Nukae nye Sézary ƒe Dɔlélea? Aleke wòkpɔa ŋusẽ ɖe wò ŋutigbalẽ dzii?

Ède dzesii kpɔ be kasia wò ŋutigbalẽ biãna, ʋãna ŋutɔ, eye wòdoa viviti le teƒe aɖewoa? Àsusu be ŋutigbalẽ ƒe dɔléle si bɔ koe wònye, gake le go siwo mebɔ o me la, ate ŋu anye nusi nu sẽ wu. Emae nye nusi ŋu míele nu ƒom tsoe egbea, Sézary Syndrome, si nye kansa si mebɔ o si gblẽa nu le ŋutigbalẽ, ʋu, kple ʋukawo ŋu. Ðewohĩ ŋkɔa atɔtɔ wò vie, gake ele vevie be nànyae.

Nukae nye Sézary ƒe Dɔlélea?

Kpuie ko la, Sézary Syndrome nye ŋutigbalẽ me kansa ƒomevi aɖe si mebɔ kura o eye wòkaka kabakaba. Le atikewɔwɔ me la, ele kansa ƒe ƒuƒoƒo aɖe si woyɔna be cutaneous T-cell lymphoma me. T-lymphocytes, alo T-cells kpuie, nye lãmenugbagbevi vevi aɖe le míaƒe ŋutilã ƒe dɔlélenutsiŋutete me. Le dɔléle sia me la, lãmenugbagbevi ƒomevi aɖe si woyɔna be T-cells va zua kansa eye wòƒoa ƒu ɖe wò ŋutigbalẽ, ʋu, kple ʋukawo me.

Bu eŋu abe asrafo siwo kpɔa míaƒe ŋutilã ta ene, `(T-cells)` siawo. Gake ne asrafo siawo tra mɔ eye wodze dɔwɔwɔ ɖe mía ŋu gɔme la, esiae dzɔna. Esia tae wò ŋutigbalẽ ƒe amadede trɔna eye wòdzena dzĩe ɖo. Ɣeaɖewoɣi la, ŋutigbalẽa hã ate ŋu ado viviti. Ate ŋu adzɔ hã be ʋuʋudedi sesẽ kple abi kple ʋuʋudedi si le veve sem. Nusi me afɔku le wue nye be kansadɔlékui siawo siwo le ʋua me ate ŋu akaka ɖe ŋutilã ƒe akpa bubuwo, si fia be woakaka ɖe ŋutinuwo me. Togbɔ be fifia la, atike blibo aɖeke meli na esia o hã la, mègatsi dzi o , atikewɔmɔnu geɖe li siwo dzi woato aɖu dzesiawo dzi eye woana gbɔdzɔe wò.

Aleke gbegbee nɔnɔme sia bɔe?

Le nyateƒe me la, esia mebɔ kura o. Eyae nye be enye dɔléle si ƒã hafi wokpɔna. Bu eŋu kpɔ, le dukɔ abe Amerika ene gɔ̃ hã me la, ame ɖeka le miliɔn ɖeka me koe wogblɔna be dɔléle sia xɔa ame ƒe sia ƒe. Eyata esia menye nusi wokpɔna zi geɖe ŋutɔ le Sri Lanka hã o. Esia tae wòle vevie be nànya esia, elabena ne dzesiawo dze la, woate ŋu ade dzesi wo kaba.

Nukawoe nye Sézary Syndrome ƒe dzesiwo?

Dɔléle sia ƒe dzesi vevitɔe nye ʋuʋudedi dzĩ si ɖe dzesi (erythroderma) . Esia menye ʋuʋudedi dzro aɖe ko o, eɖea ʋuʋudedi ŋutɔ, vevesese, ɣeaɖewoɣi ŋutigbalẽa ɖea ɖa eye wòkakana kabakaba ŋutɔ. Le ame aɖewo, vevietɔ amesiwo ƒe ŋutigbalẽ le ɣie wu gome la, ate ŋu adze abe teƒe siwo do viviti ene hã (siwo ate ŋu anye aŋutiɖiɖi, aŋutiɖiɖi, alo aŋutiɖiɖi). Le gɔmedzedzea me la, dzesi siawo kpɔkpɔ ate ŋu asesẽ elabena woɖi ŋutigbalẽ ƒe dɔléle siwo bɔ abe eczema alo psoriasis ene. Ame geɖe susuna be esia nye nulɔdɔ bɔbɔe aɖe.

Ne Sézary Syndrome le ŋgɔ yim la, wò ŋutigbalẽ adze ƒoƒoe suesuesue siwo kɔ (papules) gɔme. Teƒe aɖewo ate ŋu ase le wo ɖokui me be yewole kpekpem eye yewoli ke wu bubuwo (plaque). Mlɔeba la, ŋutigbalẽ me kansa (tumors) ate ŋu adze ame dzi. Dzesi ʋɛ bubuwo hã li, siwo ŋu míalé ŋku ɖo:

• Lãmetsiŋusẽ ƒe ʋuka siwo vuvu - le teƒewo abe kɔ, akɔta, kple akɔta ene.
• Asrã - Asrã si vaa ame dzi susu aɖeke manɔmee.
• Ðeɖiteameŋu ɣesiaɣi - Ðeɖiteameŋu ƒe seselelãme eɖanye aleke kee nèdɔ alɔ̃ o.
• Kpekpeme dzi ɖeɖe kpɔtɔ si womeɖe o.
• Taɖa ƒe vuvu - Ɣeaɖewoɣi la, ŋkume kple ŋutilã ƒe fu gɔ̃ hã ate ŋu abu.
• Ectropion (ŋku ƒe ʋuʋudedi si wogbugbɔ trɔ).
• Aklã ƒe lolome (hepatomegaly).
• Ʋuʋudedi (edema), vevietɔ le afɔ siwo le ete.
• Vuvɔ ƒe mɔmaɖemaɖeɖenuŋu - Esia fia be èsena le ɖokuiwò me be vuvɔ le ye ŋu wu ame bubuwo, le xɔme ƒe dzoxɔxɔ si sɔ gɔ̃ hã me.

Nukae hea Sézary Dɔlélea vɛ?

Esia dzɔna elabena lãmenugbagbevi siwo le lãmesẽ me siwo woyɔna be T-cells le míaƒe ŋutilã me la toa domenyiŋusẽfianu ƒe tɔtrɔ me, si wɔnɛ be wozua kansa lãmenugbagbevi siwo mesɔ o. Lãmenugbagbevi siawo siwo mesɔ o la mãna kabakaba eye wodzina ɖe edzi le mɔ si dzi womate ŋu aɖu o nu, eye mlɔeba wokakana ɖe ŋutigbalẽ, ʋukawo, kple ʋu me. Ele abe gbe wuwlui ene, eye wòkaka ɖe afisiafi dzi ɖuɖu manɔmee. Gake eŋutinunyalawo menya nusi tututu hea domenyiŋusẽfianu ƒe tɔtrɔ siawo vɛ haɖe o. Ema fia be esesẽ be míagblɔ nusitae tututu.

Nukawoe nye afɔku siwo ate ŋu ahe esia vɛ?

Togbɔ be ame sia ame ate ŋu axɔ dɔléle sia hã la, nu aɖewo ate ŋu ana wò afɔkua nadzi ɖe edzi. Le kpɔɖeŋu me, ne amegbetɔ ƒe T-cell leukemia virus (HTLV) xɔ wò la, wosusu be afɔku si le Sézary ƒe dɔlélea xɔxɔ me dzi ɖe edzi vie. Ke hã, ele vevie be míaɖo ŋku edzi be menye amesiame si ŋu dɔlékui sia le lae axɔ dɔlélea o.

Nukawoe nye kuxi siwo ate ŋu ado tso dɔléle sia me?

Sézary Syndrome nye dɔléle si ate ŋu akpɔ ŋusẽ ɖe agbenɔnɔ ƒe nyonyome dzi vevie. Bu eŋu kpɔ, aleke nàte ŋu ase le ɖokuiwò me be yeƒe lãme sẽ ne èle ŋutigbalẽ ƒom ɣesiaɣi eye nèle veve sem? Ate ŋu ahe nuwo abe alɔ̃madɔmadɔ, dzimaɖitsitsi ɣesiaɣi, kple blanuiléle vɛ. Kuxi bubu geɖewo hã li:

• Kansa ƒe T-lãmenugbagbeviwo kaka ɖe wò lãkusi, aklã, dɔgbo, kple ƒumeŋuɖui me.
• Afɔku si dzina ɖe edzi be woaxɔ ʋumenugbagbeviɣidɔ (ʋumenugbagbeviɣidɔ ƒe kansa) ƒomevi bubu.
• Dɔlékuiwo xɔxɔ enuenu ƒe afɔku dzina ɖe edzi elabena ŋutigbalẽa ƒe ametakpɔnu gblẽ, si wɔnɛ be dɔlékuiwo gena ɖe eme bɔbɔe.

Aleke ɖɔktawo wɔa dea dzesi dɔléle siae?

Ne èyi ɖɔkta gbɔ la, awɔ ŋutilãmedɔ gbã eye wòabia wò dzesiwo. Abia nya tso wò atikewɔwɔ ŋutinya si va yi hã ŋu. Ðɔktaa awɔe nenemaNe wosusu be Sézary Syndrome le eŋu la, ate ŋu awɔ dodokpɔ geɖe, abe:

• Ʋudodokpɔ: Esia le vevie ŋutɔ. Esia ateŋu alɔ ``Ʋu Xexlẽme Blibo (CBC)`` (esia léa ŋku ɖe lãmenugbagbevi ƒomevi vovovowo ƒe alafa memamã si le ʋua me ŋu, vevietɔ ʋumenugbagbevi ɣiwo), ``Sézary ʋu ƒe xexlẽme`` (si léa ŋku ɖe ``Sézary lãmenugbagbeviwo`` (kansa lãmenugbagbevi siwo bɔ ɖe dɔléle sia me) ƒe xexlẽme si le ʋua me ŋu), kple ``ʋu si le gota ƒe ʋuʋu`` (ʋu ƒe kpɔɖeŋu ƒe dodokpɔ to nusuekpɔmɔ̃ dzi).
• Ŋutilã, ʋuka, alo ƒumeŋuɖui ƒe lãmenugbagbeviwo ɖeɖe: Le esia me la, woɖea lãkusi sue aɖe tso ŋutigbalẽa ƒe akpa si dɔlélea le alo ƒumeŋuɖui aɖe me heɖonɛ ɖe nudokpɔƒe. Le afima la, atikeŋutinunyala aɖe tsɔa nusuekpɔmɔ̃ léa lãmenugbagbeviawo ƒe kpɔɖeŋua kpɔna le afima. Esia ate ŋu adi nuwo abe dɔdzẽ ƒe dzesiwo kple tɔtrɔ siwo vaa lãmenugbagbeviwo me ene.
• Dodokpɔwo be woakpɔe ɖa be kansa la kaka hã: Ne ʋu kple lãmenugbagbeviwo ƒe dodokpɔwo ɖo kpe edzi be Sézary ƒe dɔlélea le ŋuwò la, woawɔ dodokpɔwo atsɔ akpɔe ɖa be kansa la kaka ɖe ŋutilã ƒe akpa bubuwo hã. Esiawo ate ŋu anye akɔta ƒe X-ray, CT scan (kɔmpiuta dzi tomography), kple Positron Emission Tomography (PET) scan (si léa ŋku ɖe lãmenugbagbeviwo ƒe dɔwɔwɔ le ŋutilã me ŋu).

Nukawoe nye Sézary Syndrome ƒe atikewo?

Wò ɖɔkta aŋlɔ atike na wò le wò dzesiwo kple afisi wò kansa le (afisi wòkakae) nu. Atike vovovowo li na esia. Menye amesiamee xɔa atike ɖekae o.

• Fotodamɔnu: Esia zãa kekeli ƒomevi tɔxɛwo tsɔ tsrɔ̃a kansadɔlékui siwo le ŋutigbalẽa me. Woyɔa mɔnu ɖeka be extracorporeal photopheresis. Le esia me la, woɖea ʋumenugbagbevi ɣiwo ɖa le wò ʋu me (leukapheresis), wotsɔa atike aɖe da dɔ na wo eye wotsɔa wo dea kekeli si woyɔna be ultraviolet (UV light) me, eye emegbe wogatrɔna vaa wò ŋutilã me. Alo woate ŋu ana UV keklẽŋusẽdɔdamɔnu na ŋutigbalẽa tẽ.
• Kekeli ƒe dɔdamɔnu: Esia bia be woazã keklẽŋusẽ si me ŋusẽ geɖe le atsɔ atsrɔ̃ kansa lãmenugbagbeviwo. Woate ŋu awɔ esia to gotagome keklẽŋusẽdɔdamɔnu (zã mɔ̃ aɖe tsɔ naa keklẽawo) alo ŋutigbalẽ bliboa ƒe elektrɔnik keklẽŋusẽdɔdamɔnu (total skin electron beam radiation therapy) zazã me.
• Atikewo zazã: Esia bia be woana atike siwo wua kansadɔlékuiwo. Wo dometɔ aɖewo li siwo wozãna ɖe ŋutigbalẽa ŋu (atike si wozãna ɖe ŋutigbalẽ ŋu) kple esiwo gblẽa nu le ŋutilã bliboa ŋu (atikewɔwɔ si wozãna ɖe ɖoɖo nu) (wodoa wo abe atikekuiwo ene alo abe atikekui ene). Vorinostat (Zolinza®) nye atike si wotsɔna naa ŋutilã bliboa ƒe kpɔɖeŋu.
• Atikewɔwɔ si woɖo taɖodzinu na: Dɔdamɔnu siawo dzea taɖodzinu tɔxɛ siwo wokpɔna le kansa lãmenugbagbeviwo dzi ko dzi. Monoclonal antibody therapy nye atikewɔwɔ mawo dometɔ ɖeka si woɖo taɖodzinu na si wozãna na Sézary ƒe dɔlélea.
• Dɔlélenutsiŋutete ƒe dɔdamɔnu: Esia bia be nàʋã wò ŋutilã ŋutɔ ƒe dɔlélenutsiŋutete be wòawɔ avu kple kansa lãmenugbagbeviwo. Woate ŋu awɔ esia to atikewo abe Interferon (Intron A®, Intron A Multidose Pen.®)) zazã me.
• Atike bubuwo ƒe atikewɔwɔ: Wozãa corticosteroids (atike siwo nye steroid ƒomevi) kple retinoid ƒomevi ƒe krem ​​alo gel (e.g. bexarotene (Targretin®)) hã tsɔ léa ŋutigbalẽ ƒe dzesiwo nu.

"Gake ɖo ŋku edzi be menye atikewɔwɔ siawo katãe sɔ na amesiame o. Wò ɖɔktae atso nya me le atike nyuitɔ kekeake si nàzã na wò nɔnɔmea ŋu. Aɖe nusianu me na wò."

Nukawoe nye atikewɔwɔa ƒe nugbegblẽ le ame ŋu?

Kansa dada akpa gãtɔ ƒe nugbegblẽ le ame ŋu aɖewo. Esia sɔ. Ele vevie be nànya wo do ŋgɔ. Le goawo katã me la, `(chemotherapy)` kple `(radiation therapy)` àte ŋu akpɔ mɔ na nuwo abe ɖeɖiteameŋu, ʋuʋudedi, ʋuʋudedi, nuɖuɖu ƒe dzodzro, kple ɖa ƒe vuvu. `(Fotodada)` ateŋu akpɔ ŋusẽ ɖe ŋutigbalẽ dzi - ɣeaɖewoɣi ŋutigbalẽ ƒuƒu, biã. `(Targeted therapy)`, `(immunomodulators)` (atike siwo trɔa dɔlélenutsiŋutete) kple `(immunotherapy)` ateŋu ahe ʋuʋudedi, aɖuɖɔtoe, ɖeɖiteameŋu, kple tɔtrɔ le ʋu ƒe akpa aɖewo me `(ʋu ƒe xexlẽme si mesɔ o)` vɛ ɣeaɖewoɣi. Anyo be nàƒo nu tso esia ŋu kple wò ɖɔkta eye nànyae do ŋgɔ. To mɔ sia dzi la, ne nane dzɔ la, àte ŋu aka ɖe edzi be yematsi dzodzodzoe o.

Ðe woate ŋu ada esia keŋkeŋa?

Esiae nye kuxi gãtɔ kekeake na ame geɖe. Nublanuitɔe la, atike aɖeke meli na Sézary Syndrome o. Gake ema mefia be nàɖe asi le mɔkpɔkpɔ ŋu o . Atikewɔwɔ geɖe li siwo ate ŋu akpe ɖe ame ŋu wòaɖu dzesiawo dzi, axe mɔ na lãmenugbagbevi siwo mesɔ o la be woagamã ahatsi o, eye woana kansa nagakaka blewu o. Dɔdamɔnu siawo ate ŋu ana wò agbenɔnɔ nanyo ɖe edzi.

Ɣeyiɣi didi kae dɔléle sia nɔa anyi?

Sézary Syndrome nye dɔléle si nɔa anyi didi. Esia fia be menye dɔléle si vaa ame gbɔ eye wòdzona o. Ðewohĩ ahiã be woawɔ atike na wò le mɔ aɖe nu eye woawɔ atike na wò le wò agbemeŋkekewo katã me.

Aleke mɔnukpɔkpɔ siwo li be woatsi agbe la le?

Esia nye nya vevi aɖe, eye mekpɔa ŋusẽ ɖe amesiame dzi le mɔ ɖeka nu o. Le nyatakaka siwo li fifia nu la, amesiwo ŋu Sézary Syndrome le la dometɔ siwo ade 24% nɔa agbe ƒe atɔ̃ ya teti le dɔlélea megbe. Gake ɖo ŋku edzi be akɔntabubu si wotu ɖe ame bubuwo ƒe nuteƒekpɔkpɔwo dzi koe nye esia. Amesiame to vovo. Nɔnɔme sia ate ŋu anyo ɖe edzi ne atikewɔmɔnu yeye siwo wɔa dɔ nyuie wu va li. Esi wònye be Sézary Syndrome nye dɔléle si mebɔ o ta la, esesẽna be woaƒo nyatakaka siwo sɔ nu ƒu. Ne nya aɖe le asiwò ku ɖe wò nɔnɔmea ŋu la, anyo wu be nàbia wò ɖɔkta. Ate ŋu aɖe nyateƒenya siwo ku ɖe wò nɔnɔmea ŋu me.

Aleke malé be na ɖokuinye?

Sézary Syndrome ƒe agbenɔnɔ fia be ele be nàlé be na ɖokuiwò etɔxɛe esime nèle atike wɔm na wò le wò agbemeŋkekewo katã me. Ðewohĩ ahiã be nàɖe afɔ bubuwo atsɔ akpɔ wò ŋutigbalẽ ta.

• Ele vevie ŋutɔ be nàƒo asa na ɣe ƒe keklẽ tẽ . Dɔdamɔnu aɖewo ate ŋu ana ŋutigbalẽa nase veve ɖe ɣe ƒe keklẽ nu.
• Wɔ ɖe ɖɔkta ƒe mɔfiamewo dzi pɛpɛpɛ. No wò atike ɖe game dzi.
• Na wò ŋutigbalẽ nanɔ dzadzɛ eye nàzã tsilénu (ne èbia ɖɔkta vɔ).
• Ƒo nuɖuɖu si naa lãmesẽ.
• Dze agbagba nànɔ dzidzɔ kpɔm le susu me alesi nàte ŋui.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Sézary Syndrome ƒe dzesiwo ate ŋu atrɔ eye woagblẽ ɖe edzi kabakaba. Eyata nya tɔtrɔ ɖesiaɖe si ava wò ŋutilã me. Ne ède dzesi nusiwo gbɔna dometɔ aɖe la, gblɔe na wò ɖɔkta enumake:

• Ne ède dzesi tɔtrɔwo le wò ŋutigbalẽ me - dzodzro alo viviti kaka ɖe wò ŋutigbalẽ ƒe akpa geɖe, ʋuʋudedi suewo le lolom, alo ʋuʋudedi yeyewo dodo - esiawo nye dzesi siwo fia be wò nɔnɔmea ate ŋu anɔ gbegblẽm ɖe edzi.
• Ne èxɔ dɔlékuiwo enuenu eye woxɔa ɣeyiɣi didi hafi wohayana. Ne dzoxɔxɔ kple ŋutilã le veve sem enuenu.

Nya kawoe wòle be nàbia ɖɔktaa?

Ne Sézary Syndrome le ŋuwò la, biabia geɖe ate ŋu anɔ asiwò. Aɖe vi ne èdzro wo me kple wò ɖɔkta. Àte ŋu abia wò ɖɔkta nya siawo abe esiawo ene:

• Dɔdamɔnu kawoe nyo nam wu?
• Nu gbegblẽ kawoe mate ŋu akpɔ mɔ na tso atikewɔwɔ siawo me? Aleke mawɔ akpɔ wo gbɔe?
• Ðe dɔdamɔnu dodokpɔ aɖewo li (numekuku le atikewɔwɔ yeyewo ŋu) siwo me mate ŋu akpɔ gome lea?
• Ðe kansa ate ŋu atrɔ ava le atikewɔwɔ vɔ megbea? Ke ne ewɔe ya ɖe?
• Tɔtrɔ kawoe wòle be mawɔ le nye agbenɔnɔ me?

Vovototo kae le Mycosis Fungoides kple Sézary Syndrome dome?

Ðewohĩ èse ŋkɔ eve siawo kpɔ. Mycosis fungoides hã nye ŋutigbalẽ me T-cell lymphoma ƒomevi aɖe si gblẽa nu le ŋutigbalẽa ŋu. Eyae nye be ele ƒome ɖeka me kple Sézary ƒe dɔlélea. Enaa ɖa biã alo do viviti hã nɔa ŋutigbalẽa ŋu. Gake vovototo vevitɔe nye be, to vovo na Sézary ƒe dɔlélea la, kansa lãmenugbagbeviwo (Sézary lãmenugbagbeviwo) ƒe agbɔsɔsɔ gã aɖeke mele amesiwo ŋu mycosis fungoides le siwo le tsatsam le woƒe ʋu me o. Gake ɣeaɖewoɣi la, mycosis fungoides ate ŋu asẽ eye wòava zu Sézary ƒe dɔléle. Eyata kadodo aɖe le wo ame evea dome.

Mlɔeba la, nu siwo dzi wòle be woaɖo ŋkui

Sézary Syndrome nye kansa si mebɔ o, si tsina kabakaba, si gblẽa nu le ŋutigbalẽ kple ʋu ŋu. Ate ŋu akaka ɖe ŋutilã ƒe akpa bubuwo hã. Gbã la, dzesiawo ate ŋu adze abe ŋutigbalẽ ƒe dɔléle si bɔ abe eczema ene, gake Sézary Syndrome ƒe dzesiwo nu sẽna ɖe edzi kabakaba eye womedzona bɔbɔe o. Togbɔ be womate ŋu ada dɔlélea keŋkeŋ o hã la, ɖɔktawo ate ŋu ada wò dzesiwo eye woaxe mɔ na dɔlélea be wòagakaka tso ŋutigbalẽ kple ʋu me ayi ŋutinu bubuwo me o.

Eyata ne ède dzesii be yeƒe ŋutigbalẽ le ƒoƒom si mebɔ o, si nɔa anyi ɖaa, si nu sẽ, biã, teƒe siwo do viviti, alo le ƒoƒom la, ele vevie be nàkpɔ ɖɔkta, vevietɔ ŋutigbalẽŋutinunyala, kaba ale si nàte ŋui. Zi alesi wokpɔe kaba la , zi nenemae woate ŋu adze atikewɔwɔ gɔme kaba. Ate ŋu ana tɔtrɔ gã aɖe nava wò agbenɔnɔ me.

` Sézary ƒe dɔléle, ŋutigbalẽ me kansa, T-cell lymphoma, ŋutigbalẽ ƒe dzesiwo, erythroderma, kansa dada, ŋutigbalẽ ƒe ʋuʋu