Mycosis fungoides and Sézary syndrome: Australian clinical practice statement

Bhabha, Friyana K; McCormack, Christopher; Wells, Jillian; Campbell, Belinda A.; Newland, Kate; Lade, Stephen; Buelens, Odette; Joske, David; Shortt, Jake; Mapp, Sally; Radeski, Dejan; Hertzberg, Mark; Khot, Amit; Weyden, Carrie van der; Khoo, Christine; Hawkes, Eliza A; Prince, H. Miles

doi:10.1111/ajd.13467

Cited by 5 publications

(5 citation statements)

References 72 publications

(167 reference statements)

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“…This variation in management choices likely reflects uncertainty regarding the comparative efficacy of available treatments, lack of consensus on optimal treatment sequencing, regional regulatory prescribing restrictions, and individual physician practice. 4 , 10 , 32 , 33 …”

Section: Discussionmentioning

confidence: 99%

“…Management of MF or SS is based on the stage of disease; international and national guidelines list a number of treatment options to be considered first-line, but with neither particular order of preference nor for subsequent therapies to be considered thereafter. 2 , 3 , 4 , 5 , 6 , 7 , 8 …”

Section: Introductionmentioning

confidence: 99%

“… 9 Thus, for the majority of patients, management represents a multiline treatment journey without durable remissions. Because there is no clear consensus on the optimal treatment algorithm, 2 , 3 , 4 , 5 , 6 , 7 individual treatment pathways are highly variable among centers. 10 , 11 To date, there is only 1 randomized clinical trial specifically for relapsed or refractory SS or advanced MF, the MAVORIC study 12 compared mogamulizumab with vorinostat, and in a recently published post hoc analysis, a clear benefit in time-to-next-treatment (TTNT) was observed for patients with B2 blood involvement receiving mogamulizumab (median TTNT, 13.1 vs 3.5 months; P < .0001).…”

Section: Introductionmentioning

confidence: 99%

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International study of treatment efficacy in SS shows superiority of combination therapy and heterogeneity of treatment strategies

Campbell,

Dobos,

Haider

et al. 2023

Blood Advances

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Despite increasing availability of therapies, patients with Sezary syndrome (SS) commonly endure multi-line treatment journeys, mostly with partial responses of short duration. Measuring clinical benefit is challenging; time-to-next-treatment (TTNT) provides a robust, objective measurement of efficacy. This international observational study, from three quaternary centers, examines patterns of clinical care and therapeutic benefit as measured by TTNT. TTNT was calculated for monotherapies and combination therapies, with consideration given to treatment line. 178 patients with SS (73% de novo, 27% secondary) were included, receiving 721 lines of systemic therapy, with median follow-up of 56.9 months. Across all lines, 58 different therapeutic regimens were prescribed (54 were systemic therapies), and classified into 17 treatment groups. First-line, the most common treatments were extracorporeal photopheresis (ECP)-containing combination therapy (20%) and retinoid monotherapy (19%). Median TTNT for all first-line therapies was short (5.4 months). First-line, combination therapies had longer median TTNT than monotherapies: 10.0 vs 5.0 months (p=0.004), respectively. Later delivery of combination therapies was associated with shorter clinical benefit, with median TTNT reduced to 6.2 and 2.2 months for midline (2nd-4th line) and late line (>4th line), respectively (p<0.001). First-line, ECP-containing treatments were associated with longer median TTNT compared to non ECP-containing treatments: 9.0 vs 4.9 months (p=0.007). For both ECP-monotherapy and ECP-containing combination therapy, significant reductions in TTNT were seen in later lines. These data suggest therapeutic benefit from first-line delivery of combination therapy for patients with SS, and favor early inclusion of ECP in the treatment algorithm for those who can access it.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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International study of treatment efficacy in SS shows superiority of combination therapy and heterogeneity of treatment strategies

Campbell,

Dobos,

Haider

et al. 2023

Blood Advances

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“…Chemotherapy drugs have high response rates, however, the duration of response is short, with rapid recurrence. There are no standardized chemotherapy protocols, but most studies have evaluated the efficacy of gemcitabine, chlorambucil, methotrexate, pralatrexate, liposomal doxorubicin, CHOP, and CHOP-like regimens (137,140,142).…”

Section: Treatmentmentioning

confidence: 99%

Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management

Miyashiro

Sanches

2023

Front. Oncol.

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Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS.

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“…Poucos pacientes receberam monoquimioterapia (16,5%) e poliquimioterapia (8,5%), pois apresentam taxas altas de resposta, mas rápida recidiva. Não há protocolos quimioterápicos padronizados, mas a maioria dos estudos avaliaram a eficácia da gencitabina, clorambucila, metotrexato e CHOP (Gilson et al, 2019;Bhabha et al, 2020).…”

Section: Tratamentosunclassified

Micose fungoide e síndrome de Sézary: caracterização demográfica, clínica, histopatológica, imunopatológica, molecular, laboratorial e evolutiva de coorte observada no ambulatório de linfomas

Miyashiro¹

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eterna gratidão. Sempre ao meu lado e me apoiando incondicionalmente nas minhas decisões.A Joyce Lica Hosotani Miyashiro, minha esposa, amiga, companheira, confidente. Presente comigo desde o início, não hesitou em abrir mão de momentos para que eu pudesse concluir esta jornada. Sem seu apoio e sua compreensão, a finalização desta tese não seria possível. Seus valiosos conselhos me fazem uma pessoa melhor. Mesmo grávida do Victor, conseguiu me auxiliar e cuidar de nosso bem maior, que está por vir.A meu irmão Eder Rafael Miyashiro que, apesar de estar longe fisicamente, sempre prestou muito apoio. Sua ajuda nas numerosas dúvidas matemáticas e computacionais que surgiram ao longo do caminho foi essencial.Aos meus sogros, Luzia Rioko Ueda Hosotani e Mario Kiyonori Hosotani (in memorian), que me acolheram em seu lar e em sua família. Assim como sua filha Lica, sempre apoiaram minhas escolhas e compreenderam minhas ausências em prol de meus projetos. AGRADECIMENTOSExpresso meu sentimento de gratidão e admiração ao Professor Doutor José Antonio Sanches Jr. Em 2007 tive a oportunidade de ser acolhido para uma iniciação científica e, desde então, tornou-se meu orientador e meu mentor. O convívio ao longo dos anos de graduação me fez enxergar seu amor pela Dermatologia e pelo cuidado com os pacientes, me influenciando na escolha desta especialidade. Como profissional e como pessoa, me ensina, me estimula, e me faz crescer a cada dia.

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Mycosis fungoides and Sézary syndrome: Australian clinical practice statement

Cited by 5 publications

References 72 publications

International study of treatment efficacy in SS shows superiority of combination therapy and heterogeneity of treatment strategies

International study of treatment efficacy in SS shows superiority of combination therapy and heterogeneity of treatment strategies

Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management

Micose fungoide e síndrome de Sézary: caracterização demográfica, clínica, histopatológica, imunopatológica, molecular, laboratorial e evolutiva de coorte observada no ambulatório de linfomas

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