重症肌无力症的白细胞和淋巴细胞数

为评价重症肌无力患者胸腺切除后是否导致淋巴样细胞数降低,作者将80例重症肌无力病人分为2大组,即胸腺切除组和非胸腺切除组。胸腺切除组50例全部为中度或重度的全身性重症肌无力(据Osserman的分类),病理学再分为三个小组:①胸腺增生23例;②胸腺瘤15例;③胸腺退化12例,其中4例有滤泡生发中心。30例非胸腺切除者为第一对照组,150例各种疾患而非重症肌无力的成人为第二对照组。胸腺切除组50例病者在术前的淋巴样细胞数比术后5.5±5年显著为高,而白细胞数在术前后无差别。50例病人胸腺切除前的白细胞和淋巴样细胞数与30例     

重症肌无力的诊断与外科治疗

重症肌无力（MG）是一种自身免疫性疾病,由于抗体作用在神经肌肉接点使功能性乙酰胆碱受体数量减少,从而导致神经肌肉传导障碍。病理学研究表明,67%以上的重症肌无力患者胸腺异常。以淋巴滤泡增知最为常见。据报道,10%～25%的重症肌无力患者伴有胸腺瘤,而30%～60%的胸腺瘤患者伴有重症肌无力。可见胸腺瘤是重症肌无力的重要病因,切除胸腺可有很好的疗效。本文就该病的诊断与外科治疗作一概述。     

胸腺生发中心及Bcl-2蛋白表达水平与获得性自身免疫性重症肌无力的关系

目的探讨胸腺生发中心及细胞凋亡相关基因Bcl-2蛋白表达水平与获得性自身免疫性重症肌无力的关系。方法将31例重症肌无力患者的胸腺标本作为观察组，25例先心病胸腺标本作为正常对照组，分别行SP和HE染色，检测生发中心和Bcl-2的表达水平。结果正常对照组无生发中心发现。而观察组Bcl-2表达水平明显高于对照组，且多为高表达者，两者对比差异极显著(P＜0.01)。结论生发中心的出现和数目增多是胸腺增生的一个重要的形态学标志，Bcl-2高表达提示增生胸腺中免疫细胞的凋亡受到抑制，可能是获得性自身免疫性重症肌无力发生的重要机制之一。     

31例老年重症肌无力患者胸腺病变的CT和病理对照研究

目的探讨纵隔CT对老年重症肌无力（MG）患者胸腺异常的诊断价值。方法对31例行胸腺切除治疗的老年MG患者的胸部CT和胸腺病理进行对照研究。结果31例MG患者CT诊断胸腺异常23例（74%）,其中胸腺增生7例（23%）,胸腺瘤16例（51%）,正常胸腺8例（26%）;病理诊断胸腺异常29例（94%）;其中胸腺增生17例（55%）,胸腺瘤12例（39%）,正常胸腺2例（6%）。CT诊断胸腺增生7例,异常率为41%,CT诊断胸腺瘤12例,异常率为100%,CT检查与病理检查结果比较,差异有统计学意义（P〈0.05）。CT检查对呈斑点状、索条状胸腺增生易出现漏诊。结论老年MG患者94%有胸腺异常,胸腺瘤占39%,但CT检查对鉴别胸腺增生与正常胸腺及胸腺瘤的价值有限。     

梅毒性淋巴结炎临床病理诊断

目的探讨梅毒性淋巴结炎的临床病理学表现和鉴别诊断,以提高对此病的认识。方法对5例梅毒性淋巴结炎的临床病理资料、实验室结果进行回顾分析。结果5例梅毒性淋巴结炎中,4例表现为腹股沟淋巴结无痛性肿大,1例伴颈部淋巴结肿大,淋巴结直径1．5cm～3．8cm,平均2．5cm。病理学改变主要包括：①淋巴结包膜及包膜外纤维组织增生,伴慢性炎细胞浸润,使包膜增厚;②淋巴滤泡反应性增生,滤泡间以浆细胞为主的炎细胞浸润;③闭塞性血管内膜炎;④小血管周围炎;⑤上皮样组织细胞增生形成小肉芽肿,有时可见郎罕氏多核巨细胞;⑥淋巴结边缘窦内单核样B细胞增生;⑦淋巴滤泡内外小灶碎屑样坏死。梅毒血清学试验均阳性。结论梅毒性淋巴结炎组织学上有相对特征性和提示性,结合血清学检查可明确诊断,病理医师提高对该病的认识可减少漏诊。     

以肾病综合征为首发症状的多中心型Castleman病一例

Castleman病又称血管滤泡性淋巴组织增生或巨大淋巴结增生，是一种少见的慢性淋巴组织不典型增生性疾患，而多中心型Castleman病（浆细胞型）更为罕见。     

滤泡性细支气管炎研究进展

滤泡性细支气管炎是一个组织病理学诊断,以细支气管壁伴有生发中心的淋巴样滤泡增生为特征.常与慢性感染和炎症性气道疾病,例如囊性纤维化、支气管扩张、慢性误吸、结缔组织疾病、包括获得性免疫缺陷综合征在内的免疫缺陷综合征有一定的相关性.在胸部影像学主要表现为两肺小结节或网状阴影,伴有胸腔内淋巴结肿大,结节和毛玻璃样影可以两肺弥漫性分布,主要位于支气管血管周围.结合临床表现和影像学表现可以用于滤泡性细支气管炎的诊断.治疗缺乏特效治疗手段,一般预后较好.     

滤泡性细支气管炎研究进展

滤泡性细支气管炎是一个组织病理学诊断,以细支气管壁伴有生发中心的淋巴样滤泡增生为特征.常与慢性感染和炎症性气道疾病,例如囊性纤维化、支气管扩张、慢性误吸、结缔组织疾病、包括获得性免疫缺陷综合征在内的免疫缺陷综合征有一定的相关性.在胸部影像学主要表现为两肺小结节或网状阴影,伴有胸腔内淋巴结肿大,结节和毛玻璃样影可以两肺弥漫性分布,主要位于支气管血管周围.结合临床表现和影像学表现可以用于滤泡性细支气管炎的诊断.治疗缺乏特效治疗手段,一般预后较好.     

电视胸腔镜胸腺切除术治疗重症肌无力15例疗效观察

目的 探讨电视胸腔镜（VATS）胸腺切除治疗重症肌无力（MG）的可行性和治疗效果.方法 15例MG患者采用VATS行胸腺切除及前纵隔脂肪清扫术,观察围手术期情况及早期临床疗效.结果 除1例中转开胸外,余14例均在VATS下完成手术,无围术期死亡.按照Monden标准,肌无力症状消失5例,改善8例,无变化2例,有效率为86.7％（13/15）.结论 VATS胸腺切除和前纵隔脂肪组织清扫治疗MG安全有效.     

胸腺瘤伴重症肌无力患者瘤组织中Bcl-2和Fas的表达水平

目的 探讨凋亡相关基因bcl-2、Fas在胸腺瘤伴重症肌无力患者瘤组织中的表达状况及其临床意义。方法经手术治疗的25例胸腺瘤伴重症肌无力患者的肿瘤组织标本为病例组，25例先天性心脏病患者手术时切取的正常胸腺组织标本为对照组，通过免疫组化的方法检测两组标本中Bcl-2和Fas蛋白的表达水平。结果胸腺瘤中Bcl-2及Fas表达水平均显著高于对照组，经Ridit分析两者差异均有统计学意义（U值分别为2．645、3．200，P均〈0．05），但Bcl-2和Fas的表达水平与胸腺瘤患者的重症肌无力Ossermen分型、术前病程、年龄及性别等临床因素均无显著相关。结论 Bcl-2和Fas在胸腺瘤伴重症肌无力的发病中可能具有重要作用。     

Factors influencing improvement and remission rates after thymectomy for myasthenia gravis.

BACKGROUND: Thymectomy has become an accepted option in the treatment of myasthenia gravis (MG). However, the optimal selection of patients for surgery remains controversial. OBJECTIVE: The objective of the study is to define factors influencing improvement and remission rates after thymectomy for MG. METHODS: We retrospectively reviewed our experience in the surgical management of patients with MG over the last 20 years, and analyzed if patient's age, sex, preoperative Osserman Stage, thymic histology, duration of symptoms, or radiological findings influenced outcome. RESULTS: There were 25 women and 10 men. Most of them presented with Osserman stage IIA (n = 12) and stage IIB (n = 17). Hyperplasia of the thymus was observed in 15 patients (43%), thymoma in 12 (34%), and atrophic or normal thymus in 8 (23%). Computed tomography (CT) had a positive predictive value of 91% in detecting thymoma and of 80% in detecting thymic hyperplasia. Over a mean follow-up of 96 months, MG improved markedly in 26 patients. Postoperative improvement was significantly greater in patients with advanced MG (stage IIB, III and IV), whereas a higher rate of remission occurred in patients with mild MG (stage I and IIA), and in those with thymic hyperplasia. No relation was observed between outcome and age, sex, duration of symptoms and abnormal CT findings. CONCLUSIONS: The beneficial effects of thymectomy are characterized by postoperative clinical improvement in most patients with moderate to severe preoperative myasthenic involvement and by a higher remission rate in patients with thymic hyperplasia and/or mild myasthenic symptoms.     

血清Ryanodine受体抗体检测对胸腺瘤并重症肌无力的诊断价值

目的 探讨血清Ryanodine受体抗体（RyR-Ab）对胸腺瘤并重症肌无力（MG）的诊断价值。方法 选择经病理检查证实为胸腺病变并MG的患者35例,其中胸腺瘤并MG患者（MGT组）17例、胸腺增生并MG患者（MGH组）12例、胸腺萎缩并MG患者（MGA组）6例,同时选择胸腺正常的MG患者（NTMG组）27例,健康对照者（NC组）50例;采用ELISA法检测其血清RyR-Ab。结果 MGT组血清RyR-Ab阳性率为76．5％,MGH组为8．3％,MGA组为16．6％,NTMG组为0,NC组为0。结论MGT患者血清RyR-Ab阳性率较高,检测血清RyR-Ab有助于MGT的诊断。     

Microscopic-sized "microthymoma" in patients with myasthenia gravis

BACKGROUND: In 2005, Cheuk et al reported two patients with microscopic-sized thymomas and proposed the term microthymoma to distinguish it from the nodular hyperplasia of thymic epithelium, so-called microscopic thymoma. Here, we present microthymomas that were found in 196 patients with myasthenia gravis (MG) who had undergone thymectomy. MATERIALS AND METHODS: Thymic tissues in 196 patients with MG who underwent thymectomy or thymothymomectomy were examined. Of these patients, 73 patients had thymoma indicated by CT before surgery, and the other 123 patients had no mediastinal tumors. From the resected thymic tissues, an average of 14 hematoxylin-eosin-stained sections (range, 4 to 55 sections) were prepared for microscopic examination. The histologic type of the thymoma was classified according to the World Health Organization (WHO) classification. RESULTS: From the 196 patients, we found three microthymomas in 3 patients (1.5%). While these three tumors could not be seen grossly in pathology section, they were found microscopically (range, 2 to 4 mm). The histologic subtype according to the WHO classification system was B1 in one patient and B2 in two patients. CONCLUSION: Microthymoma was found in 3 of 196 patients (1.5%) with MG. Microthymoma might exist in thymus of patients with MG, even in patients who have no thymoma indicated by CT.     

早发型与晚发型重症肌无力患者的临床特点分析

目的 分析早发型与晚发型重症肌无力(MG)的临床特点.方法 回顾分析同期住院的819例MG患者资料,比较早发型及晚发型MG组患者的构成情况、首发症状、临床分型、新斯的明试验及重复电刺激阳性率、合并甲状腺功能异常及胸腺病理类型等临床特点.结果 MG发病仍以早发型人群为主,占77.66%,两组男女比例分别为1∶1.08及1∶0.81(P＞0.05),皆以ⅡB型为主(41.51%比51.37%,P＜0.05),MG患者新斯的明试验和重复电刺激阳性率差异无统计学意义.甲状腺功能异常在早发型MG组较晚发型MG组多见(P＜0.05),早发型MG组患者胸腺异常以增生为多见(达67.57%,P＜0.01),而晚发型MG组患者胸腺异常则胸腺瘤和胸腺增生比例相当(分别为48.68%和47.37%).结论 早发型与晚发型MG患者在临床分型、合并甲状腺功能异常及胸腺病理方面具有不同的特点,值得注意.

Abstract：

objective To compare the clinical characteristics of early-onset and late-onset myasthenia gravis(MG).Methods A total of 819 MG patients admitted in our department during the sanle period were reviewed retrospectively.The patients with MG were divided into two groups by the age of onset, which were the early-onset MG(＜49 years)and late-onset MG(≥50 years).Several clinical features were compared in the two groups including the percentage, initial symptoms, MG types,the positive rates of neostigmine test and repetitive nerve stimulation, thyroid function and thymie abnormalities. Results The more common onset age of MG was 0-49 years(early-onset).In both groups the male and female ratio was presented as the most common type in both groups(41.51%and 51.37% respectively in early-onset and late-onset groups).Type Ⅰ was more common in early-onset group while typeⅡB Was more common in lateonset group(P＜0.05).There Was no significant difference in the positive rates of neostigmine test and repetitive nerve stimulation between the two groups.The thyroid function alnormality ratio was higher in early-onset group. Hyperplastic thymus was common in early-onset group(67.57%)while both hyperplastic thymus and thymoma were common in late-onset group(48.68% and 47.37%).Conclusions The clinical characteristics of early-onset and late-onset myasthenia gravis(MG)Was different in MG type, the positive rate of thyroid function abnormalities and thymic pathologic type.     

Increased frequency of thymic T follicular helper cells in myasthenia gravis patients with thymoma

Background

To investigate the presence of T follicular helper (TFH) cells and their associated molecules in myasthenia gravis (MG) patients with thymoma.

Methods

TFH cells are detected in thymus around the thymoma region of 50 patients and atrophic thymus in 10 patients as control. The percentage of TFH cells among CD4⁺ T cells and the expression level of surface markers CXC chemokine receptor 5 (CXCR5), inducible co-stimulator (ICOS), programmed cell death 1 and the cytoplasmic marker B cell lymphoma 6 (Bcl-6) were analyzed by immunohistochemistry (IHC) staining, immunofluorescence (IF) and western blotting (WB).

Results

Higher percentage of thymic TFH cells was found in MG patients with thymoma compared with both thymoma patients without MG and control group. The expression levels of the four markers in thymoma of MG patients were significantly higher than thymoma patients without MG and control group. No significant difference was found in the levels of programmed cell death 1 (PD-1) and Bcl-6 between thymoma patients without MG and the control, while the levels of CXCR5 and ICOS in thymoma patients without MG were higher than control group.

Conclusions

These results suggested thymic TFH cells might involve in the pathogenesis of MG with thymoma. However, it needs further study to test if the inhibition of the function of TFH cells could effectively alleviate the severity of MG.     

Thymic histology in myasthenia gravis.

Histological features of resected thymuses of 18 patients with myasthenia gravis were evaluated. Thymoma were seen in 16.6% of the patients. In the non-thymomatous group, thymic hyperplasia with follicle formation was seen in 33.3%, involuted thymus with occasional lymphoid follicle formation in 11.1%, thymitis with B cell infiltration in 16.6%, involuted thymus in 11.1% and normal thymus in 11.1% of patients. Immuno-histological staining for B and T lymphocytes delineated the group labelled as thymitis with B cell infiltration. Mast cell and eosinophils were frequently seen in thymuses with thymic hyperplasia with follicle formation and thymitis with B cell infiltration. Thymic hyperplasia with follicle formation was more frequent in young patients and these patients had better prognosis.     

老年重症肌无力患者浆细胞样树突细胞与调节性T细胞的变化与临床意义

目的 研究老年重症肌无力(MG)患者淋巴细胞浆细胞样树突细胞(pDC)、调节性T细胞(Treg)、血清白细胞介素(IL)-4、γ-干扰素(IFN)-γ 的含量变化及临床意义.方法 老年MG患者112例按照影像学及病理学诊断划分为无胸腺受累组60例与胸腺受累组52例,同期健康体检人员68例为对照组.上述3组应用流式细胞术...     

A case of dermatomyositis associated with thymic abnormalities]

A case of dermatomyositis (DM) associated with thymic cyst with lymphoid follicle formations has been reported. A 57-year-old man developed polyarthralgia, muscle weakness, heliotrope rash and Gottron's sign. Laboratory findings showed elevated values of creatine-phosphokinase, aldolase and transaminase. He was diagnosed as DM and was treated with prednisolone 50 mg/day. Muscle weakness was improved immediately, although rash persisted unchangeably. He was examined for coexistence of malignant tumor which had been reported frequently as associated with DM. Enlargement of thymus was found by computer tomography and then thymectomy was performed. In his thymus, lymphoid follicle formations, which are as often encountered in other autoimmune diseases, were found. So far, the beneficial effect of thymectomy on the improvement of immunological abnormalities not only in patients with myasthenia gravis but also in patients with other autoimmune diseases has been recognized. In this case, intractable rash in DM improved after thymectomy. Further study needs to clarify the relationships between DM and thymic abnormalities.     

The thymic theme of acetylcholinesterase splice variants in myasthenia gravis

Cholinergic signaling and acetylcholinesterase (AChE) influence immune response and inflammation. Autoimmune myasthenia gravis (MG) is mediated by antibodies to the acetylcholine receptor and current therapy is based on anti-AChE drugs. MG is associated with thymic hyperplasia, showing signs of inflammation. The objectives of this study were to analyze the involvement of AChE variants in thymic hyperplasia. We found lower hydrolytic activities in the MG thymus compared with adult controls, accompanied by translocation of AChE-R from the cytoplasm to the membrane and increased expression of the signaling protein kinase PKC-betaII. To explore possible causal association of AChE-R changes with thymic composition and function, we used an AChE-R transgenic model and showed smaller thymic medulla compared with strain-matched controls, indicating that AChE-R overexpression interferes with thymic differentiation mechanisms. Interestingly, AChE-R transgenic mice showed increased numbers of CD4(+)CD8(+) cells that were considerably more resistant in vitro to apoptosis than normal thymocytes, suggesting possibly altered positive selection. We further analyzed microarray data of MG thymic hyperplasia compared with healthy controls and found continuous and discrete changes in AChE-annotated GO categories. Together, these findings show that modified AChE gene expression and properties are causally involved in thymic function and development.