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A decrease in the percentage of CD3+ cells is correlated with clinical improvement during plasmapheresis in patients with myasthenia gravis.

Plasmapheresis not only removes circulating antibodies but also modulates cellular immunity, including lymphocyte subsets. To investigate the effect of double-filtration plasmapheresis (DFPP) on the ratio of lymphocyte subsets in patients with myasthenia gravis (MG), we examined the percentages of B-cells, T-cells, T helper (Th) cells, T suppressor (Ts) cells, natural killer (NK) cells, NKT cells, and Th/Ts ratio before and after a single DFPP session and after a course of DFPP. A total of 26 patients were recruited; their peripheral blood lymphocyte subsets were assayed using flow cytometry.

MYASTHENIA GRAVIS: MEDICAL ASPECTS.

The diagnosis of myasthenia gravis is often difficult and calls for a broader use of pharmacological and electrodiagnostic tests. The decamethonium-edrophonium test, which has proved superior to other procedures for this purpose, is based on neurophysiological principles and depicts the behaviour of the neuromuscular junction. A state of resistance to depolarizing agents in the limited form of myasthenia and a state of a non-depolarizing (competitive) block in advanced cases has been shown. This test has demonstrated that the neuromuscular defect exists throughout the skeletal musculature, inc

Patients with normocalcemic versus hypercalcemic hyperparathyroidism: What's really the difference?

INTRODUCTION: Primary hyperparathyroidism (HPT) is typically characterized by elevated parathyroid hormone (PTH) and hypercalcemia; however, … to present with hyperplasia (43.3 ​%, p ​< ​0.001). More patients with normocalcemic HPT underwent thymectomy compared to patients with hypercalcemia (39.3 ​% versus 20.8 ​% respectively, p ​< ​0.001). 92.0 ​% of normocalcemic patients achieved cure post-operatively, which was significantly less than in the hypercalcemic group (97.0 ​%, p ​= ​0.02). The 8.0 …

Risk factors and prognostic factors of pleural metastases in thymic epithelial tumors: A narrative review.

INTRODUCTION: Pleural metastases are common in thymic epithelial tumors (TET), which include pleural recurrence and stage IVa at initial dia … ecreased risk for pleural dissemination associated with Video-Assisted Thoracoscopic Surgery (VATS) thymectomy. In patients with stage IVa at diagnosis group, three of 7 papers mentioned that resection influenced the prognosis. Two papers discussed the number of nodules in the pleura but arrived at different conclusions. One paper suggested that patients old …

Enabling immune checkpoint blockade efficacy in T-lymphopenia by restoring CD8 T cell dynamics with IL-7 cytokine therapy.

INTRODUCTION: T-lymphopenia (TLP) is a frequently observed condition in cancer patients, often exacerbated by conventional chemo/radiotherapy, which impairs the efficacy of subsequent immune checkpoint blockade (ICB) therapy. This study aimed to understand the impact of TLP on ICB responsiveness and explore potential therapeutic strategies to enhance antitumor immunity. METHODS: To investigate ICB responsiveness depending on the severity of TLP, first, we established TLP mouse models that mimic clinically observed mild and severe TLP through thymectomy and anti-Thy1-induced peripheral T cell …

CD4+ T Cells of Myasthenia Gravis Patients Are Characterized by Increased IL-21, IL-4, and IL-17A Productions and Higher Presence of PD-1 and ICOS.

Myasthenia gravis (MG) is an autoimmune disease mediated by autoantibodies predominantly against the acetylcholine receptor (AChR). Specific … ression of the chemokine and the costimulatory receptors. Thymocytes obtained from patients who had thymectomy were also analyzed. IL-21, IL-4, IL-10, and IL-17A productions in CD4+ T cells were increased in AChR-MG compared to those in healthy controls. IS treatment enhanced IL-10 and reduced IFN-γ production in AChR-MG patients compared to those in IS-nega …

Myasthenia gravis and pregnancy: anaesthetic management--a series of cases.

BACKGROUND AND OBJECTIVE: Myasthenia gravis is an autoimmune neuromuscular disease, usually affecting women in the second and third decades. … ); and epidural block was performed in three patients (75%). One patient underwent an uncomplicated thymectomy under general anaesthesia during pregnancy and, in the postpartum period, there was a myasthenic crisis in another patient. Concerning the three patients without a myasthenia gravis diagnosis at partum, one woman already being followed for presentin …

Total parathyroidectomy without autotransplantation for renal hyperparathyroidism: experience with a qPTH-controlled protocol.

BACKGROUND: Controversy regarding the optimal surgical treatment for secondary hyperparathyroidism (sHPT) continues. Subtotal parathyroidectomy (PTX) with a small remnant and total parathyroidectomy with autotransplantation prevail, although impaired by considerable recurrence rates. Concerns about postoperative management and long-term supplementation prevent broader acceptance of total parathyroidectomy without autotransplantation. MATERIALS AND METHODS: The standardized surgical procedure with intraoperative PTH assessment (qPTH) included cervical thymectomy, histological proof of four par…

[Clinical features of thymic neuroendocrine carcinoma: analysis of 21 cases].

OBJECTIVE: To improve the understanding of thymic neuroendocrine carcinoma (TNC) by retrospective analysis of the clinical data of 21 cases. METHODS: The clinical data of 21 patients with TNC treated in the Peking Union Medical College Hospital from 1998 to 2010 were retrospectively analyzed. RESULTS: There were 12 males and 9 females, with onset age ranging from 13 to 67 years and the mean age of 43 years. The clinical manifestation was diverse, in which the malaise of chest and back accounted for 57.1% (12/21), Cushing's syndrome 33.3% (7/21), multiple endocrine neoplasia type 1 accounted

Invasive thymoma with paraneoplastic retinopathy.

A 60-year-old man was admitted for progressive visual loss in both eyes. He was diagnosed with retinopathy and chest computed tomography revealed an invasive thymoma. In western blot analysis, serum autoantibodies against recoverin, photoreceptor-specific calcium-binding protein, and heat shock cognate protein 70 which were identified as the cause of cancer-associated retinopathy (CAR). Immunofluorescence staining showed that thymoma cells also expressed recoverin. These observations strongly suggested that similar pathogenesis of CAR was involved in the presented case.

Delayed kinetics of T lymphocyte anergy and deletion in lpr mice.

The Fas/APO-1 (Fas) antigen is a cell surface protein that mediates apoptosis and belongs to the tumor necrosis factor receptor family. The lymphoproliferative (lpr) anomaly in mice results from a retroviral disruption within the fas gene. Mice that are homozygous for the lpr mutation accumulate large numbers of T lymphocytes and exhibit an autoimmune syndrome resembling systemic lupus erythematosus. A possible explanation for this process is that in the absence of Fas antigen, lpr T cells may be resistant to normal peripheral deletional signals. The bacterial superantigen staphylococcal enter

The behaviour of carcinoma of the large bowel in man following transplantation into immune deprived mice.

The growth of carcinoma of the human large bowel was studied in the first 2 passages in immune deprived mice. The tumours were obtained from large bowel resections on 3 people. There was a strong histological similarity between the patient's tumour and the tumour that grew subcutaneously in the mice 2-8 months after implantation. One dissimilarity observed was a higher mitotic index in some of the tumours growing in the immune deprived animals. In the second passage of the bowel tumours, cells were implanted into groups of 8-10 animals in the following sites: subcutaneous, intramuscular, intra

From Traditional to Targeted Immunotherapy in Myasthenia Gravis: Prospects for Research.

Treatment of Myasthenia Gravis (MG) is still based on non-specific immunosuppression. Long-term high dose corticosteroids is still a major cause of side effects, in young as well as in elderly patients in whom comorbidities further increase the burden of chronic immunosuppression. Moreover, awareness of the limits of traditional therapies has led to the concept of "refractory MG." The therapeutic approach to MG is therefore progressively evolving from the classic combination of corticosteroids and immunosuppressive drugs to new biological compounds targeting different immunopathological steps.

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… Extension of the clinical observation up to 5 years still showed benefit from thymectomy and prednisone in non-thymomatous MG, albeit the patients' sample was small ( 17 ). … At present, thymectomy is recommended for antiAChR-positive MG, increasingly performed with non-invasive techniques ( 18 , 19 ). A further observation emerging from the above studies is that, even after thymectomy, MG still requires corticosteroids and immunosuppressive drugs for several years. …

Thymic TFH cells involved in the pathogenesis of myasthenia gravis with thymoma.

Follicular helper CD4+ T (TFH) cells are the specialized providers of B cell help in germinal centers (GCs). Formation of GCs in thymi is the primary thymi characteristic in MG patients. TFH cells are involved in the pathogenic process of many autoimmune diseases including systemic lupus erythematosus, rheumatoid arthritis and autoimmune thyroid disease. The role thymic TFH cells played in MG with thymoma has not been elucidated. Here, we analyzed surface markers CXCR5, Bcl-6, ICOS and IL-21 on TFH cells in thymus derived from thymoma patients with ocular MG (OMG), generalized MG (GMG) or with

Thymic epithelial tumours: from basic principles to individualised treatment strategies.

Thymic epithelial tumours represent a wide range of anatomical, clinical, histological and molecular malignant entities that may be aggressive and difficult to treat. The histopathological classification distinguishes thymomas from thymic carcinomas. Thymomas may be associated with autoimmune disorders. The management of thymic epithelial tumours is a paradigm of co-operation between clinicians, surgeons and pathologists, from establishing the diagnosis to organising the multimodal therapeutic strategy. Surgery is the mainstay of the curative-intent treatment, as complete resection represents

Clinical correlates with anti-MuSK antibodies in generalized seronegative myasthenia gravis.

The term seronegative myasthenia gravis (SNMG) refers to the generalized disease without detectable anti-acetylcholine receptor (anti-AChR) antibodies. In these patients, IgG antibodies against the muscle-specific kinase (MuSK) have been described, which reduced agrin-induced AChR clustering in vitro. We have assayed anti-MuSK antibodies in 78 patients with SNMG, who have been followed for many years in our Institution. Here we describe the clinical phenotype of the 37 patients whose results were positive on this assay. MG with anti-MuSK antibodies was characterized by a striking prevalence of

Effect of clinicopathologic features on survival of patients with thymic carcinomas and thymic neuroendocrine tumors: A population-based analysis.

BACKGROUND: Thymic carcinomas (TCs) and thymic neuroendocrine tumors (TNETs) are aggressive cancers with poor survival outcome and limited investigation. This study is to investigate clinicopathologic features on TC and TNET patients' prognosis of a large cohort. MATERIALS AND METHODS: The Surveillance, Epidemiology, and End Results database were used to identify a total of 362 TC and TNET patients with documented clinicopathologic features we investigated. The characteristics and overall survival of the TC and TNET patients were studied. RESULTS: Two hundred and forty TC and 122 TNET patien

Postoperative radiotherapy of patients with thymic epithelial tumors (TET): a retrospective analysis of outcome and toxicity.

PURPOSE: The purpose of this study was to evaluate postoperative radiotherapy regarding outcome and toxicity in patients with thymic epithelial tumors (TET) after surgery. MATERIALS AND METHODS: We retrospectively analyzed medical records of 41 patients with TET treated with postoperative radiotherapy at our institution between 1995 and 2012. The impact of prognostic factors (e.g., Masaoka stage, histological subtype) was investigated and radiation-related toxicity was assessed. RESULTS: Median age was 59.8 years and median follow-up was 61 months. In 24.4 %, TETs were associated with parane

Surgical resection of thymoma still represents the first choice of treatment.

OBJECTIVE: The aim of this study was to analyze the clinicopathological factors, treatment strategies and survival rates after surgical resection of thymoma. METHODS: Between 12/1997 and 5/2010, 42 patients underwent surgical resection of the thymus. The presence of a thymoma was determined by histological examination in 23 patients, while patients with hyperplasia of the thymus (n = 19) were excluded from further analysis. RESULTS: Myasthenia gravis coexisted in 9/23 (39.1%) patients. Thymomas were classified according to the Masaoka staging system (I: n = 6 [26.1%], IIa: n = 7 [30.4%], IIb