A new squat lobster of the genus Munidopsis (Crustacea, Decapoda, Munidopsidae) from the Mediterranean Sea

8 páginas, 4 figuras[EN]A new species of Munidopsis Whiteaves, 1874 is reported from the south of Crete, at 2046-2104 m, in the eastern Mediterranean Sea. The species (M. ariadne) is morphologically closely related to M. maunga Schnabel and Bruce, 2006, from New Zealand. The species is also close to M. polymorpha Koelbel, 1892, from Lanzarote, Canary Islands, and M. talismani A. Milne Edwards and Bouvier, 1894, from the NW Africa. The new species is described and illustrated and a diagnosis along with full illustration is provided for the latter 2 species.[ES]Una nueva especie de crustáceo del género Munidopsis (Crustacea: Decapoda: Munidopsidae) del mar Mediterráneo. – Se describe una especie nueva de Munidopsis Whiteaves, 1874 capturada al sur de Creta, a 2046-2104 m, en el Mediterráneo oriental. La especie (M. ariadne) es próxima morfológicamente a M. maunga Schnabel and Bruce, 2006, de Nueva Zelanda. La especie está también relacionada con M. polymorpha Koelbel, 1892, de Lanzarote, Islas Canarias, y M. talismani A. Milne Edwards and Bouvier, 1894, del NO de África.Peer reviewe

Spatial surface-pattern analyses and boundary detection techniques applied in forest ecology

[EN] We review methods for uni- and multivariate surface pattern analysis and boundary detection used in forest ecology. A continuous surface pattern is defined as the locations of points (trees) in the space and the associated variable or variables. We illustrate useful methods to describe spatial patterns and infer the generating processes. We show the statistical basis and applied examples of univariate methods for binary (join counts) and quantitative variables (Moran and Geary correlograms, semivariograms, fractal dimension). We explain the calculus and interpretation of multivariate methods to describe surface patterns (Mantel test and correlogram) and their relationships with ordination methods. Finally, we show examples of techniques for boundary detection. Most analysed patterns corresponded to Pinus uncinata forests from the upper altitudinal limit in the Pyrenees or from a relict population. We discuss the advantages and disadvantages of each methodology and their applications in forest ecology.[ES] En este trabajo se revisan los métodos de análisis univariable y multivariable de los patrones de superficies y de detección de fronteras más utilizados en ecología forestal. El patrón de superficies es un patrón espacial continuo definido por las posiciones de los puntos (árboles) en el espacio y una o varias variables asociadas a cada punto. Se ilustran métodos útiles para describir patrones espaciales e inferir los procesos que los generaron. Se muestra el fundamento estadístico y ejemplos aplicados de métodos de análisis univariables para variables binarias (conteo contiguo) y cuantitativas (correlogramas de Moran y Geary, semivariogramas, dimensión fractal). Se detalla el cálculo e interpretación de métodos multivariables para la descripción de patrones de superficies (correlograma y test de Mantel) y su relación con los métodos de ordenación. Finalmente, se muestran ejemplos de métodos para la detección de fronteras. La mayor parte de los patrones reales analizados provienen de bosques de Pinus uncinata del límite altitudinal superior en los Pirineos o bien de una población relíctica. Se discuten las ventajas y desventajas de cada metodología y sus aplicaciones en ecología forestal.Los datos de Vinuesa se obtuvieron en el proyecto AMB95-0160 (CICyT).Peer reviewe

Pegcetacoplan versus Eculizumab in Paroxysmal Nocturnal Hemoglobinuria

Background Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease characterized by chronic complement-mediated hemolysis. C5 inhibition controls intravascular hemolysis in untreated PNH but cannot address extravascular hemolysis. Pegcetacoplan, a pegylated peptide targeting proximal complement protein C3, potentially inhibits both intravascular and extravascular hemolysis. Methods We conducted a phase 3 open-label, controlled trial to assess the efficacy and safety of pegcetacoplan as compared with eculizumab in adults with PNH and hemoglobin levels lower than 10.5 g per deciliter despite eculizumab therapy. After a 4-week run-in phase in which all patients received pegcetacoplan plus eculizumab, we randomly assigned patients to subcutaneous pegcetacoplan monotherapy (41 patients) or intravenous eculizumab (39 patients). The primary end point was the mean change in hemoglobin level from baseline to week 16. Additional clinical and hematologic markers of hemolysis and safety were assessed. Results Pegcetacoplan was superior to eculizumab with respect to the change in hemoglobin level from baseline to week 16, with an adjusted (least squares) mean difference of 3.84 g per deciliter (P Conclusions Pegcetacoplan was superior to eculizumab in improving hemoglobin and clinical and hematologic outcomes in patients with PNH by providing broad hemolysis control, including control of intravascular and extravascular hemolysis. (Funded by Apellis Pharmaceuticals; PEGASUS ClinicalTrials.gov, NCT03500549. opens in new tab.

Standards of pathology in the diagnosis of systemic mastocytosis: recommendations of the EU-US cooperative group

Pathology plays a central role in the diagnosis of systemic mastocytosis (SM), its delineation from other neoplasms and reactive conditions, and in monitoring of SM under therapy. The morphologic hallmark of SM is the accumulation of spindle-shaped, hypogranulated mast cells (MCs) in bone marrow (BM) and other extracutaneous tissues. Four of the 5 World Health Organization–defined diagnostic criteria (ie, compact MC aggregates [=major criterion]; atypical MC morphology; activating KIT point mutations; aberrant expression of CD25 and/or CD2 and/or CD30 in MCs [=minor criteria]) can be addressed by the pathologist. The final classification of SM variants as either BM mastocytosis, indolent SM, smoldering SM, aggressive SM (ASM), SM with an associated hematologic neoplasm (SM-AHN), or MC leukemia (MCL) has important prognostic significance and requires the integration of certain morphological, clinical, radiological, and biochemical data, referred to as B- and C-findings. Substantial diagnostic challenges may be posed to the pathologist and clinician especially in the so-called advanced SM variants, that is, ASM, MCL, and SM-AHN. In this article, updated recommendations of the EU-US Cooperative Group regarding standards of pathology in the diagnosis of SM, presented during the year 2020 Working Conference held in September in Vienna, are reported.T. I. George was supported by the ARUP Institute for Clinical and Experimental Pathology. K. Hartmann was supported by the Swiss National Science Foundation, grant number 310030_207705. D. D. Metcalfe, J. J. Lyons, and M. Carter were supported by the Division of Intramural Research, National Institutes of Allergic and Infectious Diseases, National Institutes of Health (NIH). The content is solely the responsibility of the authors and does not represent the official views of the NIH. P. Valent was supported by the Austrian Science Funds (FWF), projects F4701-B20 and F4704-B20

Cancer Stem Cell-Like Cells Derived from Malignant Peripheral Nerve Sheath Tumors

This study aims to examine whether or not cancer stem cells exist in malignant peripheral nerve sheath tumors (MPNST). Cells of established lines, primary cultures and freshly dissected tumors were cultured in serum free conditions supplemented with epidermal and fibroblast growth factors. From one established human MPNST cell line, S462, cells meeting the criteria for cancer stem cells were isolated. Clonal spheres were obtained, which could be passaged multiple times. Enrichment of stem cell-like cells in these spheres was also supported by increased expression of stem cell markers such as CD133, Oct4, Nestin and NGFR, and decreased expression of mature cell markers such as CD90 and NCAM. Furthermore, cells of these clonal S462 spheres differentiated into Schwann cells, smooth muscle/fibroblast and neurons-like cells under specific differentiation-inducing cultural conditions. Finally, subcutaneous injection of the spheres into immunodeficient nude mice led to tumor formation at a higher rate compared to the parental adherent cells (66% versus 10% at 2.5×105). These results provide evidence for the existence of cancer stem cell-like cells in malignant peripheral nerve sheath tumors

Efficacy and safety of avapritinib in advanced systemic mastocytosis:interim analysis of the phase 2 PATHFINDER trial

Advanced systemic mastocytosis (AdvSM) is a rare, KIT D816V-driven hematologic neoplasm characterized by mast cell infiltration and shortened survival. We report the results of a prespecified interim analysis of an ongoing pivotal single-arm phase 2 trial (no. NCT03580655) of avapritinib, a potent, selective KIT D816V inhibitor administered primarily at a once-daily starting dose of 200 mg in patients with AdvSM (n = 62). The primary endpoint was overall response rate (ORR). Secondary endpoints included mean baseline change in AdvSM–Symptom Assessment Form Total Symptom Score and quality of life, time to response, duration of response, progression-free survival, overall survival, changes in measures of disease burden and safety. The primary endpoint was successfully met (P = 1.6 × 10(-9)), with an ORR of 75% (95% confidence interval 57–89) in 32 response-evaluable patients with AdvSM who had sufficient follow-up for response assessment, including 19% with complete remission with full or partial hematologic recovery. Reductions of ≥50% from baseline in serum tryptase (93%), bone marrow mast cells (88%) and KIT D816V variant allele fraction (60%) were observed. The most frequent grade ≥3 adverse events were neutropenia (24%), thrombocytopenia (16%) and anemia (16%). Avapritinib demonstrated a high rate of clinical, morphological and molecular responses and was generally well tolerated in patients with AdvSM