Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International Consortium.

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.

[Spontaneous cerebellar hematoma. Analysis of 23 cases]. / Hematoma cerebelar espontâneo. Análise de 23 casos.

A series of 23 patients with spontaneous cerebellar hematoma is analysed retrospectively. Several degrees of consciousness impairment, and headache occurred in all; 12 patients presented motor deficits among the more important clinical signs. Arterial hypertension (69.5%) and diabetes (34%) stand out in the pathological background, and their association was frequent (30%). Computerized tomography (CT) disclosing severe ventricular dilatation (69.5%) associated or not with intraventricular presence of blood is considered as an unsatisfactory prognosis sign, and indicative for emergency therapy. Emergency external ventricular draining is the best indication for the majority of these cases. Posterior fossa craniectomy for emptying the hematoma may be the indication in severe cases. Death and morbidity in this pathology are high as yet. However, CT contribution for diagnosis is quicker and better than benefits obtained through other methods. Results observed in the present series are illustrative on this statement, when compared to data of other series diagnosed through other methods.

Hematoma cerebelar espontâneo: análise de 23 casos / Stontaneous cerebellar hematoma: analysis of 23 cases

Uma série de 23 pacientess com hematoma espontâneo de cerebelo é analisada retrospectivamente, mostrando que: todos apresentavam algum grau de diminuiçäo do nível de consciência e cefaléia; 12 apresentavam déficits motores dentre os sinais clínicos mais importantes. Dos antecedentes patológicos destacam-se hipertensäo arterial (69,5%) e diabetes (34%), havendo associaçäo de ambos em 30% dos casos. Dos aspectos da tomografia computadorizada (TC) mais relevantes a dilataçäo ventricular aguda, encontrada em 69,5% dos pacientes, associada ou näo a presença de sangue intraventricular, é considerada sinal de mau prognóstico, indicando por si só terapêutica de urgência, reservando a craniectomia da fossa posterior para esvaziamento do hematoma para casos específicos. A mortalidade e a morbilidade desta patología é alta; porém, pela TC o diagnóstico se tornou mais rápido e eficiente. Os pacientess desta série puderam beneficiar-se deste procedimento, como se verifica quando comparados a pacientess de outras séries, diagnosticadas por outros métodos