv cov ntshav, tawv nqaij, thiab cov qog ntshav. Cov kabmob kuj tseem tuaj yeem kis mus rau lwm yam kabmob.

Sézary syndrome yeej tsis tshua pom muaj ntau, tab sis nws ua txog 3 txog 5 feem pua ​​ntawm cov kab mob T-cell hlais ntshav. Koj tuaj yeem hnov ​​nws hu ua Sézary erythroderma lossis Sézary's lymphoma.

Cov cim thiab tsos mob zoo li cas?

Qhov pom ntawm tus mob Sézary syndrome yog erythroderma, tawv nqaij liab, khaus uas yuav los txog li 80 feem pua ​​ntawm lub cev. Lwm yam cim thiab tsos mob muaj xws li:

• o ntawm daim tawv nqaij
• cov tawv nqaij ua pliav thiab hlav
• tsam mob txhab ntshav
• tuab ntawm daim tawv nqaij ntawm xib teg thiab cov qab xib taws
• kev txawv txav ntawm cov rau tes thiab ntiv taw
• daim tawv muag qis uas tig sab nraud
• plaub hau poob
• teeb meem tswj lub cev kub

Sézary syndrome kuj tseem tuaj yeem ua rau tus po lossis ntxiv teeb meem nrog lub ntsws, nplooj siab, thiab lub plab zom mov. Yog muaj tus mob qog nqaij hlav ntau yuav ua rau muaj qhov mob qog nqaij hlav cancer ntau dua.

Daim duab ntawm erythroderma

Leej twg yuav muaj kev pheej hmoo?

Txhua tus neeg muaj tus mob Sézary syndrome, tab sis feem ntau yuav cuam tshuam rau cov neeg muaj hnub nyoog 60 xyoo.

Dab tsi ua rau nws?

Qhov tseeb yog vim li cas tsis paub meej. Tab sis feem coob ntawm cov neeg muaj tus mob Sézary muaj cov chromosomal txawv txav hauv DNA ntawm cov qog nqaij hlav cancer, tab sis tsis nyob hauv lub hlwb zoo. Cov no tsis yog kab mob txuas nrog, tab sis cov kev hloov tshwm sim hauv lub neej.

Feem ntau cov kev txawv txav yog cov DNA ploj los ntawm cov chromosomes 10 thiab 17 lossis DNA ntxiv rau cov chromosomes 8 thiab 17. Tseem, nws tsis paub meej tias cov kev txawv txav no ua rau mob cancer.

Nws kuaj tau li cas?

Kev kuaj lub cev ntawm koj cov tawv nqaij yuav ceeb toom tus kws kho mob txog sijhawm Sézary syndrome. Kev kuaj mob ntsuam xyuas tuaj yeem suav nrog kuaj ntshav kom paub cov cim (antigens) nyob rau thaj chaw ntawm cov ntshav hauv cov ntshav.

Ib yam li lwm yam mob qog nqaij hlav, kuaj ntshav me yog qhov zoo tshaj plaws los mus kuaj mob. Kev kuaj ib qho nqaij ntawm lub cev, tus kws kho mob yuav coj ib qho qauv me me ntawm cov nqaij mos. Tus kws khomob tseem yuav kuaj tus qauv hauv qab lub tshuab tsom saib cov qog nqaij hlav cancer.

Cov qog ntshav thiab lub cev pob txha kuj tuaj yeem raug kuaj. Kev ntsuam xyuas cov duab, xws li CT, MRI, lossis PET scans, tuaj yeem pab txiav txim siab seb puas yog mob cancer tau kis mus rau cov qog ntshav lossis lwm yam hauv lub cev.

Tus mob Sézary mob li cas?

Qeb qhia tau tias ntev npaum li cas mob cancer kis tau thiab qhov kev kho zoo tshaj plaws yog dabtsi.Sézary syndrome yog staged raws li hauv qab no:

• 1A: Tsawg dua li 10 feem pua ​​ntawm daim tawv nqaij them nyob rau hauv cov kab liab liab lossis cov quav hniav.
• 1B: Ntau tshaj 10 feem pua ​​ntawm daim tawv nqaij yog xim liab.
• 2A: Muaj pes tsawg ntawm daim tawv nqaij koom tes nrog. Cov qog ntshav ua kom loj, tab sis tsis mob qog noj ntshav.
• 2B: Ib lossis ntau dua cov qog uas loj dua 1 centimeter tau tsim rau ntawm daim tawv nqaij. Cov qog ntshav ua kom loj, tab sis tsis mob qog noj ntshav.
• 3A: Feem ntau ntawm cov tawv nqaij yog xim liab thiab tej zaum yuav muaj qog, plaques, lossis thaj ua rau thaj. Cov qog ntshav ua haujlwm yog qhov tseem ceeb lossis nthuav dav, tab sis tsis yog mob qog. Cov ntshav yuav yog lossis tsis muaj ob peb Sézary hlwb.
• 3B: Muaj qhov txhab tshwm thoob cov tawv nqaij feem ntau. Cov qog ntshav yuav hlob los yog yuav tsis loj. Tus naj npawb ntawm Sézary hlwb hauv cov ntshav muaj tsawg.
• 4A (1): Cov tawv nqaij voos npog npog qee qhov ntawm daim tawv nqaij. Cov qog ntshav yuav hlob los yog yuav tsis loj. Tus naj npawb ntawm Sézary hlwb hauv cov ntshav muaj ntau.
• 4A (2): Cov tawv nqaij voos npog npog qee qhov ntawm daim tawv nqaij. Muaj ntau cov qog ntshav thiab lub hlwb ua haujlwm tsis meej nyob rau hauv kev kuaj mob me me. Sézary cells yuav los yog tsis yog cov ntshav.
• 4B: Cov tawv nqaij voos npog npog qee qhov ntawm daim tawv nqaij. Cov qog ntshav hauv lub cev yuav ua haujlwm li qub. Sézary cells yuav los yog tsis yog cov ntshav. Lymphoma cell tau kis mus rau lwm yam kabmob lossis ntaub so ntswg.

Yuav kho nws li cas?

Ntau yam ua rau koj xav kho tus mob zoo tshaj plaws. Ntawm lawv yog:

• theem ntawm kev kuaj mob
• muaj hnub nyoog
• lwm yam teeb meem kev noj qab haus huv

Hauv qab no yog qee qhov kev kho mob ntawm Sézary syndrome.

Psoralen thiab UVA (PUVA)

Ib cov tshuaj hu ua psoralen, uas nyhav hauv cov qog nqaij hlav cancer, txhaj rau hauv cov leeg ntshav. Nws pib ua haujlwm thaum dhau los ntawm ultraviolet A (UVA) lub teeb qhia rau koj cov tawv nqaij. Txoj kev no rhuav tshem cov qog nqaij hlav cancer tsuas yog ua kom tsawg rau cov nqaij mos noj qab nyob zoo xwb.

Kev Saib Xyuas Kho Mob Tshaj Plaws / Photopheresis (ECP)

Tom qab tau txais cov tshuaj tshwj xeeb, qee cov qe ntshav raug tshem tawm ntawm koj lub cev. Lawv tau raug kho nrog UVA lub teeb ua ntej rov ua dua koj lub cev.

Kev siv hluav taws xob

Kev siv hluav taws xob ntau xoo hluav taws xob yog siv los rhuav tshem cov qog nqaij hlav cancer. Hauv cov kab hluav taws xob sab nraud, lub tshuab xa hluav taws xob mus rau thaj chaw ntawm koj lub cev. Hlawv roj yuav pab txo qhov mob thiab lwm yam mob. Kev kho hluav taws xob tag nrho cov tawv nqaij (TSB) kev siv hluav taws xob siv lub tshuab hluav taws xob sab nraud coj los siv hluav taws xob rau ntawm daim tawv nqaij hauv koj lub cev tag nrho.

Koj tuaj yeem muaj UVA thiab kev tiv thaiv hluav taws xob UV UV (UVB) siv hluav taws xob tshwj xeeb rau koj cov tawv nqaij.

Kws khomob

Kev siv tshuaj kho mob yog ib qho kev kho mob hauv nruab nrog uas cov tshuaj muaj zog siv los tua cov qog noj ntshav lossis txwv lawv cov kev faib tawm. Qee cov tshuaj siv tshuaj kho mob muaj nyob hauv cov qauv tshuaj, thiab lwm qhov yuav tsum tau txhaj tshuaj nrog.

Kev Siv Tshuaj Tiv Thaiv (biologic therapy)

Cov tshuaj xws li interferons tau siv los ua kom koj tus kheej lub cev tiv thaiv kab mob qog noj ntshav.

Cov tshuaj siv los kho Sézary syndrome suav nrog:

• alemtuzumab (Campath), yog ib qho tshuaj tiv thaiv monoclonal
• bexarotene (Targretin), ib qho retinoid
• brentuximab vedotin (Adcetris), tiv thaiv tshuaj-tshuaj tiv thaiv kev tsis haum tshuaj
• chlorambucil (Leukeran), kev siv tshuaj kho mob
• corticosteroids kom pab daws cov tsos mob ntawm daim tawv nqaij
• cyclophosphamide (Cytoxan), kev siv tshuaj kho mob
• denileukin difitox (Ontak), kev hloov pauv ntawm biologic teb
• gemcitabine (Gemzar), tshuaj tua kab mob antimetabolite
• interferon alfa lossis interleukin-2, tshuaj tiv thaiv kab mob
• lenalidomide (Revlimid), tus mob angiogenesis inhibitor
• liposomal doxorubicin (Doxil), kev siv tshuaj kho mob
• methotrexate (Trexall), tshuaj tua kab mob antimetabolite
• pentostatin (Nipent), tshuaj tua kab mob antimetabolite
• romidepsin (Istodax), ib qho keeb kwm deacetylase inhibitor
• vorinostat (Zolinza), ib qho tshuaj tiv thaiv histone deacetylase inhibitor

Koj tus kws kho mob yuav sau ntawv tawm tshuaj sib xyaw lossis tshuaj ntxiv nrog rau lwm cov kev kho mob. Qhov no yuav ua raws theem ntawm kev mob qog noj ntshav thiab qhov koj yuav tau txais kev khomob tshwj xeeb li cas.

Kev kho rau theem 1 thiab 2 feem ntau yuav muaj:

• pleev cov tshuaj tiv thaiv corticosteroids
• retinoids, lenalidomide, histone deacetylase inhibitors
• PUVA
• hluav taws xob nrog TSEB lossis UVB
• kev kho biologic los ntawm nws tus kheej lossis nrog kev kho tawv nqaij
• tshuaj pleev ib ce
• kev kho cov kab mob hauv nruab nrog cev, tejzaum ua ke nrog kev kho tawv nqaij

Theem 3 thiab 4 tuaj yeem kho nrog:

• pleev cov tshuaj tiv thaiv corticosteroids
• lenalidomide, bexarotene, histone deacetylase inhibitors
• PUVA
• ECP ib leeg lossis nrog TSEB
• hluav taws xob nrog TSEB lossis UVB thiab UVA hluav taws xob
• kev kho biologic los ntawm nws tus kheej lossis nrog kev kho tawv nqaij
• tshuaj pleev ib ce
• kev kho cov kab mob hauv nruab nrog cev, tejzaum ua ke nrog kev kho tawv nqaij

Yog tias cov kev kho mob tsis ua haujlwm ntxiv lawm, txoj kev hloov kho lub qog tuaj yeem yog kev xaiv.

Cov chaw kuaj mob

Kev tshawb fawb txog kev kho mob qog noj ntshav yog tseem niaj hnub, thiab kev kho mob hauv tsev kho mob yog ib feem ntawm cov txheej txheem ntawd. Hauv kev sim tshuaj, koj tuaj yeem nkag mus rau kev kho mob hauv av uas tsis muaj nyob rau lwm qhov chaw. Yog xav paub ntau ntxiv txog kev sim tshuaj, nug koj tus kws khomob lossis mus ntsib ClinicalTrials.gov.

Outlook

Sézary syndrome yog ib hom mob cancer tshwj xeeb. Nrog rau kev kho mob, koj tuaj yeem ua rau qeeb qeeb mob lossis txawm mus rau kev kho kom zoo. Tab sis lub cev tsis muaj zog tiv thaiv kab mob tuaj yeem ua rau koj muaj feem kis mob yooj yim thiab lwm yam mob qog nqaij hlav.

Qhov nruab nrab ntawm cov neeg muaj sia nyob tau ntev txog 2 txog 4 xyoos, tab sis cov qib no tau txhim kho nrog cov kev kho mob tshiab dua.

Mus ntsib koj tus kws kho mob thiab pib kho sai li sai tau kom ntseeg tau qhov kev pom zoo tshaj plaws.