Sézary Syndrome: Matšoao le tebello ea bophelo

Litaba

• Matšoao le matšoao ke afe?
• Setšoantšo sa erythroderma
• Ke mang ea kotsing?
• E bakoa ke eng?
• E fumanoa joang?
• Sézary syndrome e etsoa joang?
• E alafshoa joang?
• Psoralen le UVA (PUVA)
• Extracorporeal photochemotherapy / photopheresis (ECP)
• Phekolo ea radiation
• Chemotherapy
• Immunotherapy (phekolo ea biologic)
• Liteko tsa bongaka
• Ponahalo

Sézary syndrome ke eng?

Sézary syndrome ke mofuta oa T-cell lymphoma. Lisele tsa Sézary ke mofuta o itseng oa sele e tšoeu ea mali. Boemong bona, lisele tse nang le mofetše li ka fumanoa maling, letlalo le li-lymph node. Kankere e ka fetela le lithong tse ling.

Sézary syndrome ha e tloaelehe haholo, empa e etsa liphesente tse 3 ho isa ho tse 5 tsa li-lymphomas tsa letlalo tse sehiloeng. U kanna oa utloa e bitsoa Sézary erythroderma kapa Sézary's lymphoma.

Matšoao le matšoao ke afe?

Letšoao le tsebahalang la Sézary syndrome ke erythroderma, lekhopho le lefubelu, le hlohlonang leo qetellong le ka koahelang karolo ea 80 lekholong ea 'mele. Matšoao a mang a kenyelletsa:

• ho ruruha hoa letlalo
• matlapa a letlalo le lihlahala
• li-lymph node tse atolositsoeng
• botenya ba letlalo liatleng le bohatong
• ho se tloaelehang hoa manala le menoana ea menoana
• dintshi tse tlase tse hlahellang kantle
• ho lahleheloa ke moriri
• bothata ba ho laola mocheso oa 'mele

Sézary syndrome le eona e ka baka spleen e holileng kapa mathata ka matšoafo, sebete le pampiri ea meno. Ho ba le mofuta ona o matla oa mofets'e ho eketsa menyetla ea ho ba le mofetše o mong.

Setšoantšo sa erythroderma

Ke mang ea kotsing?

Mang kapa mang a ka ba le Sézary syndrome, empa ho ka etsahala hore e ame batho ba ka holimo ho lilemo tse 60.

E bakoa ke eng?

Lebaka le tobileng ha le hlake. Empa batho ba bangata ba nang le Sézary syndrome ba na le ho se ts'oane ha chromosomal ho DNA ea lisele tse nang le mofetše, empa eseng lisele tse phetseng hantle. Tsena ha se liphoso tse futsitsoeng, empa liphetoho tse etsahalang nakong ea bophelo bohle.

Lintho tse sa tloaelehang haholo ke tahlehelo ea DNA ho tsoa ho li-chromosome tsa 10 le 17 kapa litlatsetso tsa DNA ho li-chromosome tsa 8 le 17. Leha ho le joalo, ha ho na bonnete ba hore lintho tse sa tloaelehang tsena li baka mofets'e.

E fumanoa joang?

Ho hlahloba letlalo la hao ho ka hlokomelisa ngaka ka monyetla oa lefu la Sézary. Ho etsa tlhahlobo ea ts'oaetso ho ka kenyelletsa liteko tsa mali ho supa matšoao (li-antigen) ka holim'a lisele tsa mali.

Joalo ka mefuta e meng ea mofetše, biopsy ke eona tsela e molemohali ea ho fihlella tlhahlobo. Bakeng sa biopsy, ngaka e tla nka sampole e nyane ea lisele tsa letlalo. Ngaka ea mafu e tla hlahloba sampole ka tlas'a microscope ho batla lisele tsa mofetše.

Lymph nodes le moko oa masapo le tsona li ka ba biopsied. Ho etsa liteko tsa ho etsa litšoantšo, joalo ka litlhahlobo tsa CT, MRI, kapa PET, ho ka thusa ho tseba hore na mofetše o nametse li-lymph node kapa litho tse ling.

Sézary syndrome e etsoa joang?

Staging e bolela hore na mofetše o hasane hakae le hore na mekhoa e metle ea kalafo ke efe.Sézary syndrome e hlophisitsoe ka tsela e latelang:

• 1A: Letlalo le ka tlase ho liperesente tse 10 le koahetsoe ke mabala a khubelu kapa lipoleiti.
• 1B: Karolo e fetang 10 lekholong ea letlalo e khubelu.
• 2A: Letlalo lefe kapa lefe lea ameha. Lymph node li holisitsoe, empa ha li na mofetše.
• 2B: Hlahala e le 'ngoe kapa ho feta e kholo ho feta sentimita e le' ngoe letlalong. Lymph node li holisitsoe, empa ha li na mofetše.
• 3A: Boholo ba letlalo le khubelu 'me le kanna la ba le lihlahala, lipoleiti kapa matheba. Lymph node li tloaelehile kapa lia holisoa, empa ha li na mofetše. Mali a ka ba le lisele tsa Sézary tse seng kae kapa che.
• 3B: Ho na le liso ka holim'a letlalo. Lymph node e kanna ea holisoa kapa ea se ke ea holisoa. Palo ea lisele tsa Sézary maling e tlase.
• 4A (1): Liso tsa letlalo li koahela karolo efe kapa efe ea letlalo. Lymph node e kanna ea holisoa kapa ea se ke ea holisoa. Palo ea lisele tsa Sézary maling e phahame.
• 4A (2): Liso tsa letlalo li koahela karolo efe kapa efe ea letlalo. Ho na le li-lymph node tse atolositsoeng mme lisele li shebahala li sa tloaeleha haholo tlasa tlhahlobo ea microscopic. Lisele tsa Sézary li kanna tsa ba maling kapa ha li eo.
• 4B: Liso tsa letlalo li koahela karolo efe kapa efe ea letlalo. Li-lymph node li ka ba tse tloaelehileng kapa tse sa tloaelehang. Lisele tsa Sézary li kanna tsa ba maling kapa ha li eo. Lisele tsa lymphoma li hasane ho litho tse ling kapa lisele.

E alafshoa joang?

Ho na le lintlha tse 'maloa tse susumetsang hore na ke kalafo efe e ka u tsoelang molemo. Tse ling tsa tsona ke:

• sethaleng sa ho hlahlojoa
• lilemo
• mathata a mang a bophelo

Tse latelang ke tse ling tsa kalafo ea Sézary syndrome.

Psoralen le UVA (PUVA)

Moriana o bitsoang psoralen, o atisang ho bokella liseleng tsa mofetše, o keneloa ka mothapong. E qala ho buloa ha e pepesetsoa leseli la ultraviolet A (UVA) le lebisitsoeng letlalong la hao. Ts'ebetso ena e senya lisele tsa mofetše ka tšenyo e nyane feela ho lisele tse phetseng hantle.

Extracorporeal photochemotherapy / photopheresis (ECP)

Kamora ho fumana lithethefatsi tse khethehileng, lisele tse ling tsa mali lia ntšoa 'meleng oa hau. Li alafshoa ka leseli la UVA pele li khutlisetsoa 'meleng oa hau.

Phekolo ea radiation

Ho sebelisoa X-rays e matla ho senya lisele tsa mofetše. Mahlaseling a ka ntle a sefate, mochini o romela mahlaseli libakeng tse lebisitsoeng 'meleng oa hau. Phekolo ea radiation e ka kokobetsa bohloko le matšoao a mang hape. Phekolo ea radiation ea radiation ea letlalo (TSEB) e sebelisa mochini o ka ntle oa radiation ho lebisa lielektrone letlalong la 'mele oa hau kaofela.

U ka ba le kalafo ea radiation ea UVA le ultraviolet B (UVB) u sebelisa leseli le ikhethang le lebisitsoeng letlalong la hao.

Chemotherapy

Chemotherapy ke kalafo e hlophisehileng eo ho eona ho sebelisoang lithethefatsi tse matla ho bolaea lisele tsa mofets'e kapa ho emisa karohano ea tsona. Lithethefatsi tse ling tsa chemotherapy li fumaneha ka lipilisi, 'me tse ling li lokela ho fuoa methapo.

Immunotherapy (phekolo ea biologic)

Lithethefatsi tse kang li-interferon li sebelisetsoa ho susumetsa sesole sa hau sa 'mele ho loantša mofetše.

Lithethefatsi tse sebelisetsoang ho phekola lefu la Sézary li kenyelletsa:

• alemtuzumab (Campath), lesole la 'mele le monoclonal
• bexarotene (Targretin), retinoid
• brentuximab vedotin (Adcetris), khokahano ea anti-anti-drug
• chlorambucil (Leukeran), sethethefatsi sa chemotherapy
• corticosteroids ho kokobetsa matšoao a letlalo
• cyclophosphamide (Cytoxan), sethethefatsi sa chemotherapy
• denileukin difitox (Ontak), phetoho ea karabelo ea biologic
• gemcitabine (Gemzar), chemotherapy ea antimetabolite
• interferon alfa kapa interleukin-2, tse hlasimollang 'mele
• lenalidomide (Revlimid), angiogenesis inhibitor
• liposomal doxorubicin (Doxil), sethethefatsi sa chemotherapy
• methotrexate (Trexall), chemotherapy ea antimetabolite
• pentostatin (Nipent), chemotherapy ea antimetabolite
• romidepsin (Istodax), histone deacetylase inhibitor
• vorinostat (Zolinza), histone deacetylase inhibitor

Ngaka ea hau e ka 'na ea u fa meriana kapa lithethefatsi hammoho le mekhoa e meng ea phekolo. Sena se tla ipapisa le sethala sa mofetše le hore na o arabela joang kalafong e itseng.

Kalafo ea sethala sa 1 le 2 e kanna ea kenyelletsa:

• lihlooho tsa lihlooho tsa corticosteroids
• li-retinoids, lenalidomide, histone deacetylase inhibitors
• PUVA
• radiation le TSEB kapa UVB
• biologic therapy ka boeona kapa ka kalafo ea letlalo
• chemotherapy ea lihlooho
• systemotherapy chemotherapy, mohlomong e kopantsoe le kalafo ea letlalo

Mekhahlelo ea 3 le ea 4 e ka phekoloa ka:

• lihlooho tsa lihlooho tsa corticosteroids
• lenalidomide, bexarotene, histone deacetylase inhibitors
• PUVA
• ECP feela kapa le TSEB
• radiation e nang le radiation ea TSEB kapa UVB le UVA
• biologic therapy ka boeona kapa ka kalafo ea letlalo
• chemotherapy ea lihlooho
• systemic chemotherapy, mohlomong e kopantsoe le kalafo ea letlalo

Haeba kalafo e se e sa sebetse, ho ka kenyelletsa khetho ea "cell cell".

Liteko tsa bongaka

Lipatlisiso mabapi le kalafo ea mofets'e li ntse li tsoela pele, 'me liteko tsa bongaka ke karolo ea ts'ebetso eo. Tekong ea bongaka, o ka ba le phihlello ea liphekolo tse bohloko tse sa fumaneheng kae kapa kae. Bakeng sa tlhaiso-leseling e batsi ka liteko tsa bongaka, botsa oncologist ea hau kapa etela ClinicalTrials.gov.

Ponahalo

Sézary syndrome ke mofetše o mabifi haholo. Ka kalafo, o ka khona ho fokotsa tsoelo-pele ea mafu kapa oa ba oa tšoareloa. Empa sesole sa 'mele se fokolang se ka u beha kotsing ea tšoaetso e nkang monyetla le mefuta e meng ea mofetše.

Karolelano ea pholoho e bile lilemo tse 2 ho isa ho tse 4, empa sekhahla sena sea ntlafala ka kalafo e ncha.

Bona ngaka ea hau 'me u qale kalafo kapele kamoo ho ka khonehang ho netefatsa pono e ntle ka ho fetisisa.