RESUMO
BACKGROUND: Advanced-stage mycosis fungoides (MF)/Sézary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyze the pattern of care worldwide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA versus non-USA), and whether the heterogeneity of approaches has potential impact on survival. PATIENTS AND METHODS: This study included 853 patients from 21 specialist centers (14 European, 4 USA, 1 each Australian, Brazilian, and Japanese). RESULTS: Heterogeneity of treatment approaches was found, with up to 24 different modalities or combinations used as first-line and 36% of patients receiving four or more treatments. Stage IIB disease was most frequently treated by total-skin-electron-beam radiotherapy, bexarotene and gemcitabine; erythrodermic and SS patients by extracorporeal photochemotherapy, and stage IVA2 by polychemotherapy. Significant differences were found between USA and non-USA centers, with bexarotene, photopheresis and histone deacetylase inhibitors most frequently prescribed for first-line treatment in USA while phototherapy, interferon, chlorambucil and gemcitabine in non-USA centers. These differences did not significantly impact on survival. However, when considering death and therapy change as competing risk events and the impact of first treatment line on both events, both monochemotherapy (SHR = 2.07) and polychemotherapy (SHR = 1.69) showed elevated relative risks. CONCLUSION: This large multicenter retrospective study shows that there exist a large treatment heterogeneity in advanced MF/SS and differences between USA and non-USA centers but these were not related to survival, while our data reveal that chemotherapy as first treatment is associated with a higher risk of death and/or change of therapy and thus other therapeutic options should be preferable as first treatment approach.
Assuntos
Micose Fungoide/terapia , Síndrome de Sézary/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Austrália/epidemiologia , Brasil/epidemiologia , Criança , Europa (Continente)/epidemiologia , Feminino , Humanos , Japão/epidemiologia , Masculino , Oncologia/métodos , Oncologia/estatística & dados numéricos , Pessoa de Meia-Idade , Micose Fungoide/mortalidade , Micose Fungoide/patologia , Estadiamento de Neoplasias , Estudos Retrospectivos , Síndrome de Sézary/mortalidade , Síndrome de Sézary/patologia , Estados Unidos/epidemiologia , Adulto JovemRESUMO
Background: Sezary syndrome (SS) prognostic factors are not well defined because of the rarity of this disease. The specific goal of this prospective study was to asses by multivariate analysis the predictive value with respect to survical of series of clinical, haematological ans immunological parameters taken at SS diagnosis. Patients and methods: A cohort of 62 SS patientsdiagnosed and followed since 1975 was examined, and 51 were included in the multivariate analysis model. Results: The median survical time was 31 months (range: 1 month-15.7+ years), and the five-year survical rate 33.5%. The following variables were found by univariate analysis to be associated with a poor prognosis at the time of SS diagnosis: previous history of mycosis fungoides (P = 0.013), high number of circulating leukocytes (P = 0.001), Sezary cells (SC) (P 1; n = 20) are characterized by an aggressive disase course not modifiable by traditional therapies (five-year survical: 5%
Assuntos
Humanos , Linfócitos T/citologia , Linfócitos T/química , Síndrome de Sézary/imunologia , Síndrome de Sézary/sangue , Linfócitos T/ultraestrutura , PrognósticoRESUMO
Recently, Khayat et al. reported that high-dose recombinent interleukin-2 (rIL-2) i.v. may induce tumour regression in metastatic melanoma patients through an association with cisplatin (CDDP) and alpha-interferon (alpha-IFN). Treatment-related toxicities are, however, important. Previous studies have demonstrated that rIL-2 toxicity may be reduced through a subcutaneous injection. In order to evaluate the effectiveness of low subcutaneous rIL-2 doses in a chemoimmuno-hormonotherapeutic combination, 36 metastatic melanoma patients were treated with CDDP, rIL-2, alpha-IFN and tamoxifen (TAM). The overall response rate was 47.2% five patients had complete response (14%), 12 partial response (33%) and 13 stable disease (36%). Median response duration was 6.4 months (range: 2-29+). Median overal survival was 10 months (range: 3-36+). The CDDP/rIL-2/alpha-IFN/TAM regimen was effective both on soft tissue and visceral metastases. Toxicity was low and patient management did not require an intensive care unit. A statistically significant increase in both percentage and absolute values of lymphocytes, eosinophilis, CD3+/CD4+, CD5+, CD16/56 + and HLA-DR+ cells wasfound in all patients after two treatment courses. This study shows that lower doses of subcutaneous rIL-2, as well as CDDP and alpha-IFN, associated with TAM, may have similar anticancer efficacy with respect to Khayat's schedule but lower toxicity