指状突树突细胞肉瘤2例临床病理观察

目的 探讨指状突树突细胞肉瘤(IDCS)的临床病理特征、免疫表型及其鉴别诊断.方法 报道2例少见的指状突树突细胞肉瘤并行文献复习.结果 2例患者临床症状均为局部无痛性淋巴结肿大,结外皮肤受累.镜下见肿瘤组织边界不清楚呈巢片状,部分为束状、旋涡状排列;瘤细胞弥漫分布,呈梭形、圆形、椭圆形, 胞质丰富透亮或轻度嗜酸性,核卵圆形、空泡状,核仁清楚,部分有核沟,有异型性,可见核分裂象.肿瘤通常无坏死,周边常有较多淋巴细胞、浆细胞浸润.免疫组化示瘤细胞vimentin和S-100(+),CD68(+)/(-),Ki-67部分(+);CD21、CD35,HMB45、melanA、CD4和CgA(-).结论 指状突树突细胞肉瘤是一种罕见的组织细胞和树突细胞的恶性肿瘤,确诊依靠病理检查及免疫组化,必要时可电镜辅助诊断.临床应与其他类型的组织细胞肿瘤、恶性黑色素瘤和软组织肿瘤等鉴别.肿瘤单发以手术完整切除为主,可能治愈;而多发或多器官累及者可手术结合放、化疗,但目前尚无公认有效的标准治疗方案.     

骨原发性组织细胞肉瘤3例临床病理观察

目的 探讨组织细胞肉瘤的临床病理特征、诊断与鉴别诊断及预后.方法 对3例组织细胞肉瘤进行光镜观察及免疫组化检测,并复习相关文献进行讨论.结果 3例患者均为男性,年龄18～41岁;病变分别位于胸椎、上颌骨及股骨远端和胫骨近端.影像学表现均为溶骨性改变伴周围软组织肿块.镜下肿瘤细胞弥漫分布,瘤细胞体积大、圆形或隋圆形,局灶可见梭形细胞,大部分区域瘤细胞黏附性差;瘤细胞胞质丰富、嗜伊红,部分呈泡沫状或充满微空泡,部分瘤巨细胞内可见吞噬的红细胞;核大、圆形或卵圆形,空泡状,可见偏位,部分可见增大的核仁,核分裂易见;亦可见多核瘤巨细胞;背景中常见小淋巴细胞、浆细胞、嗜中性及嗜酸性粒细胞等反应性细胞.免疫组化示肿瘤细胞CD68(KP-1及PGM1)、lysozyme和CD163(+),CK、HMB45、CD3、CD20、CD1a及MPO(-).结论 组织细胞肉瘤是一种罕见的淋巴组织恶性肿瘤,单从组织形态学上难与癌、恶性黑色素瘤、淋巴瘤、恶性纤维组织细胞瘤等相鉴别,诊断与鉴别诊断还依赖于组织形态与免疫组化结果.目前最佳治疗方案不统一,预后多数不佳.     

累及全身淋巴结的滤泡树突状细胞肉瘤临床病理观察

目的 探讨滤泡树突状细胞肉瘤(FDCS)的临床病理学特征、生物学行为、治疗和预后.方法 对1例累及全身多处淋巴结的FDCS进行临床病理学分析、免疫组化染色及EBV编码的小RNA原位杂交检测,并结合文献进行讨论.结果 患者病变累及全身多处淋巴结,伴随全身荨麻疹和关节肿痛.瘤组织排列成束状、席纹状.瘤细胞胞膜欠清,胞质淡伊红染,胞核卵圆形,有异型,核膜清晰,呈空泡状,核仁明显,可见病理性核分裂象2～4个/10HPF.瘤组织中散在少量淋巴细胞、浆细胞和嗜酸性粒细胞.免疫组化瘤细胞clusterin、SNA和vimentin弥漫(+),CD21+35和S-100局部(+),CD1a、desmin、CK8/18和CD68(-).EBER原位杂交(-).结论 滤泡树突状细胞肉瘤是罕见的起源于淋巴结和结外淋巴组织间质细胞的恶性肿瘤,诊断依赖于组织病理学和免疫组化.     

淋巴结指突状树突细胞肉瘤临床病理观察

目的探讨淋巴结原发性指突状树突细胞肉瘤的临床病理特点及鉴别诊断,提高对该肿瘤的诊断水平。方法对2例指突细胞肉瘤进行组织病理学、免疫组化及电镜观察。结果2例肿瘤均位于颈部淋巴结,光镜检查肿瘤组织呈席纹状、旋涡状或杂乱排列,瘤细胞卵圆形、梭形,胞质少,核卵圆形或短梭形,染色质细,少数有核仁,分裂象多见。瘤细胞S-100、CD68及vimentin( ),CD21、CD34、CK、CD45、SMA及HMB45均(-)。电镜下瘤细胞胞质有大量长指状突起,无桥粒连接及Birbeck颗粒。结论指突状树突细胞肉瘤是一种罕见的恶性肿瘤,预后差。诊断主要依靠电镜及免疫组化,并应与滤泡树突细胞肉瘤、朗格汉斯细胞肉瘤、恶性纤维组织细胞瘤、黑色素瘤、梭形细胞癌及其他肉瘤鉴别。     

原发性肝组织细胞肉瘤2例临床病理观察

目的探讨肝组织细胞肉瘤(HS)的临床病理学特征、免疫组化及其鉴别诊断。方法收集2例原发性肝HS的临床和病理资料,进行影像学和光镜观察及免疫组化染色,并复习相关文献。结果 2例HS均为男性,年龄分别为50岁和51岁。患者均表现为右上腹部疼痛不适。影像学示肝右叶占位。镜下肿瘤与周围肝组织分界不清,呈侵袭性生长;肿瘤细胞弥漫分布,体积大、圆形或椭圆形,大部分区域瘤细胞黏附性差;瘤细胞胞质丰富、嗜伊红,部分呈泡沫状或充满微空泡;亦可见多核瘤巨细胞,部分瘤巨细胞内可见吞噬的红细胞;背景中常见小淋巴细胞、浆细胞、中性及嗜酸性粒细胞等反应性细胞。免疫组化示肿瘤细胞CD68、CD163和lysozyme(+);CK、Her Par-1、AFP、HMB45、CD3、CD20、CD1a、CD21、CD35和MPO均(-)。结论肝HS是一种极其罕见的淋巴组织恶性肿瘤,仅从组织形态学上难与滤泡树突细胞肉瘤、恶性黑色素瘤、淋巴瘤、透明细胞型肝细胞癌等相鉴别,诊断与鉴别诊断还依赖于组织形态与免疫组化结果。目前对组织细胞肉瘤尚无统一的确切的治疗方案,预后较差。     

颈部指突状树突细胞肉瘤1例临床病理观察并文献复习

目的探讨指突状树突细胞肉瘤的临床病理特征、诊断要点及鉴别诊断。方法对1例颈部指突状树突细胞肉瘤进行组织病理学和免疫组化染色观察并随访,结合文献进行讨论。结果镜下瘤细胞呈束状、编织状排列,瘤细胞呈胖梭形或椭圆形、胞质稍红染,胞核卵圆形,可呈空泡状,核膜清楚,多数有小核仁,瘤细胞显示轻到中度异型。免疫表型:瘤细胞表达Vimentin和S-100蛋白。结论指突状树突状细胞肉瘤是一种罕见的免疫辅助细胞恶性肿瘤。其诊断依靠组织病理学和免疫组化染色,必要时电镜辅助诊断。治疗以手术切除为主,辅以化疗和(或)放疗,预后差。     

肝炎性假瘤样滤泡树突状细胞肉瘤2例临床病理观察

目的探讨肝炎性假瘤样滤泡树突状细胞肉瘤(FDCS)的临床病理学特征,提高对该肿瘤的诊断水平。方法对2例肝炎性假瘤样滤泡树突状细胞肉瘤的临床资料、组织学形态、免疫组化、原位杂交进行分析,并复习相关文献。结果肿瘤细胞呈结节状排列,炎细胞背景中散在分布多少不等的肿瘤细胞,瘤细胞胞质丰富,呈淡嗜酸性,胞核圆形、卵圆形或梭形,核染色空淡,可见细小的核仁,常见多核或单核瘤巨细胞,病理性核分裂多见。瘤细胞EBER(+)。免疫组化:CD21、CD35和D2-40(+)。结论肝炎性假瘤样滤泡树突状细胞肉瘤是一种罕见的恶性肿瘤,恶性程度和预后具有不确定性。明确诊断需要结合形态学特点、免疫组化及EBER原位杂交等检查,同时要与肝炎性肌纤维母细胞肿瘤、霍奇金淋巴瘤、指突状树突细胞肉瘤、胃肠间质瘤、特别是炎性假瘤相鉴别。     

肌纤维母细胞型恶性间皮瘤临床病理观察

目的 探讨肌纤维母细胞型恶性间皮瘤的病理诊断、鉴别诊断及预后.方法 观察并分析肌纤维母细胞型恶性间皮瘤的病理形态特点、免疫组化染色及电镜,并复习相关文献.结果 本组3例肌纤维母细胞型恶性间皮瘤中仅1例有胸痛,其他临床症状及体征不明显.光镜下见瘤细胞呈弥漫分布,梭形、胖梭形或短梭形,大小略不等,可见核分裂象,部分细胞胞质丰富呈红染或粉染.1例电镜下瘤细胞表面见少量微绒毛,胞质内可见较多张力微丝.免疫组化:肿瘤细胞CK5/6、calretinin、抗间皮抗原、vimentin和actin(pan)均(+).结论 肌纤维母细胞型恶性间皮瘤是一种少见的特殊类型的肉瘤型恶性间皮瘤,其免疫组化及电镜检测有助于诊断.需与恶性孤立性纤维性肿瘤、平滑肌肉瘤、低度恶性肌纤维母细胞肉瘤和纤维肉瘤等鉴别.     

原发性组织细胞肉瘤2例临床病理观察

目的探讨原发性组织细胞肉瘤(HS)的临床病理特征、诊断与鉴别诊断以及治疗与预后。方法对2012—2016年2例原发性HS患者标本进行临床病理学观察,并结合国内外文献进行复习。结果例1,男,38岁,反复低热3月余。检查发现双侧颈前及锁骨上区多枚淋巴结肿大。入院后行淋巴结活检。例2,女,21岁,因右上肢疼痛1月余伴病理性骨折入院。入院后行"右肱骨近端肿瘤刮除植骨内固定术"。镜下大量单核组织样细胞弥漫分布。瘤细胞体积大,胞质嗜酸或空泡状,核呈圆形、卵圆形,异型明显,病理性核分裂象多见。免疫组化:肿瘤细胞CD68、CD163、lysozyme均(+),SMA、desmin、CD3、S-100、CD30、CD20、CD43、EMA、HMB45、CD21/35、Clusterin、Langerin、CD1a均(-)。例2,BRAF V600E(+)。病理诊断:2例均确诊为组织细胞肉瘤,其中1例存在BRAF V600E基因突变。结论组织细胞肉瘤是一种高度恶性的肿瘤,临床表现缺乏特异性,诊断有赖于病理组织形态学及免疫组织化学检查,并应与淋巴造血系统其他恶性肿瘤进行鉴别。针对BRAF V600E基因突变的患者使用靶向药物威罗菲尼治疗取得了一定的疗效,但仍需进一步大规模临床验证。     

眼眶颗粒细胞瘤临床病理观察

目的探讨眼眶颗粒细胞瘤的临床病理学特征及鉴别诊断要点。方法收集1例发生于右侧眼眶的颗粒细胞瘤临床及影像学资料、观察其病理学形态及免疫表型,并复习相关文献。结果患者女性,34岁。以右侧眼球活动受限伴视物模糊3年为主要症状。MRI示右侧眼眶内软组织肿块。镜下观察大部分瘤细胞弥漫生长,细胞边界不清,局部瘤细胞排列成小巢状,瘤细胞体积大,胞质内含有丰富、粗大的嗜酸性颗粒,个别细胞胞质见较大的圆形小滴(嗜酸性小体),局部可见片状泡沫细胞,肿瘤细胞核小,圆形、卵圆形位于中央,个别核增大,核不规则,有轻度异型性,核仁不明显,核分裂象少见,增殖活性低。免疫组化:S-100、CD56、CD68和vimentin(+),α-IN弱(+),Ki-67阳性指数为1%,而CK、EMA、GFAP、nestin、Syn、Cg A、SMA、actin、desmin和HMB45均为(-)。结论眼眶颗粒细胞瘤罕见,需与副节瘤、脑膜瘤、神经鞘瘤、低级别胶质瘤、腺泡状软组织肉瘤等进行鉴别,其确诊需结合临床、组织病理学及免疫组化信息进行综合分析。     

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.     

经颅黑质超声诊断帕金森病的临床评价

目的 通过对帕金森病患者及正常对照组进行经颅超声(transcranial sonography,TCS)检查,结合帕金森病患者的Hoehn Yahr（H Y）分级及帕金森病统一评分量表评分结果,了解我国大陆地区帕金森病患者有无黑质(substantia nigra, SN)回声增强表现。方法 对入选对象进行TCS检查,并对检查结果进行评价。结果 帕金森病患者 SN阳性率明显高于正常对照者（P＜0.05）。帕金森病SN异常组 H Y 分期明显高于帕金森病SN正常组(P＜0.05),表明帕金森病患者SN高回声面积与H Y分期相关。TCS检查对帕金森病的敏感性为80.5%,特异性为79.9%。结论 我国帕金森病患者SN强回声检出率显著高于对照组,说明我国帕金森病患者也存在SN回声增强这一现象,与国内外报道相一致。TCS检查对帕金森病的诊断具有一定的意义,敏感性及特异性较高。