Kab mob Sézary Syndrome yog dab tsi? Nws cuam tshuam li cas rau koj daim tawv nqaij?

Koj puas tau pom tias koj daim tawv nqaij hloov xim liab, khaus heev, thiab qee qhov tsaus ntuj? Tej zaum koj yuav xav tias nws tsuas yog ib qho mob ntawm daim tawv nqaij xwb, tab sis qee zaum, nws tuaj yeem yog qee yam loj dua. Qhov ntawd yog qhov peb tab tom tham txog hnub no, Sézary Syndrome, ib qho mob qog noj ntshav tsawg uas cuam tshuam rau daim tawv nqaij, ntshav, thiab cov qog ntshav. Tej zaum koj yuav tsis meej pem me ntsis los ntawm lub npe, tab sis nws yog ib qho tseem ceeb uas yuav tsum paub txog nws.

Sézary Syndrome yog dab tsi?

Hais yooj yim xwb, Sézary Syndrome yog ib hom mob qog nqaij hlav tawv nqaij uas tsis tshua muaj thiab kis tau sai heev. Hauv kev kho mob, nws yog ib pawg mob qog nqaij hlav hu ua cutaneous T-cell lymphoma. T-lymphocytes, lossis luv luv T-cells, yog ib hom cell tseem ceeb hauv peb lub cev tiv thaiv kab mob. Hauv tus kab mob no, ib hom cell hu ua T-cells dhau los ua mob qog nqaij hlav thiab sib sau ua ke hauv koj daim tawv nqaij, ntshav, thiab cov qog ntshav.

Xav txog nws zoo li cov tub rog uas tiv thaiv peb lub cev, cov '(T-cells)' no. Tab sis thaum cov tub rog no mus yuam kev thiab pib ua haujlwm tawm tsam peb, qhov no yog qhov tshwm sim. Qhov no yog vim li cas koj daim tawv nqaij hloov xim thiab zoo li liab. Qee zaum daim tawv nqaij kuj tuaj yeem tsaus ntuj. Kuj tseem muaj qhov ua rau khaus thiab mob thiab pob khaus. Qhov txaus ntshai tshaj plaws yog tias cov hlwb qog nqaij hlav hauv cov ntshav no tuaj yeem kis mus rau lwm qhov ntawm lub cev, uas yog, mus rau cov kabmob sab hauv. Txawm hais tias tam sim no tsis muaj kev kho mob tiav rau qhov no, tsis txhob txhawj , muaj ntau txoj kev kho mob los tswj cov tsos mob thiab muab kev pab rau koj.

Qhov teeb meem no feem ntau tshwm sim li cas?

Qhov no yeej tsis tshua muaj. Yog li ntawd, nws yog ib yam kab mob uas tsis tshua pom. Xav txog, txawm tias nyob hauv lub tebchaws zoo li Asmeskas, tsuas yog ib tus neeg hauv ib lab tus neeg tau tshaj tawm tias muaj tus kab mob no txhua xyoo. Yog li qhov no tsis yog ib yam dab tsi uas pom ntau zaus hauv Sri Lanka. Yog vim li cas nws thiaj li tseem ceeb kom paub txog qhov no, vim tias yog tias cov tsos mob tshwm sim, lawv tuaj yeem txheeb xyuas tau sai.

Cov tsos mob ntawm Sézary Syndrome yog dab tsi?

Cov tsos mob tseem ceeb ntawm tus kab mob no yog ib qho pob liab tshwj xeeb (erythroderma) . Qhov no tsis yog tsuas yog khaus xwb, nws khaus heev, mob heev, qee zaum daim tawv nqaij tev tawm thiab kis mus sai heev. Rau qee tus neeg, tshwj xeeb tshaj yog cov neeg uas muaj daim tawv nqaij tsaus dua, nws kuj tuaj yeem tshwm sim ua cov pob tsaus (uas tuaj yeem yog xim grey, ntshav, lossis xim av). Thaum ntxov, cov tsos mob no yuav nyuaj rau pom vim tias lawv zoo li cov mob ntawm daim tawv nqaij xws li eczema lossis psoriasis. Ntau tus neeg xav tias qhov no yog kev ua xua yooj yim.

Thaum tus mob Sézary Syndrome loj zuj zus tuaj, koj daim tawv nqaij yuav pib tsim cov pob me me, tsa (papules). Qee qhov chaw yuav tuab dua thiab khov dua li lwm qhov (plaque). Thaum kawg, mob qog nqaij hlav (qog nqaij hlav) yuav tshwm sim. Kuj tseem muaj ob peb yam tsos mob ntxiv, uas peb yuav saib:

• Cov qog ntshav o - nyob rau hauv cov chaw xws li caj dab, qhov tso, thiab qhov ncauj tsev menyuam.
• Kub taub hau - Kub taub hau uas tshwm sim yam tsis muaj laj thawj.
• Qaug zog tas li - Muaj kev xav tias qaug zog txawm tias koj tau pw tsaug zog npaum li cas los xij.
• Poob phaus yam tsis paub.
• Poob Plaub Hau - Qee zaum cov plaub muag thiab txawm tias cov plaub hau ntawm lub cev kuj tuaj yeem poob tau.
• Ectropion (qhov muag ntxeev).
• Kev loj hlob ntawm daim siab (hepatomegaly).
• O (edema), tshwj xeeb tshaj yog nyob rau hauv qab ob txhais ceg.
• Tsis kam txias - Qhov no txhais tau tias koj xav tias txias dua li lwm tus, txawm tias nyob rau hauv chav tsev kub li qub.

Dab tsi ua rau muaj Sézary Syndrome?

Qhov no tshwm sim vim tias cov hlwb noj qab haus huv hu ua T-cells hauv peb lub cev hloov pauv caj ces, ua rau lawv hloov mus ua cov hlwb mob qog noj ntshav tsis zoo. Cov hlwb tsis zoo no faib sai thiab loj hlob tsis tuaj yeem tswj tau, thaum kawg kis mus rau daim tawv nqaij, cov qog ntshav, thiab cov ntshav. Nws zoo li cov nroj tsuag, kis mus rau txhua qhov chaw yam tsis muaj kev tswj hwm. Txawm li cas los xij, cov kws tshaj lij tseem tsis paub meej tias dab tsi ua rau muaj cov kev hloov pauv caj ces no. Qhov ntawd txhais tau tias nws nyuaj rau hais meej tias yog vim li cas.

Cov yam ua rau muaj kev pheej hmoo no yog dab tsi?

Txawm hais tias leej twg los yeej muaj tus kab mob no, tab sis muaj tej yam uas ua rau koj muaj feem yuav mob ntxiv. Piv txwv li, yog tias koj tau kis tus kab mob human T-cell leukemia virus (HTLV), koj yuav muaj feem yuav mob Sézary syndrome ntau dua me ntsis. Txawm li cas los xij, nws yog ib qho tseem ceeb uas yuav tsum nco ntsoov tias tsis yog txhua tus neeg uas muaj tus kab mob no yuav mob.

Cov teeb meem uas yuav tshwm sim los ntawm tus kab mob no yog dab tsi?

Kab mob Sézary Syndrome yog ib yam kab mob uas muaj peev xwm cuam tshuam rau lub neej zoo. Xav txog seb, koj yuav ua li cas thiaj li zoo thaum koj khawb koj daim tawv nqaij tas li thiab mob? Nws tuaj yeem ua rau muaj tej yam xws li pw tsis tsaug zog, ntxhov siab tas li, thiab nyuaj siab. Kuj tseem muaj ntau yam teeb meem ntxiv:

• Cov qe ntshav T uas muaj mob qog nqaij hlav kis mus rau koj lub ntsws, lub siab, lub po, thiab cov pob txha.
• Muaj feem yuav mob lwm hom lymphoma (mob qog nqaij hlav ntawm lub cev lymphatic).
• Muaj feem yuav kis tau kab mob ntau zaus vim tias txheej tiv thaiv ntawm daim tawv nqaij raug puas tsuaj, ua rau cov kab mob nkag tau yooj yim dua.

Cov kws kho mob kuaj mob li cas?

Thaum koj mus ntsib kws kho mob, nws yuav kuaj koj lub cev ua ntej thiab nug koj txog koj cov tsos mob. Nws kuj tseem yuav nug txog koj keeb kwm kev mob yav dhau los. Tus kws kho mob yuavYog tias muaj kev xav tias muaj tus kab mob Sézary Syndrome, nws yuav ua ntau yam kev kuaj mob, xws li:

• Kev kuaj ntshav: Qhov no tseem ceeb heev. Qhov no yuav suav nrog "Kev suav ntshav tag nrho (CBC)" (qhov no kuaj xyuas feem pua ​​ntawm ntau hom cell hauv cov ntshav, tshwj xeeb yog cov cell ntshav dawb), "Kev suav ntshav Sézary" (uas kuaj xyuas tus lej ntawm "Sézary cells" (cov cell mob qog noj ntshav uas feem ntau yog cov kab mob no) hauv cov ntshav), thiab "kev kuaj ntshav ntawm cov ntshav sab nraud" (kev kuaj ntshav los ntawm lub tshuab microscopic).
• Kev kuaj tawv nqaij, cov qog ntshav hauv lub cev, lossis cov pob txha: Hauv qhov no, ib daim ntaub me me raug coj los ntawm thaj chaw uas raug mob ntawm daim tawv nqaij lossis ib lub pob thiab xa mus rau chav kuaj mob. Nyob ntawd, tus kws kho mob kuaj xyuas cov ntaub so ntswg hauv qab lub tshuab microscope. Qhov no tuaj yeem nrhiav cov khoom xws li cov cim qog thiab kev hloov pauv hauv cov hlwb.
• Kev kuaj xyuas seb puas muaj mob qog noj ntshav kis mus rau lwm qhov chaw: Yog tias kev kuaj ntshav thiab cov nqaij ntawm lub cev lees paub tias koj muaj mob Sézary syndrome, kev kuaj yuav raug ua kom pom tias mob qog noj ntshav puas tau kis mus rau lwm qhov chaw ntawm lub cev. Cov no yuav suav nrog kev thaij duab X-ray hauv siab, CT scans (computerized tomography), thiab Positron Emission Tomography (PET) scan (uas saib cov haujlwm ntawm cov cell hauv lub cev).

Cov kev kho mob rau Sézary Syndrome yog dab tsi?

Koj tus kws kho mob yuav sau ntawv kho mob raws li koj cov tsos mob thiab theem ntawm koj tus mob qog noj ntshav (nws kis mus deb npaum li cas). Muaj ntau txoj kev kho mob rau qhov no. Tsis yog txhua tus neeg tau txais kev kho mob zoo ib yam.

• Kev Kho Mob Los Ntawm Duab: Qhov no siv cov teeb tshwj xeeb los tua cov hlwb qog nqaij hlav hauv daim tawv nqaij. Ib txoj kev hu ua extracorporeal photopheresis. Hauv qhov no, cov qe ntshav dawb raug tshem tawm ntawm koj cov ntshav (leukapheresis), kho nrog tshuaj thiab raug rau lub teeb ultraviolet (UV teeb), thiab tom qab ntawd rov qab mus rau koj lub cev. Xwb, UV phototherapy tuaj yeem muab ncaj qha rau ntawm daim tawv nqaij.
• Kev Kho Mob Hluav Taws Xob: Qhov no suav nrog kev siv cov hluav taws xob muaj zog heev los tua cov hlwb qog noj ntshav. Qhov no tuaj yeem ua tiav los ntawm kev siv kev kho mob hluav taws xob sab nraud (siv lub tshuab los xa cov hluav taws xob) lossis kev kho mob hluav taws xob tag nrho ntawm daim tawv nqaij (kev kho mob hluav taws xob tag nrho ntawm daim tawv nqaij).
• Kev Kho Mob Siv Tshuaj Kho Mob: Qhov no suav nrog kev muab cov tshuaj uas tua cov hlwb qog noj ntshav. Muaj cov hom uas siv rau ntawm daim tawv nqaij (kev kho mob siv tshuaj kho mob sab nraud) thiab cov hom uas cuam tshuam rau tag nrho lub cev (kev kho mob siv tshuaj kho mob thoob plaws lub cev) (muab ua tshuaj ntsiav lossis txhaj tshuaj). Vorinostat (Zolinza®) yog ib qho piv txwv ntawm cov tshuaj uas muab rau tag nrho lub cev.
• Kev kho mob tsom rau cov hom phiaj: Cov kev kho mob no tawm tsam cov hom phiaj tshwj xeeb uas tsuas pom muaj nyob rau ntawm cov hlwb qog noj ntshav xwb. Kev kho mob Monoclonal antibody yog ib qho kev kho mob tsom rau cov hom phiaj uas siv rau Sézary syndrome.
• Kev Kho Mob Tiv Thaiv Kab Mob: Qhov no suav nrog kev txhawb nqa koj lub cev lub cev tiv thaiv kab mob kom tawm tsam cov hlwb qog noj ntshav. Qhov no tuaj yeem ua tiav los ntawm kev siv cov tshuaj xws li Interferon (Intron A®, Intron A Multidose Pen.®)).
• Lwm yam kev kho mob siv tshuaj: Corticosteroids (tshuaj hom steroid) thiab retinoid-type creams lossis gels (piv txwv li bexarotene (Targretin®)) kuj siv los tswj cov tsos mob ntawm daim tawv nqaij.

"Tiamsis nco ntsoov, tsis yog txhua yam kev kho mob no zoo ib yam rau txhua tus. Koj tus kws kho mob yuav txiav txim siab seb yuav kho koj tus mob li cas. Nws yuav piav qhia txhua yam rau koj."

Cov kev mob tshwm sim ntawm kev kho mob yog dab tsi?

Feem ntau cov kev kho mob qog noj ntshav muaj qee qhov kev phiv. Qhov no yog ib txwm muaj. Nws yog ib qho tseem ceeb uas yuav tsum paub txog lawv ua ntej. Feem ntau, '(kev kho mob chemotherapy)' thiab '(kev kho mob hluav taws xob)' koj tuaj yeem xav tias yuav muaj tej yam xws li qaug zog, xeev siab, ntuav, tsis xav noj mov, thiab plaub hau poob. '(Kev kho mob Phototherapy)' tuaj yeem cuam tshuam rau daim tawv nqaij - qee zaum daim tawv nqaij qhuav, liab. '(Kev kho mob tsom mus rau qhov muag), '(cov tshuaj immunomodulators)' (cov tshuaj uas hloov lub cev tiv thaiv kab mob) thiab '(kev kho mob immunotherapy)' qee zaum tuaj yeem ua rau o, raws plab, qaug zog, thiab hloov pauv ntawm cov ntshav '(kev suav cov ntshav tsis zoo)'. Nws yog ib lub tswv yim zoo los tham nrog koj tus kws kho mob txog qhov no thiab paub txog nws ua ntej. Ua li ntawd, yog tias muaj qee yam tshwm sim, koj tuaj yeem paub tseeb tias tsis txhob ntshai.

Qhov no puas kho tau tag nrho?

Qhov no yog qhov teeb meem loj tshaj plaws rau ntau tus neeg. Hmoov tsis zoo, tsis muaj kev kho rau Sézary Syndrome. Tab sis qhov ntawd tsis tau txhais hais tias koj yuav tsum tso tseg kev cia siab . Muaj ntau txoj kev kho mob uas tuaj yeem pab tswj cov tsos mob, tiv thaiv cov hlwb tsis zoo los ntawm kev faib thiab loj hlob, thiab qeeb qeeb kev kis tus kab mob qog noj ntshav. Cov kev kho mob no tuaj yeem txhim kho koj lub neej zoo.

Tus kab mob no kav ntev npaum li cas?

Kab mob Sézary yog ib yam mob uas mob ntev mus tas li. Qhov no txhais tau tias nws tsis yog ib yam mob uas tuaj thiab mus. Tej zaum koj yuav xav tau kev kho mob thiab kev saib xyuas mob rau koj lub neej.

Muaj feem yuav muaj sia nyob li cas?

Qhov no yog ib qho teeb meem rhiab heev, thiab nws tsis cuam tshuam rau txhua tus tib yam. Raws li cov ntaub ntawv tam sim no, kwv yees li 24% ntawm cov neeg uas muaj Sézary Syndrome muaj sia nyob tsawg kawg yog tsib xyoos tom qab tus kab mob. Tab sis nco ntsoov, qhov no tsuas yog kev kwv yees dav dav raws li kev paub ntawm lwm tus. Txhua tus neeg txawv. Qhov xwm txheej no yuav zoo dua thaum muaj cov kev kho mob tshiab, zoo dua. Vim tias Sézary Syndrome yog ib qho kab mob tsis tshua muaj, nws nyuaj rau sau cov ntaub ntawv txaus. Yog tias koj muaj lus nug txog koj tus mob, nws yog qhov zoo tshaj plaws los nug koj tus kws kho mob. Nws tuaj yeem piav qhia qhov tseeb txog koj tus mob.

Kuv yuav saib xyuas kuv tus kheej li cas?

Nyob nrog Sézary Syndrome txhais tau tias koj yuav tsum tau saib xyuas koj tus kheej tshwj xeeb thaum koj tab tom kho mob mus tas li. Tej zaum koj yuav tsum tau ua cov kauj ruam ntxiv los tiv thaiv koj daim tawv nqaij.

• Nws yog ib qho tseem ceeb heev uas yuav tsum tsis txhob raug tshav ntuj ncaj qha . Qee cov kev kho mob tuaj yeem ua rau daim tawv nqaij rhiab heev rau tshav ntuj.
• Ua raws li tus kws kho mob cov lus qhia kom meej. Noj koj cov tshuaj raws sijhawm.
• Khaws koj daim tawv nqaij kom huv si thiab siv cov tshuaj pleev kom noo noo (tom qab sab laj nrog kws kho mob).
• Noj zaub mov kom noj qab nyob zoo.
• Sim ua kom koj lub siab zoo siab li sai tau.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

Cov tsos mob ntawm Sézary Syndrome tuaj yeem hloov pauv thiab hnyav zuj zus sai. Yog li ntawd, ceev faj txog txhua yam kev hloov pauv hauv koj lub cev. Yog tias koj pom ib qho ntawm cov hauv qab no, qhia rau koj tus kws kho mob tam sim ntawd:

• Yog koj pom muaj kev hloov pauv ntawm koj daim tawv nqaij - liab lossis tsaus ntuj nthuav mus rau ntau qhov chaw ntawm koj daim tawv nqaij, cov pob me me loj zuj zus, lossis cov pob tshiab tshwm sim - cov no yog cov cim qhia tias koj tus mob yuav hnyav zuj zus tuaj.
• Yog koj muaj mob ntau zaus thiab lawv siv sijhawm ntev heev los kho. Yog koj kub taub hau thiab mob lub cev ntau zaus.

Koj yuav tsum nug tus kws kho mob li cas?

Yog koj muaj Sézary Syndrome, tej zaum koj yuav muaj ntau lo lus nug. Nws yuav pab tau yog koj tham nrog koj tus kws kho mob. Koj tuaj yeem nug koj tus kws kho mob cov lus nug zoo li no:

• Cov kev kho mob twg zoo tshaj plaws rau kuv?
• Cov kev kho mob no kuv yuav xav tau cov kev mob tshwm sim dab tsi? Kuv yuav ua li cas thiaj daws tau lawv?
• Puas muaj kev sim tshuaj kho mob (kev tshawb fawb txog kev kho mob tshiab) uas kuv tuaj yeem koom nrog?
• Puas yog mob qog noj ntshav rov qab los tom qab kho tas lawm? Yog tias nws rov qab los, yuav ua li cas?
• Kuv yuav tsum hloov dab tsi hauv kuv txoj kev ua neej?

Qhov txawv ntawm Mycosis Fungoides thiab Sézary Syndrome yog dab tsi?

Tej zaum koj twb tau hnov ​​ob lub npe no ua ntej lawm. Mycosis fungoides kuj yog ib hom kab mob T-cell lymphoma uas cuam tshuam rau daim tawv nqaij. Ntawd yog, nws yog tib tsev neeg li Sézary syndrome. Nws kuj ua rau muaj pob liab lossis tsaus ntawm daim tawv nqaij. Txawm li cas los xij, qhov txawv tseem ceeb yog tias, tsis zoo li Sézary syndrome, cov neeg uas muaj mycosis fungoides tsis muaj cov qe ntshav qog nqaij hlav (Sézary cells) ntau hauv lawv cov ntshav. Txawm li cas los xij, qee zaum mycosis fungoides tuaj yeem hnyav thiab loj hlob mus rau hauv Sézary syndrome. Yog li muaj kev sib txuas ntawm ob qho no.

Thaum kawg, tej yam uas yuav tsum nco ntsoov

Kab mob Sézary Syndrome yog ib hom mob qog nqaij hlav uas tsis tshua muaj, loj hlob sai, uas cuam tshuam rau daim tawv nqaij thiab cov ntshav. Nws kuj tseem tuaj yeem kis mus rau lwm qhov ntawm lub cev. Thaum xub thawj, cov tsos mob yuav zoo li mob tawv nqaij xws li eczema, tab sis cov tsos mob ntawm Sézary Syndrome zuj zus sai thiab tsis ploj mus yooj yim. Txawm hais tias tus mob tsis tuaj yeem kho tau tag nrho, cov kws kho mob tuaj yeem kho koj cov tsos mob thiab tiv thaiv tus kab mob kom tsis txhob kis mus rau ntawm daim tawv nqaij thiab cov ntshav mus rau lwm yam kabmob.

Yog li ntawd, yog tias koj pom muaj tej yam txawv txawv, khaus tas li, khaus heev, liab, tsaus nti, lossis tuab ntawm koj daim tawv nqaij, nws yog ib qho tseem ceeb uas yuav tsum mus ntsib kws kho mob, tshwj xeeb yog kws kho mob tawv nqaij, sai li sai tau. Thaum ntxov pom nws , kev kho mob sai dua tuaj yeem pib. Nws tuaj yeem ua qhov sib txawv loj rau koj lub neej.

` Sézary syndrome, mob qog nqaij hlav ntawm daim tawv nqaij, T-cell lymphoma, cov tsos mob ntawm daim tawv nqaij, erythroderma, kev kho mob qog nqaij hlav, khaus ntawm daim tawv nqaij