成人T-急性淋巴细胞白血病的特点及治疗

T急性淋巴细胞白血病(T-ALL)是一种生物学特征异质性很大的疾病,其免疫表型、基因表达谱对疾病的治疗和预后有重要的指导意义.许多研究报告认为无论儿童还是成人T-ALL治疗效果均不理想.随着治疗强度的加大,T-ALL疗效有所改善,但仍有相当比例的患者无法治愈.本文就T-ALL的临床、生物学特点及治疗现状和进展做一综述.     

套细胞淋巴瘤发病机制的研究进展

套细胞淋巴瘤(MCL)是一种侵袭性的非霍奇金淋巴瘤(NHL),该病的经典遗传学标志为t(11;14)(q13;q32)移位和细胞周期蛋白(cyclin) D1过表达,以难治和易复发为临床特征,并且总体预后不良.近年,随着对MCL细胞遗传学及分子发病机制研究的不断深入,靶向药物的开发与应用,使得该病的临床疗效有所提高.本文就近年来MCL发病机制中细胞遗传学、信号通路、转录因子、肿瘤微环境等改变的研究最新进展作一综述,旨在为MCL的早期诊断和靶向治疗提供新的方向.     

老年人急性髓细胞白血病的治疗进展

老年人急性髓细胞白血病(AML)患者接受治疗后,通常耐受性差,预后不佳.对于一般状况较好、无严重脏器合并症的老年人AML患者,标准剂量诱导化疗的疗效优于小剂量化疗及支持治疗;对于接受支持治疗的患者,小剂量化疗的疗效优于强化治疗方案;而无合并症、一般状况较好的老年人AML患者,可行非清髓性预处理异基因造血干细胞移植(allo-HSCT)及微移植等治疗.此外,新型药物的问世使老年人AML患者的预后得到改善.笔者拟就老年人AML的治疗进展进行综述.     

嵌合抗原受体T细胞在恶性血液肿瘤的临床应用中的研究进展

随着肿瘤免疫学理论和技术的发展,恶性血液肿瘤的治疗迎来新的纪元,过继性细胞免疫治疗(ACI)已成为研究热点.其中,利用嵌合抗原受体(CAR)修饰的T细胞治疗恶性血液肿瘤,在各项试验中均获得显著疗效.笔者拟就CAR-T细胞的构建及靶点选择,近年来与CAR-T细胞治疗相关的体内、外试验和临床研究,以及CAR-T细胞治疗目前尚待解决的问题进行综述.     

T细胞急性淋巴细胞白血病中的染色体易位及其受累基因的研究进展

染色体易位在T细胞急性淋巴细胞白血病(T-ALL)中是非常普遍的现象.易位涉及的癌基因在T-ALL的发病机制中起重要作用.T-ALL染色体易位主要包括涉及T细胞受体(TCR)基因位点的染色体易位和形成融合基因的染色体易位.通过对染色体易位导致相关癌基因表达改变的研究来探讨T-ALL的发病机制,为发展临床上有效的相关特异性治疗提供可能.本文就T-ALL中染色体易位和涉及基因的研究进展作一综述.     

63例T细胞急性淋巴细胞白血病患者MIC分型与临床预后分析

T细胞急性淋巴细胞白血病(T-ALL)约占ALL的20%,其生物学及临床特征不同于B细胞急性淋巴细胞白血病(B-ALL).我们回顾性分析了我院2000年1月至2007年6月63例住院初诊T-ALL患者的临床、免疫学分型和细胞遗传学资料,并就其与预后的关系进行讨论.     

急性T淋巴细胞白血病的分子遗传学研究进展

急性T淋巴细胞白血病(T-cell acute lymphoblastic leukemia,T-ALL)是以T淋巴细胞增殖为特征的恶性克隆性疾病.大约50%～70%的T-ALL患者可检出核型异常,细胞遗传学的改变已成为T-ALL最重要的诊断指标.近年来,随着荧光原位杂交(FISH)、微阵列-比较基因组杂交(array-CGH)以及cDNA芯片等细胞遗传学及分子生物学技术的广泛应用,人们认识到T-ALL患者的预后与染色体易位、缺失、基因突变等细胞和分子遗传学改变密切相关并取得一定进展.现将有关T-ALL分子遗传学的研究进展综述如下.     

一例难治性急性T淋巴细胞白血病硼替佐米联合化疗治疗临床分析

近年来,对于急性T淋巴细胞白血病(T-ALL)的治疗取得了一定的进展,治愈率也有所提高,但是对于复发的T-ALL患者,其预后仍不乐观.蛋白酶体抑制剂硼替佐米通过抑制核因子κB(NF-κB)依赖性的基因转录,已经广泛应用于多发性骨髓瘤(MM)、套细胞淋巴瘤(MCL)以及急性髓系白血病患者(AML)的治疗[1-3].然而,蛋白酶体抑制剂是否对于T-ALL患者有疗效以及如何选择适宜的患者值得探讨.     

成人急性淋巴细胞白血病自体造血干细胞移植联合化疗的临床研究

目的 观察成人急性淋巴细胞白血病(ALL)患者行自体造血干细胞移植(auto-HSCT)的疗效,并探讨相关的预后危险因素.方法 对2001年11月至2012年1月行auto-HSCT的86例成人ALL患者进行随访,并对所有患者的临床特征及治疗转归进行回顾性分析,应用Kaplan-Meier法进行生存分析,用COX回归模型评估独立预后因素.结果 可评价疗效的81例auto-HSCT患者中标危47例,高危34例.标危组患者1、3、5、10年无病生存(LFS)率分别为(82.3±5.7)％、(76.9±6.5)％、(74.1±6.8)％、(67.4±8.9)％;累积复发(RR)率分别为(13.6±5.2)％、(21.6±6.4)％、(24.5±6.8)％、(31.3±9.0)％.高危组患者1、3、5、10年LFS率分别为(55.8±8.9)％、(39.8±9.3)％、(39.8±9.3)％、(39.8±9.3)％;RR率分别为(38.8±9.2)％、(56.4±10.0)％、(56.4±10.0)％、(56.4±10.0)％.而疾病类型为T-ALL、初诊时WBC≥30×109/L、乳酸脱氢酶增高、TCR重排阳性及移植物来源于骨髓均为影响预后的不良因素,进一步应用COX多因素回归模型进行预后因素分析显示,移植物来源于骨髓是独立的预后不良因素.结论 成人ALL患者如果没有合适的供者,autoHSCT也是治疗的一种有效手段.     

弥漫性大B细胞淋巴瘤的治疗最新进展

弥漫性大B细胞淋巴瘤(DLBCL)是一组具有生物学异质性的B细胞恶性肿瘤.CD20单克隆抗体(如利妥昔单抗)的应用使得DLBCL患者的预后得到明显改善,但是仍有部分患者应用联合利妥昔单抗的化疗方案后疗效不佳.因此,根据DLBCL的不同亚型及细胞起源,选择不同的治疗方案,成为目前DLBCL治疗关注的重点.本文就DLBCL的亚型及细胞起源等方面,总结DLBCL治疗方法及其预后研究的最新进展.     

儿童遗传性心律失常的诊治进展

回顾在遗传性心律失常领域最新发表的相关研究,主要关注与儿童心源性猝死关系密切的离子通道病,包括长QT综合征（LQTS）、短QT综合征（SQTS）、Brugada综合征（BrS）和儿茶酚胺敏感性多形性室性心动过速（CPVT）,总结它们在发病机制及诊治方面的进展。     

The acoustic characteristics of the skull

Many investigators have stated that the difficulties of imaging with acoustical energy through the skull result from the marked attenuation of the energy by the skull. In the literature measurements of total attenuation have been confused with those for absorption.Measurements made by us show that absorption by compact bone varies between 2–3 dB cm^?1 MHz^?1 and, in the low megaHertz region appears to be directly proportional to frequency.It has also between shown that the convoluted inner surface of the ivory bone of the inner table of the skull may degrade the collimation and directionality of the beam by refraction.Cancellous bone, such as is present in the dipole of the skull, greatly attenuates the energy. It is postulated that this largely results from scattering. It is also postulated that the energy propagates through cancellous bone as two components, one in the soft tissues and the other partly in the bony spicules. Observations suggest that attenuation due to scattering much more markedly affects the latter of these components and scatters more greatly the higher frequencies in a pulse of broad bandwidth.The energy in each component has varying propagation paths so that the later cycles in the pulse of each component are subject to increasing interference as a result of the variations in propagation times. The two components moreover may have different propagation times so that interference may occur between the pulses of each component as well.All of these phenomena degrade the collimation, coherence, directionality, beam width, pulse length, frequency and other properties of the ultrasonic energy upon which imaging through the skull depends.The interference effects described above are least for the first cycle in the pulse which usually is not the cycle of highest amplitude. Since, in the free field, most of the energy is concentrated around the beam axis, most of the energy in the field which is deflected from its normal propagation path is deflected away from the beam axis. Thus the directionality of the beam is least degraded in the beam axis. The effects of the skull in degrading the properties of the ultrasonic pulse would therefore be lessened if the amplitude of the first cycle of the pulse and the directionality of its energy could be used for imaging.     

Organ Preservation: Current Concepts and New Strategies for the Next Decade

SUMMARY: Organ transplantation has developed over the past 50 years to reach the sophisticated and integrated clinical service of today through several advances in science. One of the most important of these has been the ability to apply organ preservation protocols to deliver donor organs of high quality, via a network of organ exchange to match the most suitable recipient patient to the best available organ, capable of rapid resumption of life-sustaining function in the recipient patient. This has only been possible by amassing a good understanding of the potential effects of hypoxic injury on donated organs, and how to prevent these by applying organ preservation. This review sets out the history of organ preservation, how applications of hypothermia have become central to the process, and what the current status is for the range of solid organs commonly transplanted. The science of organ preservation is constantly being updated with new knowledge and ideas, and the review also discusses what innovations are coming close to clinical reality to meet the growing demands for high quality organs in transplantation over the next few years.     

2017年呼吸疾病主要进展

2017年,国内外学者在呼吸系统疾病的临床和基础领域均进行了深入研究,不仅对相关指南进行了更新,并且针对一些临床热点、难点问题达成专家共识,现就2017年呼吸疾病相关进展作一简单介绍。     

家族性噬血细胞性淋巴组织细胞增生症1例并文献复习

目的加强对家族性噬血细胞性淋巴组织细胞增生症（familially hemophagocytic lymphohistiocytosis,FHL）的认识。方法报道确诊为FHL的新病例1例,结合国内外报道的FHL的病例,对该病的临床特点进行汇总分析。结果FHL2常与PRF1基因突变相关,约20%~40%的患者存在穿孔素基因突变。结论对于有阳性家族史,基因诊断明确,应尽早行化疗或者造血干细胞移植。若无家族史,未发现与继发性HLH相关的原发病因,可考虑行基因筛查以明确是否存在FHL的可能。     

COVID‐19 and COVID‐19 vaccine‐related dermatological reactions: An interesting case series with a narrative review of the potential critical and non‐critical mucocutaneous adverse effects related to virus,therapy, and the vaccination

This narrative review article was conducted to lay out a summarized but exhaustive review of current literature over mucocutaneous manifestations in 4 dimensions of SARS‐CoV‐2 pandemic: virus itself, treatment‐related, vaccine‐induced, and alteration of chronic dermatologic diseases following infection. Virus and vaccine‐related were mainly self‐limited and non‐severe. Treatment‐related reactions could be life‐threatening.     

Burkitt's lymphoma of maxillary gingiva: A case report

Burkitt's lymphoma(BL) is an aggressive form of nonHodgkin's B-cell lymphoma with three variants namely endemic, sporadic, and immunodeficiency-associated types. It is endemic in Africa and sporadic in other parts of the world. While the endemic form is widely reported to occur in early childhood and commonly involves the jaw bones, the sporadic form typically presents as an abdominal mass. This presentation reports a rare case of sporadic form of BL clinically manifesting as a generalized gingival enlargement in an immunocompetent adult male which demonstrated an aggressive behavior. The patient reported with a prominent anterior gingival swelling of 6 mo duration which slowly enlarged in size and associated with multiple lymph node involvement. Microscopic examination of the lesion using H, E and immunohistochemical diagnosis confirmed the diagnosis as BL. The patient succumbed to the disease before any therapy could be instituted. Since a wide array of causes can be attributed to gingival enlargements, it is necessary to consider malignancies as one of the important differential diagnosis so as to facilitate the need for appropriate diagnosis and prompt treatment.     

Real‐life effectiveness of biological therapies on symptoms in severe asthma with comorbid CRSwNP

BackgroundWe aimed to evaluate the effectiveness of different antibody therapies on nasal polyp symptoms in patients treated for severe asthma.MethodsWe performed a retrospective analysis of patients with severe asthma and comorbid CRSwNP who were treated with anti‐IgE, anti‐IL‐5/R or anti‐IL‐4R. CRSwNP symptom burden was evaluated before and after 6 months of therapy.ResultsFifty patients were included hereof treated with anti‐IgE: 9, anti‐IL‐5/R: 26 and anti‐IL‐4R: 15 patients. At baseline median SNOT‐20 was similar among groups (anti‐IgE: 55, anti‐IL‐5/R: 52 and anti‐IL‐4R: 56, p = 0.76), median visual analogue scale (VAS) for nasal symptoms was 4, 7 and 8 (p = 0.14) and VAS for total symptoms was higher in the anti‐IL‐4R group (4, 5 and 8, p = 0.002). After 6 months SNOT‐20 improved significantly in all patient groups with median improvement of anti‐IgE: −8 (p < 0.01), anti‐IL‐5/R: −13 (p < 0.001) and anti‐IL‐4R: −18 (p < 0.001), with larger improvement in the anti‐IL‐4R group than in anti‐IgE (p < 0.001) and anti‐IL‐5/R (p < 0.001) groups. VAS nasal symptoms improved by median anti‐IgE: 0 (n.s.), anti‐IL‐5/R: −1 (p < 0.01) and anti‐IL‐4R: −3 (p < 0.001), VAS total symptoms by anti‐IgE: −1 (n.s.), anti‐IL‐5/R: −2 (p < 0.001) and anti‐IL‐4R: −2 (p < 0.001).ConclusionsTreatment by all antibodies showed effectiveness in reducing symptoms of CRSwNP in patients with severe asthma, with the largest reduction observed in anti‐IL‐4R‐treated patients.     

Recalcitrant giant orf recurrence after amputation: A case report and review of the literature

Orf is caused by a parapoxvirus. We present a recurrent, giant digital orf case in a female patient with a history of hairy cell leukemia. In spite of shave excision, the lesion progressed and recurred after digital amputation. Treatment with topical imiquimod cream and systemic subcutaneous interferon alfa‐2a was successful.