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1.
Mastocytosis is a type of myeloid neoplasm characterized by the clonal, neoplastic proliferation of morphologically and immunophenotypically abnormal mast cells that infiltrate one or more organ systems. Systemic mastocytosis (SM) is a more aggressive variant of mastocytosis with extracutaneous involvement, which might be associated with multi-organ dysfunction or failure and shortened survival. Over 80% of patients with SM carry the KIT D816V mutation. However, the KIT D816V mutation serves as a weak oncogene and appears to be a late event in the pathogenesis of mastocytosis. The management of SM is highly individualized and was largely palliative for patients without a targeted form of therapy in past decades. Targeted therapy with midostaurin, a multiple kinase inhibitor that inhibits KIT, has demonstrated efficacy in patients with advanced SM. This led to the recent approval of midostaurin by the United States Food and Drug Administration and European Medicines Agency. However, the overall survival of patients treated with midostaurin remains unsatisfactory. The identification of genetic and epigenetic alterations and understanding their interactions and the molecular mechanisms involved in mastocytosis is necessary to develop rationally targeted therapeutic strategies. This review briefly summarizes recent developments in the understanding of SM pathogenesis and potential treatment strategies for patients with SM. 相似文献
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Myelodysplastic syndromes (MDS) are highly heterogeneous myeloid neoplasms, and a large number of
patients are difficult to diagnose and classify by blood and bone marrow examination. As a surface marker of
granulocyte, studies have shown CD10 can be used to define the degree of granulocyte maturation in MDS patients.
However, whether it can be used for differential diagnosis of MDS and other hematological diseases remains
inconclusive. To explore the value of CD10 for differential diagnosis of MDS, 60 newly diagnosed MDS, 20 aplastic
anemia (AA) patients, and 35 iron-deficient anemia (IDA) patients were selected for this study. Bone marrow (BM)
specimens were processed for surface marker analysis and labeled with pre-conjugated monoclonal antibodies. Stained
cells were detected by flow cytometry. Our results indicated that CD10-positive granulocytes were significantly
decreased in BM of MDS patients than AA and IDA patients, and the level of CD10-positive mature granulocytes was
not associated with the clinical stages of malignancy. Receiver operating characteristic (ROC) areas under the curve
(AUC) of CD10-positive granulocytes was 0.86 and 0.85, respectively, in MDS patients than the IDA group and AA
group with good specificity and sensitivity. Further, CD10-positive granulocytes were increased after effective
treatment. In conclusion, we found the decrease in CD10-positive granulocytes has a differential diagnostic value of MDS. 相似文献
4.
In the first critical assessment of knowledge economy dynamic paths in Africa and the Middle East, but for a few exceptions, we find overwhelming support for diminishing cross-country disparities in knowledge-based economy dimensions. The paper employs all the four components of the World Bank's Knowledge Economy Index (KEI): economic incentives, innovation, education, and information infrastructure. The main finding suggests that sub-Saharan African (SSA) and the Middle East and North African (MENA) countries with low levels of KE dynamics and catching-up their counterparts of higher KE levels. We provide the speeds of integration and time necessary to achieve full (100%) integration. Policy implications are also discussed. 相似文献
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In the present work, inter-diffusion of nickel and titanium and formation of Ni-Ti intermetallic compounds on Ti-6Al-4V substrate have been studied. Initially, nickel was electrodeposited on the alloy using a modified Watts bath solution at a current density of 2 A dm?2 for 1?h. The coated specimens were then heat treated for different durations at 750, 800 and 850 °C under argon atmosphere. The effects of temperature and time on the characteristics, hardness and wear resistance of intermetallic phases were investigated. The results showed that a multilayer structure was formed after heat treatment, an outer layer of residual nickel, an area of intermetallic layers with different compositions followed by a solid solution of Ni-Ti. It was also observed that an increase in time or temperature at first led to the formation of thicker intermetallic layers; however, after passing a critical point, the intermetallic layers seem to dissolve into the substrate. Furthermore, the wear rates of the diffusion treated samples were four times lower compared to Ti-6Al-4V alloy when sliding against AISI 52100 hardened steel. 相似文献
7.
目的: 探讨自身免疫性溶血性贫血(AIHA)患者外周血中调节性B细胞(Bregs)的表达及其在该病发病中的意义。方法:选择16例自身免疫性溶血性贫血患者和14例健康志愿者为研究对象,用流式细胞术分析外周血CD19+IL-10+调节性B细胞及CD19+CD24hiCD27+调节性B细胞的表达;ELISA方法检测培养上清液中IL-10的水平。结果:自身免疫性溶血性贫血患者外周血中CD19+IL-10+调节性B细胞、CD19+CD24hiCD27+调节性B细胞的表达分别为(1.27±0.39)%、(9.85±2.18)%,健康志愿者组分别为(2.92±0.71)%、(26.47±4.31)%,两组比较差异有统计学意义(P<0.05);自身免疫性溶血性贫血患者细胞培养上清中IL-10的水平低于健康志愿者组(P<0.05)。结论:自身免疫性溶血性贫血患者外周血中CD19+IL-10+调节性B细胞及CD19+CD24hiCD27+调节性B细胞比例降低,提示调节性B细胞可能参与自身免疫性溶血性贫血的发病过程。 相似文献
8.
F. Schröder R. M. Ward A. R. Walpole R. P. Turner M. M. Attallah J.-C. Gebelin 《Materials Science & Technology》2015,31(3):372-384
Linear friction welding of the Ti6Al4V alloy is studied. A new definition of the energy input rate is proposed, based on an integration over time of the in-plane force and velocity; a strong correlation with the upset rate is then found. The effective friction coefficient is estimated to be 0·5±0·1 for varying frequencies and amplitudes, with only a weak dependence on the processing conditions displayed. A model is proposed that accounts for both the conditioning and equilibrium stages of the process, which is shown to be in good agreement with the experimental data. The model is used to study the mechanism by which the flash is formed. A criterion is proposed by which the rippled nature of its morphology can be predicted. 相似文献
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