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Although several decades have passed since the description of myeloproliferative neoplasms (MPN), many aspects of their pathophysiology have not been elucidated. In this review, we discuss the mutational landscape of patients with essential thrombocythemia (ET), prognostic scores and salient pathology, and clinical points. We discuss also the diagnostic challenges of differentiating ET from prefibrotic MF. We then focus on post-essential thrombocythemia myelofibrosis (post-ET MF), a rare subset of MPN that is usually studied in conjunction with post-polycythemia vera MF. The transition of ET to post-ET MF is not well studied on a molecular level, and we present available data. Patients with secondary MF could benefit from allogenic hematopoietic stem cell transplantation, and we present available data focusing on post-ET MF.

FLT3 internal tandem duplicate (ITD) is associated with unfavorable prognosis of acute myeloid leukemia; targeted therapy improves clinical outcome. We propose that FLT3-ITD detected by next generation sequencing (NGS) should be reported with the same nomenclature pattern as single nucleotide variants so that the mutation can be better interpreted clinically.A Python-based web application was developed to generate FLT3-ITD nomenclature as recommended by the Human Genome Variation Society (HGVS). Assembled FLT3-ITD sequences from 84 patients and 11 artificially created ITD sequences were used for the validation of this web-based application. Each sequence was inspected manually to confirm that the nomenclature was accurate.Accurate nomenclatures were generated for 113 of 114 sequencing results and 7 artificial sequences. One assembled sequence and four artificial sequences were not named accurately; warning statements were automatically generated to alert further inspection. Of the 105 unique FLT3-ITDs, the ITD lengths range from 18 to 300 bp. Depending whether the ITD involves intron or extends into exon 15, three patterns were recognized. Only 44 (42%) ITDs were pure duplications, and three types of variants were identified at the 5' of ITD. When ITD involves intronic sequence, the protein may comprise inserted amino acids encoded by the intron, due to disrupted RNA splicing.The web application generates accurate FLT3-ITD nomenclature from NGS results except in rare situations. The HGVS nomenclatures provide information on the molecular architecture of FLT3-ITDs and reveal details of complex insertions with partial duplications.

In order to introduce multifunctional properties into the recycled polyphenylene sulfite (PPS) nonwoven fabrics, highly conductive and hydrophobic fabrics with excellent Joule heating performance were fabricated by depositing in-situ polymerized polypyrrole (PPy) onto PPS nonwoven fabric and followed by a silicone coating process. The resultant PPy-coated nonwoven fabric exhibited high electrical conductivity. The surface resistivity of PPy-coated nonwoven fabric was less than 8.8 Ω/sq. The high conductivity of PPy coating provided the fabric with excellent electrical heating properties in conjunction with a photothermal heating properties. The saturation temperature of the samples can get to 125.6°C with 4V DC power. The thin silicone coating can help to resist the oxidation of PPy and endow the nonwoven fabrics with excellent water-resistant features. This study provided a simple and effective method to transform the recycled PPS nonwoven fabric into a multifunction material.

A comprehensive understanding of the slip phenomenon on liquid/solid interfaces is essential for multiple real-world applications of superhydrophobic materials, especially those involving drag reduction. In the current contribution, the so-called "slip-length" on an irregularly structured superhydrophobic surface was systematically evaluated, with respect to varying liquid surface tension and viscosity. The superhydrophobic polymer-nanoparticle composite (SPNC) material used exhibits a dual-scale surface roughness and was fabricated via coating a surface with a mixture of polydimethylsiloxane solution and functionalized silica particles. A cone-and-plate rheometric device was employed to quantify the slip length. To independently study the impact of surface tension and viscosity, three types of aqueous solutions were used: sodium dodecyl sulfate, ethanol, and polyethylene glycol. Our experimental results demonstrate that a decreasing surface tension results in a decreasing slip length when the fluid viscosity is held constant. Meanwhile, the slip length is shown to increase with increasing viscosity when the surface tension of the various liquids is matched to isolate effects. The study reveals a linear relationship between slip length and both capillary length and viscosity providing a reference to potentially predict the degree of achievable drag reduction for differing fluids on SPNC surfaces.

Echinococcus shiquicus is currently limited to the Qinghai–Tibet plateau, a large mountainous region in China. Although the zoonotic potential remains unknown, progress is being made on the distribution and intermediate host range. In this study, we report E. shiquicus within Gansu and Qinghai provinces in regions located not only around the central areas but also the southeast edge of the plateau and describe their genetic relationship with previous isolates from the plateau. From 1879 plateau pikas examined, 2.39% (95% CI 1.79–3.18) were infected with E. shiquicus. The highest prevalence of 10.26% (4.06–23.58) was recorded in Makehe town, Qinghai province. Overall the prevalence was marginally higher in Qinghai (2.5%, CI 1.82–3.43) than in Gansu (2%, CI 1.02–3.89). The cox1 and nad1 genes demonstrated high and low haplotype and nucleotide diversities, respectively. The median-joining network constructed by the cox1–nad1 gene sequences demonstrated a star-like configuration with a median vector (unsampled haplotype) occupying the centre of the network. No peculiar distinction or common haplotype was observed in isolates originating from the different provinces. The presence of E. shiquicus in regions of the southeast and northeast edges of the Qinghai–Tibet plateau and high genetic variation warrants more investigation into the haplotype distribution and genetic polymorphism by exploring more informative DNA regions of the mitochondrial genome to provide epidemiologically useful insight into the population structure of E. shiquicus across the plateau and its axis.

A polysilsesquioxane (PSQ) sheet with novel three-dimensional (3-D) networks was prepared by a one-pot reaction of an organic silane terminated with a hexadecyl functionality at room temperature. The effects of experimental parameters on the morphology of PSQ were systematically investigated. It was found that the interconnected structure of the material was mainly dominated by the alkyl long chain in the silane. The mechanism behind the formation of this unique structure was proposed: the hydrolyzed silane collided with each other at first, then the hydrolyzate condensed to form aggregates, and finally the network structure was assembled under intermolecular driving forces among these aggregates with the balanced hydrogen bond interaction and hydrophobic effect of the alkyl chain in silane. The excellent hydrophobic properties of 3-D PSQ have made it a potential candidate for the treatment of oil spills, with a maximum adsorption capacity for crude oil of 6.8 ± 0.2 g g−1.

Disassembly planning is an effective way to recycle valuable parts or materials from end-of-life products. Due to the parts are easily affected by some unpredictable factors in the real disassembly process (such as defective parts and human factors), there may be failure operations that cannot successfully disassemble the corresponding parts in the real world. Therefore, a partial disassembly sequence planning with uncertainty is proposed in the paper, where the success rates of disassembly operations are formulated to model an expected profit-based disassembly sequence planning. Also, a general variable neighborhood search algorithm is developed to optimize the mathematical model. Meanwhile, in the approach, a heuristic procedure is utilized to obtain a high-quality initial solution, and four different neighborhood structures are introduced to improve the disassembly solution. Its superiority and effectiveness are well illustrated by two case studies and comparison with two existing metaheuristics, i.e., artificial bee colony algorithm and genetic algorithm.

Primary cutaneous Ewing sarcoma is a very rare entity with less than 100 cases reported in the literature, sharing the same morphological and immunohistochemical characteristics as their osseous counterparts. Herein, to the best of our knowledge, we report the first case in English literature of a molecularly confirmed Ewing sarcoma with diffuse and strong SOX10 immunoreactivity. This exceedingly rare immunohistochemical finding along with the rarity of this tumor could easily lead to a misdiagnosis with significant repercussions. Our case highlights the difficulty in diagnosing primary cutaneous Ewing sarcoma as well as the pivotal role molecular diagnostics can play in specific scenarios.

Digital twin (DT) technology is an effective way to realize intelligent manufacturing, which has been increasingly received attention in both academia and industry. Thus, it is rather necessary and significant to collaboratively accomplish the research and development (R&D) of DT technology (RDDT). To explore a school-enterprise collaborative R&D strategy on DT technology, this paper proposes a differential game-based approach to compute the optimal R&D effort levels and optimal incomes of both parties in the school-enterprise collaborative innovation (SECI) system. First, using Berman’s continuous dynamic programming theory, the optimal R&D effort levels, the optimal incomes of both parties, and total optimal income in the SECI system are calculated in three cases: Nash non-cooperative game, Stackelberg master-slave game and cooperative game. Second, the equilibria of the three game cases are analyzed and compared. Finally, a numerical example is used to verify the validity of the conclusion, and we find that the optimal benefit of two parties in cooperative game are significantly better than those of Nash non-cooperative game and Stackelberg master-slave game, which effectively demonstrates the superiority of school-enterprise collaborative R&D on DT technology.

Reactive intralymphovascular immunoblastic proliferations (ILVIPs) may mimic aggressive lymphomas and are rarely reported. Herein, we characterize the clinicopathologic features of 8 patients with ILVIPs. No patients had lymphadenopathy, hepatosplenomegaly, or other findings suggestive of lymphoma. The ILVIPs involved the small or large intestine (n=5) and appendix (n=3). Patients were evaluated for abdominal pain, suspected appendicitis, intestinal obstruction, diverticulitis, volvulus, or tumor resection. Histologic sections showed expanded lymphovascular spaces filled by intermediate to large immunoblasts, positive for CD38, CD43, CD45, CD79a, and MUM1/IRF4 in all cases tested. Five of 6 (83%) cases were positive for CD30. CD20 was weakly positive in a subset of cells in 2 (25%) cases, and PAX5 was weakly positive in 4 (50%) cases. The immunoblasts expressed polytypic light chains in all cases tested. In 1 case, a subset of immunoblasts expressed T-cell markers indicating the presence of a T-cell component. The immunoblasts were negative for ALK, BCL-2, BCL-6, CD10, CD56, CD138, and Epstein-Barr virus-encoded small RNA in all cases assessed. The proliferation index shown by Ki-67 was high with a median of 80%. In all 6 cases tested, the immunoblasts were shown within lymphatic channels highlighted by D2-40. In conclusion, ILVIPs can be rarely observed in patients with inflammatory or infectious conditions, especially in gastrointestinal tract surgical specimens. The immunoblasts are predominantly of B-lineage with a postgerminal center immunophenotype and are located within lymphatic channels. It is essential to distinguish reactive ILVIPs from aggressive lymphomas to avoid unnecessary therapy.

A novel but simple design of transmission line with a parasitic patch that works as a phase shifter at the frequency range of 1.5 - 3 GHz is presented. Variable capacitors attached to a parasitic element above the transmission line change the phase of the propagating wave with minimum reflection. A 90 degree of phase shift is achieved with capacitance of 4.5 pF variation.

It has been shown that phages have evolved anti-CRISPR (Acr) proteins to inhibit host CRISPR-Cas systems. Most acr genes are located upstream of anti-CRISPR-associated (aca) genes, which is instrumental for identifying these acr genes. Thus far, eight Aca families (Aca1–Aca8) have been identified, all proteins of which share low sequence homology and bind to different target DNA sequences. Recently, Aca1 and Aca2 proteins were discovered to function as repressors by binding to acr-aca promoters, thus implying a potential anti-anti-CRISPR mechanism. However, the structural basis for the repression roles of Aca proteins is still unknown. Here, we elucidated apo-structures of Aca1 and Aca2 proteins and their complex structures with their cognate operator DNA in two model systems, the Pseudomonas phage JBD30 and the Pectobacterium carotovorum template phage ZF40. In combination with biochemical and cellular assays, our study unveils dimerization and DNA-recognition mechanisms of Aca1 and Aca2 family proteins, thus revealing the molecular basis for Aca1- and Aca2-mediated anti-CRISPR repression. Our results also shed light on understanding the repression roles of other Aca family proteins and autoregulation roles of acr-aca operons.

Context.— In the 2017 revised World Health Organization classification of tumors of hematopoietic and lymphoid tissues, some mature T-cell lymphomas were reclassified and a few new provisional entities were established based on new data from clinical and laboratory studies. T follicular helper cell lymphoma is identified by T follicular helper cell markers. Anaplastic large cell lymphoma, ALK negative, is a better-defined entity based on genetic abnormalities, and breast implant–associated anaplastic large cell lymphoma is recognized as a provisional entity. The gastrointestinal T-cell lymphomas are reclassified, with addition of a new provisional entity, indolent T-cell lymphoproliferative disorder of the gastrointestinal tract, characterized by an indolent clinical course. Objective.— To review the diagnostic approaches to reclassified and newly established entities of mature T-cell lymphomas, focusing on significant immunophenotypic features and molecular genetic abnormalities. Relevant new discoveries after the publication of the 2017 World Health Organization classification are included. Data Sources.— Information from the literature most relevant to the 2017 World Health Organization revised classification and publications after 2016. Conclusions.— Incorporating clinical, morphologic, and immunophenotypic features usually provides sufficient evidence to reach a preliminary diagnosis of mature T-cell lymphoma. Molecular genetic studies can be very helpful for the final diagnosis and classification, especially in challenging cases. Some molecular genetic features have been found in breast implant–associated anaplastic large cell lymphoma, distinct from anaplastic large cell lymphoma, ALK negative. Immunohistochemical staining of 4 markers may enable further subtyping of peripheral T-cell lymphomas.

Late embryogenesis abundant (LEA) proteins are involved in the responses and adaptation of plants to various abiotic stresses, including dehydration, salinity, high temperature, and cold. Here, we report the first comprehensive survey of the LEA gene family in “Chinese Spring” wheat (Triticum aestivum). A total of 179 TaLEA genes were identified in T. aestivum and classified into eight groups. All TaLEA genes harbored the LEA conserved motif and had few introns. TaLEA genes belonging to the same group exhibited similar gene structures and chromosomal locations. Our results revealed that most TaLEA genes contained abscisic acid (ABA)-responsive elements (ABREs) and various cis-acting elements associated with the stress response in the promoter region and were induced under ABA and abiotic stress treatments. In addition, 8 genes representing each group were introduced into E. coli and yeast to investigate the protective function of TaLEAs under heat and salt stress. TaLEAs enhanced the tolerance of E. coli and yeast to salt and heat, indicating that these proteins have protective functions in host cells under stress conditions. These results increase our understanding of LEA genes and provide robust candidate genes for future functional investigations aimed at improving the stress tolerance of wheat.

High-temperature adsorptive heat transformer for steam generation has been experimentally investigated by introducing composite zeolite/CaCl2-water working pair based on a direct contact method. Composite adsorbents are prepared by immersing zeolite into different mass concentrations of CaCl2 solutions. SEM (Scanning Electron Microscope) is used to observe the surface structure of the composite zeolite. XRF (X-Ray Fluorescence) is selected to analyze the element mass ratios in adsorbents. BET (Brunauer-Emmett-Teller models) is employed to calculate the pore characteristic of pores inside zeolite. Characterization results confirm the success of preparation for composite zeolite. Adsorption properties including equilibrium water uptake and integral adsorption heat are measured for basic evaluation. Overall volumetric adsorption heat is increased by 13.1% for CA40% (immersion of zeolite in CaCl2 solution concentration at 40%) compared with that for 13X. Cyclic experiments are conducted to test the design of system. Superheated steam above 200 °C is generated for 13X and different composite zeolites from hot water below 80 °C. Dry gas at 130 °C is used for regeneration. Gross temperature lift is more than 100 °C for single stage zeolite adsorptive heat transformer. Dynamic steam generation on interface between water and zeolite is enhanced with more heat released by using composite zeolite. Subsequently, adsorption equilibrium is easier to be achieved inside the whole range of the packed bed. Effective time ratio for steam generation is elevated by 18.6% for CA40% compared with that for 13X. Mass of generated steam is raised by 12.9% simultaneously. Both the time and mass of generated steam have been obviously promoted with the increase of CaCl2 impregnated in zeolite. COPex (Exergy Coefficient of Performance) is kept constant while SHP (Specific Heating Power for steam generation) is increased by 12.6%.