Summary
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy derived from the precursors of plamacytoid dendritic cells, with an aggressive clinical course and ...high frequency of cutaneous and bone marrow involvement. Neoplastic cells express CD4, CD43 (also termed SPN), CD45RA and CD56 (also termed NCAM1), as well as the plasmacytoid dendritic cell‐associated antigens CD123 (also termed IL3RA), BDCA‐2 (also termed CD303, CLEC4E) TCL1 and CTLA1 (also termed GZMB). The median survival is only a few months as the tumour exhibits a progressive course despite initial response to chemotherapy. The best modality of treatment remains to be defined. Generally, patients receive acute leukaemia‐like induction, according to acute myeloid leukaemia (AML)‐type or acute lymphoid leukaemia (ALL)‐type regimens. The frequent neuromeningeal involvement indicates systematic pre‐emptive intrathecal chemotherapy in addition to intensive chemotherapy. Allogeneic haematopoietic stem cell transplantation (HSCT), particularly when performed in first remission, may improve the survival. Preliminary data suggest a potential role for immunomodulatory agents and novel targeted drugs. Herein epidemiology, clinical manifestations, diagnosis and management of BPDCN will be presented. In detail, this review focuses on the therapeutic aspects of BPDCN, proposing a treatment algorithm for the management of the disease, including induction chemotherapy, allogeneic HSCT and intrathecal prophylaxis at different steps of treatment, according to compliance, biological and clinical characteristics of patients.
Background. The purpose of our study was to evaluate the incidence and outcome of invasive fungal infection (IFI) among patients who underwent autologous or allogeneic hematopoietic stem cell ...transplantation (HSCT) at 11 Italian transplantation centers. Methods. This cohort-retrospective study, conducted during 1999–2003, involved HSCT patients admitted to 11 tertiary care centers or university hospitals in Italy, who developed IFIs (proven or probable). Results. Among 3228 patients who underwent HSCT (1249 allogeneic HSCT recipients and 1979 autologous HSCT recipients), IFI occurred in 121 patients (overall incidence, 3.7%). Ninety-one episodes (2.8% of all patients) were due to molds, and 30 (0.9%) were due to yeasts. Ninety-eight episodes (7.8%) occurred among the 1249 allogeneic HSCT recipients, and 23 (1.2%) occurred among the 1979 autologous HSCT recipients. The most frequent etiological agents were Aspergillus species (86 episodes) and Candida species (30 episodes). The overall mortality rate was 5.7% among allogeneic HSCT recipients and 0.4% among autologous HSCT recipients, whereas the attributable mortality rate registered in our population was 65.3% (72.4% for allogeneic HSCT recipients and 34.7% for autologous HSCT recipients). Etiology influenced the patients' outcomes: the attributable mortality rate for aspergillosis was 72.1% (77.2% and 14.3% for allogeneic and autologous HSCT recipients, respectively), and the rate for Candida IFI was 50% (57.1% and 43.8% for allogeneic and autologous HSCT recipients, respectively). Conclusions. IFI represents a common complication for allogeneic HSCT recipients. Aspergillus species is the most frequently detected agent in these patients, and aspergillosis is characterized by a high mortality rate. Conversely, autologous HSCT recipients rarely develop aspergillosis, and the attributable mortality rate is markedly lower. Candidemia was observed less often than aspergillosis among both allogeneic and autologous HSCT recipients; furthermore, there was no difference in either the incidence of or the attributable mortality rate for candidemia among recipients of the 2 transplant types.
Anthracnose (ANT) and angular leaf spot (ALS) caused by Colletotrichum lindemuthianum and Pseudocercospora griseola, respectively, are devastating diseases of common bean around the world. Therefore, ...breeders are constantly searching for new genes with broad-spectrum resistance against ANT and ALS. This study aimed to characterize the genetic resistance of California Dark Red Kidney (CDRK) to C. lindemuthianum races 73, 2047, and 3481 and P. griseola race 63-39 through inheritance, allelism testing, and molecular analyses. Genetic analysis of response to ANT and ALS in recombinant inbred lines (RILs) from a CDRK × Yolano cross (CY) showed that the resistance of CDRK cultivar is conferred by a single dominant loci, which we named CoPv01CDRK/PhgPv01CDRK. Allelism tests performed with race 3481showed that the resistance gene in CDRK is independent of the Co-1 and Co-AC. We conducted co-segregation analysis in genotypes of 110 CY RILs and phenotypes of the RILs in response to different races of the ANT and ALS pathogens. The results revealed that CoPv01CDRK and PhgPv01CDRK are coinherited, conferring resistance to all races. Genetic mapping of the CY population placed the CoPv01CDRK/PhgPv01CDRK loci in a 245 Kb genomic region at the end of Pv01. By genotyping 19 RILs from the CY population using three additional markers, we fine-mapped the CoPv01CDRK/PhgPv01CDRK loci to a smaller genomic region of 33 Kb. This 33 Kb region harbors five predicted genes based on the common bean reference genome. These results can be applied in breeding programs to develop bean cultivars with ANT and ALS resistance using marker-assisted selection.
Background and Aims
Global warming can lead to technological ripening occurring in advance of phenolic maturity for red wine cultivars. This study evaluated the effect of post‐budburst winter pruning ...on the phenology, yield components, berry composition and phenolic maturity in Vitis vinifera L. cv. Merlot.
Methods and Results
Mechanically pre‐pruned vines followed up with hand pruning during winter (Control) were compared to vines that were mechanically pre‐pruned and followed up with hand pruning after budburst when distal buds developed shoots with either three unfolded leaves (DF3) or eight unfolded leaves (DF8). Late pruning delayed budburst, flowering and, to a lesser extent, veraison. The delays were greater for DF8 than DF3 treatments. Yield decreased by about 40 and 71% in DF3 and DF8 vines, respectively, while sugar accumulation and reduction of TA were delayed by both pruning treatments. The concentration of anthocyanin and tannin and of extractable anthocyanin and tannin (of skin and seeds analysed separately) were not influenced by the DF3 treatment while tannin concentration increased in DF8 berries.
Conclusions
Delaying hand pruning of mechanically pre‐pruned vines until after budburst of distal nodes can delay technological ripening without affecting the concentration of anthocyanin and tannin of berries. Yield, however, is substantially reduced.
Significance of the Study
We verified the feasibility of a cost‐effective technique that can be adopted to counteract the hastening of sugar accumulation and organic acid decline caused by global warming on valuable black grapes.
We introduce a high-performance hyperspectral camera based on the Fourier-transform approach, where the two delayed images are generated by the Translating-Wedge-Based Identical Pulses eNcoding ...System (TWINS) Opt. Lett. 37, 3027 (2012), a common-path birefringent interferometer that combines compactness, intrinsic interferometric delay precision, long-term stability and insensitivity to vibrations. In our imaging system, TWINS is employed as a time-scanning interferometer and generates high-contrast interferograms at the single-pixel level. The camera exhibits high throughput and provides hyperspectral images with spectral background level of -30dB and resolution of 3 THz in the visible spectral range. We show high-quality spectral measurements of absolute reflectance, fluorescence and transmission of artistic objects with various lateral sizes.
COnstellation of small Satellites for Mediterranean basin Observation (COSMO-SkyMed) is the largest Italian investment in Space Systems for Earth Observation, commissioned and funded by Italian Space ...Agency (ASI) and Italian Ministry of Defence (MoD). COSMO-SkyMed is a Dual-Use (Civilian and Defence) end-to-end Earth Observation System aimed at establishing a global service supplying provision of data, products and services compliant with well-established international standards and relevant to a wide range of applications, such as Risk Management, Scientific and Commercial Applications and Defence Applications. The system consists of a constellation of four LEO mid-sized satellites, each equipped with a multi-mode high-resolution SAR operating at X-band. Three out of four COSMO-SkyMed satellites have been successfully launched the 8th of June, the 9th of December 2007 and the 25th of October 2008 respectively, while the remaining satellite will be deployed within 2010. COSMO-SkyMed 1 and 2 completed their commissioning phase to test, verify and qualify the overall system and from the 1st of August 2008 both satellites are in the operational phase. The third satellite is still performing its commissioning and it is expected to enter in operation in the second half of 2009. The results of the commissioning phase of COSMO-SkyMed 1 and 2 are presented together with the Scientific Mission Exploitation strategy (i.e. Announcement of Opportunity, Background Mission).
Pulmonary manifestations of SSc are leading causes of disease-related morbidity and mortality. Key clinical issues relate to the early detection of fibrotic lung disease, the interpretation of its ...significance, and decisions on when to start therapy. Respiratory symptoms are common in patients with SSc, but physical examination often fails to establish if the underlying cause is interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), impaired locomotion due to systemic disease or loss of fitness. Impaired lung function is usually evident on pulmonary function testing, with the pattern of functional impairment often discriminating usefully between ILD and PAH. The presence of PAH can be indicated by echocardiography and must be confirmed by right heart catheterization. Whereas chest radiographs detect established ILD, high-resolution CT can identify earlier or very mild inflammatory changes. Treatment options for ILD are limited to immunosuppressive agents, notably cyclophosphamide, often given in combination with low-dose prednisolone. Recent studies have shown that cyclophosphamide is effective in stabilizing pulmonary function—especially in patients with severe fibrotic disease—and in improving health-related quality of life. Progression of pulmonary manifestations and responses to treatment are best monitored using pulmonary function testing. For patients with severe end-stage pulmonary fibrosis, lung transplantation may offer a viable alternative therapeutic option.
The effect of elicitors on secondary metabolism in vines is receiving much interest, since it has been shown that they are able to increase the accumulation of phenolics, especially anthocyanins. ...This research aims to investigate the biochemical and molecular effects of the application of a commercial yeast derivative (Saccharomyces cerevisiae) on the accumulation of anthocyanins in potted Sangiovese vines. Experiments were performed on three consecutive years and the yeast derivative was applied at the beginning and at the end of veraison. Technological ripening, accumulation of anthocyanins and expression of the main genes involved in their biosynthesis were assessed. Technological ripening proceeded in a similar way in both treated and untreated berries in the three years. A significant increase in the concentration of anthocyanins was instead detected, following the induction by the yeast derivative of the expression of the genes involved in their biosynthesis. The research highlights the possibility of applying a specific inactivated yeast to increase the anthocyanin concentration even under the current climate change conditions, in Sangiovese, a cultivar extremely sensitive to high temperatures.
Non-motor symptoms in Parkinson's disease (PD) have been often described at different stages of the disease but they are poorly understood. We observed specific phenotypes related to these symptoms ...in mice lacking the PD‐associated GPR37/PAEL receptor. GPR37 is an orphan G‐protein‐coupled receptor highly expressed in the mammalian central nervous system. It is a substrate of parkin and it is involved in the pathogenesis of PD. GPR37 interacts with the dopamine transporter (DAT), modulating nigro‐striatal dopaminergic signaling and behavioral responses to amphetamine and cocaine. GPR37 knockout (KO) mice are resistant to MPTP and exhibit several motor behavioral abnormalities related to altered dopaminergic system function. To evaluate non-motor behavioral domains, adult and aged, male and female GPR37 KO mice and their wild‐type (WT) littermates were analyzed in a series of cross‐sectional studies. Aged GPR37 KO female mice showed mild improvements in olfactory function, while anxiety and depression‐like behaviors appeared to be significantly increased. A reduction of the startle response to acoustic stimuli was observed only in adult GPR37 KO mice of both genders. Furthermore, HPLC analysis of major neurotransmitter levels revealed gender differences in the striatum, hippocampus and olfactory bulb of mutant mice. The absence of GPR37 receptor could have a neuroprotective effect in an age and gender‐dependent manner, and the study of this receptor could be valuable in the search for novel therapeutic targets.
Anxiety and depression phenotypes in aged GPR37 KO female mice: relevance for the study of PD non‐motor symptoms.
PDF
