Nivolumab en linfoma de Hodgkin recaído/refractario: experiencia en Aragón

PB-111 Introducción: En el linfoma de Hodgkin (LH) las alteraciones genéticas del cromosoma 9p24.1 de la célula de Reed-Sternberg, causan una sobreexpresión del ligando 1 de muerte programada (PDL-1), que conducen a una evasión del sistema inmune y resistencia terapéutica. Para pacientes refractarios primarios o que recaen después de un trasplante autólogo y de tratamiento con Brentuxumab, existen pocas posibilidades. Nivolumab es una opción para conseguir respuesta y poder realizar trasplante alogénico. Presentamos la experiencia de 4 casos clínicos de Aragón. Paciente y métodos: Caso 1: Varón de 69 años diagnosticado de LH celularidad mixta estadio IIIA en Junio de 2005. 1ª línea: ABVDx6 y radioterapia mediastínica con RC. 2ª línea: por recidiva cervical: Ifosfamida, Vinorelbina y Prednisona con RC. 3ª línea: afectación cervical y retoperitoneal: cisplatino, citarabina y dexametasona más radioterapia cervical con RC. 4ª línea: MOPPx4 y Rituximab Gemcitabina con RC. Autotrasplante en Noviembre de 2011 con RC. 5ª línea: por afectación cervical, retroperitoneal, esplénica e iliaco: Brentuximabx4 y por mala respuesta se añade Bendamustinax3 con RC. A los 4 meses recaída agresiva con amplia afectación ósea y esplénica. Se administra Nivolumab. Caso 2: Varón de 32 años con LH esclerosis nodular IIIB en 2015. Tratado en otro país con ABVDx6 con RC y recaída al año. Después BEACOPPx4, GEMOXx4 y DHAPx4 sin resultado. En nuestro país Brentuximabx4 con progresión. Se administra Nivolumab. Caso3: Varón de 56 años con LH esclerosis nodular IVsB, en Septiembre de 2017. 1ª línea: ABVDx3 y AVDx3 con refractariedad y neumonitis por Bleomicina.2ª línea:ESAHPx2 con persistencia y toxicidad. 3ª línea: Brentuximabx4 con RC, consolidando con Brentuximab- Bendamustinax4. Progresión posterior rápida. Se administra Nivolumab. Caso 4:varón de 32 años diagnosticado en Abril 2016 de LH depleción linfocítica IVB (ósea). 1ª línea:ABVDx6 con RC. Recaída Mayo 2018.2ª línea: ESHAPx2 con persistencia.3ª línea: Brentuximabx4 con progresión. Se administra Nivolumab. Resultados: En el caso 1 se consigue RC con ..

Be Free? The European Union's post-Arab Spring Women's Empowerment as Neoliberal Governmentality

This article analyses post-Arab Spring EU initiatives to promote women's empowerment in the Southern Mediterranean region. Inspired by Foucauldian concepts of governmentality, it investigates empowerment as a technology of biopolitics that is central to the European neoliberal model of governance. In contrast to dominant images such as normative power Europe that present the EU as a norm-guided actor promoting political liberation, the article argues that the EU deploys a concept of functional freedom meant to facilitate its vision of economic development. As a consequence, the alleged empowerment of women based on the self-optimisation of individuals and the statistical control of the female population is a form of bio-power. In this regard, empowerment works as a governmental technology of power instead of offering a measure to foster fundamental structural change in Middle Eastern and North African (MENA) societies. The EU therefore fails in presenting and promoting an alternative normative political vision distinct from the incorporation of women into the hierarchy of the existing market society

Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study

Background: People with Huntington's disease (HD) have been observed to have lower rates of cancers. Objective: To investigate the relationship between age of onset of HD, CAG repeat length, and cancer diagnosis. Methods: Data were obtained from the European Huntington's disease network REGISTRY study for 6540 subjects. Population cancer incidence was ascertained from the GLOBOCAN database to obtain standardised incidence ratios of cancers in the REGISTRY subjects. Results: 173/6528 HD REGISTRY subjects had had a cancer diagnosis. The age-standardised incidence rate of all cancers in the REGISTRY HD population was 0.26 (CI 0.22-0.30). Individual cancers showed a lower age-standardised incidence rate compared with the control population with prostate and colorectal cancers showing the lowest rates. There was no effect of CAG length on the likelihood of cancer, but a cancer diagnosis within the last year was associated with a greatly increased rate of HD onset (Hazard Ratio 18.94, p < 0.001). Conclusions: Cancer is less common than expected in the HD population, confirming previous reports. However, this does not appear to be related to CAG length in HTT. A recent diagnosis of cancer increases the risk of HD onset at any age, likely due to increased investigation following a cancer diagnosis

Clinical and genetic characteristics of late-onset Huntington's disease

Background: The frequency of late-onset Huntington's disease (&gt;59 years) is assumed to be low and the clinical course milder. However, previous literature on late-onset disease is scarce and inconclusive. Objective: Our aim is to study clinical characteristics of late-onset compared to common-onset HD patients in a large cohort of HD patients from the Registry database. Methods: Participants with late- and common-onset (30–50 years)were compared for first clinical symptoms, disease progression, CAG repeat size and family history. Participants with a missing CAG repeat size, a repeat size of ≤35 or a UHDRS motor score of ≤5 were excluded. Results: Of 6007 eligible participants, 687 had late-onset (11.4%) and 3216 (53.5%) common-onset HD. Late-onset (n = 577) had significantly more gait and balance problems as first symptom compared to common-onset (n = 2408) (P &lt;.001). Overall motor and cognitive performance (P &lt;.001) were worse, however only disease motor progression was slower (coefficient, −0.58; SE 0.16; P &lt;.001) compared to the common-onset group. Repeat size was significantly lower in the late-onset (n = 40.8; SD 1.6) compared to common-onset (n = 44.4; SD 2.8) (P &lt;.001). Fewer late-onset patients (n = 451) had a positive family history compared to common-onset (n = 2940) (P &lt;.001). Conclusions: Late-onset patients present more frequently with gait and balance problems as first symptom, and disease progression is not milder compared to common-onset HD patients apart from motor progression. The family history is likely to be negative, which might make diagnosing HD more difficult in this population. However, the balance and gait problems might be helpful in diagnosing HD in elderly patients