Sezary Syndrome presenting with leonine facies and treated with low-dose subcutaneous alemtuzumab

Abstract Cutaneous T-cell lymphomas (CTCL) comprise a group of diseases characterized by the accumulation of malignant T cells within the skin. Sezary syndrome represents an aggressive form of CTCL, in which the skin is diffusely affected and the peripheral blood is involved. It is characterized by the triad of generalized erythroderma, lymphadenopathy, and neoplastic T cells (Sezary cells) in the skin, lymph nodes, and peripheral blood. Leonine facies is rare and corresponds to the morphologic manifestation of diffuse dermal infiltration of the face. It can occur in cutaneous T-cell lymphomas that progress during years without therapy. We present the case of a 54-year-old man with Sezary syndrome presenting with leonine facies, unresponsive to conventional therapies; he exhibited a promising response to subcutaneous low-dose alemtuzumab. A 54-year-old male was observed in our department because of a four-year history of generalized pruritus and cutaneous lesions on the face. On physical examination he showed generalized erythroderma, lichenification, diffuse alopecia, madarosis, and diffuse infiltration of facial skin, particularly on the eyebrows and ears, giving the appearance of a leonine facies The full blood count revealed 18.59x10 9 /L leukocytes, 7.81 x10 9 /L neutrophils, 1.39 x10 9 /L lymphocytes, hemoglobin 15.2 g/dL, and platelets 395 x10 9 /L, with 45.5 percent of atypical lymphocytes. Biochemistry studies were relevant for high LDH (430 UI/L) and high β2-microglobulin (2341 ng/mL) levels. Thoracic, abdominal and pelvic CT scans were unremarkable and the bone marrow biopsy revealed invasion of atypical lymphocytic cells. Flow cytometry and molecular biology studies confirmed the presence of the phenotypically abnormal monoclonal CD4+ T cells in the peripheral blood and bone marrow. The diagnosis of Sezary syndrome (SS) (T4NxM0B2) was made. The patient started combined systemic chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) accomplishing four cycles with no response. A regimen of low dose subcutaneous (sc) alemtuzumab was then introduced, beginning with 3 mg in the first day, followed by 10 mg three times a week for twelve weeks. There was a transient interruption of the treatment at the fourth week, with a duration of six weeks, for asymptomatic reactivation of cytomegalovirus. We observed a marked reduction o

Central Iliac Arteriovenous Anastomosis for Hypertension: Targeting Mechanical Aspects of the Circulation

Dr. Lobo reports personal fees from ROX Medical, St Jude Medical, and Cardiosonic, and grants from Medtronic. Dr. Stanton reports that ROX Medical funded the ROX CONTROL HTN study. Dr. Sobotka reports personal fees and equity from ROX Medical; equity in Cibiem, Ablative Solutions, and Rainbow Medical; and personal fees from Abbott Ventures and Boston Scientific

Mexican World Heritage information on the web: Institutional presence and visibility

This study offers a global overview of the presence and visibility of web information on UNESCO World Heritage located in Mexico, via the analysis of official websites and Web 2.0 information. Cultural heritage is a determining factor in linking people to their history, and contributes to increasing cultural tourism and economic development. The study starts from the hypothesis that the design of these has an influence on the dissemination and popularity of the aforementioned heritage. The relationships between the administrative organization of the country and Internet protocols are compared. A webometric study of the official Mexican websites was carried out. An evaluation sheet was designed to allow the assessment of aspects relating to identification, presence, accessibility and content. The multilingual nature of this information and its presence on social networks and Wikipedia was analysed. The analysis of URLs confirms that the domain .mx is used in 84% of cases. The results indicate a noticeable use of Web 2.0 dissemination of the heritage assets on YouTube (51%) and Facebook (40%), followed by 23% on Twitter. The Web Accessibility Initiative (WAI) guidelines are not yet frequently applied. Finally, the results obtained make it possible to identify variables that can contribute to improvements in the visibility and dissemination of official web information.This paper was supported by the RD & I Project, HAR2012-38562 (Spanish Ministry of Economy and Competitiveness)

Health-state utilities in a prisoner population : a cross-sectional survey

Background: Health-state utilities for prisoners have not been described. Methods: We used data from a 1996 cross-sectional survey of Australian prisoners (n = 734). Respondent-level SF-36 data was transformed into utility scores by both the SF-6D and Nichol's method. Socio-demographic and clinical predictors of SF-6D utility were assessed in univariate analyses and a multivariate general linear model. Results: The overall mean SF-6D utility was 0.725 (SD 0.119). When subdivided by various medical conditions, prisoner SF-6D utilities ranged from 0.620 for angina to 0.764 for those with none/mild depressive symptoms. Utilities derived by the Nichol's method were higher than SF-6D scores, often by more than 0.1. In multivariate analysis, significant independent predictors of worse utility included female gender, increasing age, increasing number of comorbidities and more severe depressive symptoms. Conclusion: The utilities presented may prove useful for future economic and decision models evaluating prison-based health programs

Genetic variation in the Solanaceae fruit bearing species lulo and tree tomato revealed by Conserved Ortholog (COSII) markers

The Lulo or naranjilla (Solanum quitoense Lam.) and the tree tomato or tamarillo (Solanum betaceum Cav. Sendt.) are both Andean tropical fruit species with high nutritional value and the potential for becoming premium products in local and export markets. Herein, we present a report on the genetic characterization of 62 accessions of lulos (n = 32) and tree tomatoes (n = 30) through the use of PCR-based markers developed from single-copy conserved orthologous genes (COSII) in other Solanaceae (Asterid) species. We successfully PCR amplified a set of these markers for lulos (34 out of 46 initially tested) and tree tomatoes (26 out of 41) for molecular studies. Six polymorphic COSII markers were found in lulo with a total of 47 alleles and five polymorphic markers in tree tomato with a total of 39 alleles in the two populations. Further genetic analyses indicated a high population structure (with FST > 0.90), which may be a result of low migration between populations, adaptation to various niches and the number of markers evaluated. We propose COSII markers as sound tools for molecular studies, conservation and the breeding of these two fruit species

Ethylene supports colonization of plant roots by the mutualistic fungus Piriformospora indica

The mutualistic basidiomycete Piriformospora indica colonizes roots of mono- and dicotyledonous plants, and thereby improves plant health and yield. Given the capability of P. indica to colonize a broad range of hosts, it must be anticipated that the fungus has evolved efficient strategies to overcome plant immunity and to establish a proper environment for nutrient acquisition and reproduction. Global gene expression studies in barley identified various ethylene synthesis and signaling components that were differentially regulated in P. indica-colonized roots. Based on these findings we examined the impact of ethylene in the symbiotic association. The data presented here suggest that P. indica induces ethylene synthesis in barley and Arabidopsis roots during colonization. Moreover, impaired ethylene signaling resulted in reduced root colonization, Arabidopsis mutants exhibiting constitutive ethylene signaling, -synthesis or ethylene-related defense were hyper-susceptible to P. indica. Our data suggest that ethylene signaling is required for symbiotic root colonization by P. indica

Antihypertensive Effects of a Central Arteriovenous Anastomosis Are Mediated Through Profound Reduction in Systemic Vascular Resistance

This is a non-final version of an article published in final form in Circulation: Cardiovascular InterventionsDr Sobotka reports personal fees and other from ROX Medical. Dr Lobo reports personal fees from ROX Medical

DEM of triaxial tests on crushable sand

This paper presents simulations of high-pressure triaxial shear tests on a crushable sand. The discrete element method is used, featuring a large number of particles and avoiding the use of agglomerates. The triaxial model features a flexible membrane, therefore allowing realistic deformation, and a simple breakage mechanism is implemented using the octahedral shear stress induced in the particles. The simulations show that particle crushing is essential to replicate the realistic behaviour of sand (in particular the volumetric contraction) in high-pressure shear tests. The general effects of crushing during shear are explored, including its effects on critical states, and the influence of particle strength and confining pressure on the degree of crushing are discussed