T cell lymphocytosis associated with polymyositis, myasthenia gravis and thymoma

Peripheral T cell lymphocytosis is a rare finding in association with malignant thymomas. In the majority of previous cases, the tumours have behaved aggressively with symptoms arising from local invasion. We describe a patient with ocular myasthenia gravis who presented with a rapidly progressive polymyositis and neuropathy and who was subsequently found to have a thymic mass and a mild T cell lymphocytosis. The thymoma did not give rise to local symptoms and showed no evidence of progression over a 14-month period of follow-up. The possibility of an underlying thymic tumour should be considered in any patient with chronic T cell lymphocytosis if the circulating cells show mature morphology and there is no molecular evidence of monoclonality.     

Increased frequency of euthyroid ophthalmopathy in patients with Graves' disease associated with myasthenia gravis.

We previously showed that myasthenia gravis (MG) has a mild clinical expression when associated with autoimmune thyroid diseases (AITD). In the present study we have investigated the frequency of thyroid-associated ophthalmopathy (TAO) in patients with Graves' disease (GD) associated with MG as compared with GD patients without MG. A total of 418 patients with GD were studied, 31 with MG and 387 without MG. TAO was evaluated by physical examination, exophthalmometry, computerized tomography, and computerized visual fields assessment. The overall prevalence of TAO was similar in GD patients with MG (61.2%) and in those without MG (56.4%). When the analysis was restricted to GD patients with ocular MG, a greater frequency of TAO was found (84.6%), compared with GD patients without MG or with GD patients with generalized MG, although the differences did not reach the statistical significance. GD patients with MG had a significantly greater prevalence (12.9%) of euthyroid ophthalmopathy (clinically overt ophthalmopathy without previous and/or current hyperthyroidism) than those without MG (3.1%; p = 0.003). The results suggest a preferential association between the ocular manifestations of GD and MG, which may be due to immunological cross-reactivity against common autoimmune targets in the eye muscle as well as to a common genetic background.     

Type A thymoma with generalised myasthenia gravis

Thymoma is a very rare tumour arising from thymus in the anterior mediastinum. A case of a spindle cell thymoma with Myasthenia gravis in a 34-year-old female who presented with difficulty in breathing and swallowing with shortness of breath is reported.     

Hyperparathyroidism, chemodectoma, thymoma, and myasthenia gravis.

Of two patients with hyperparathyroidism, one had an associated chemodectoma, and the other had a thymoma and myasthenia gravis. There is a possible relationship to the multiple endocrine neoplasia syndromes.     

重症肌无力胸腺瘤的围术期处理

目的总结重症肌无力（myasthenia gravis,MG）胸腺瘤的围术期处理经验。方法回顾分析本科1990年7月～2008年6月连续手术治疗胸腺瘤合并MG65例患者的临床资料。结果危象预测积分〈12分者,无危象发生;12～17分者危象发生率50.0%;18～23分者危象发生率71.4%;≥24分者危象发生率75.0%。术前危象预测积分≥12分者,术后24h拔除气管插管和气管切开的患者分别为85.7%和61.4%,显著高于危象预测积分〈12分者的20.5%和6.8%。术后肌无力症状完全缓解23例、改善25例,有效率85.7%,改善不明显或加重8例。结论胸腺瘤切除是治疗重症肌无力胸腺瘤的一种较安全而有效的方法。围手术期治疗的重点是防治MG危象。     

胸腺瘤伴重症肌无力外科治疗的研究进展

胸腺瘤是一种起源于胸腺上皮细胞的肿瘤,发病率约为0.2/100 000。重症肌无力是其常见的伴随症状,此类患者可发生肌无力危象进而导致死亡。胸腺切除术已经成为胸腺瘤伴重症肌无力常见的治疗方法。该文针对胸腺瘤伴重症肌无力外科治疗的术前检查、临床诊断与分期、外科手术方式与入路选择和预后等进行综述。     

Severe asthma associated with myasthenia gravis

Severe asthma constitutes a subgroup of approximately 10% of all asthma cases. Approximately one-half of these individuals have a refractory form of the disease in which atopy and T-helper cell 2-skewed immunological response may not be as closely linked to the disease as in other phenotypes of asthma. This suggests that not all asthma is explained by a T-helper cell 2-skewed immunological response, and that other immunological mechanisms may be important in this category of nonatopic asthma. The authors present a case involving a 55-year-old Caucasian man with nonatopic, adult-onset asthma, nonsteroidal anti-inflammatory drug sensitivity and idiopathic urticaria. This individual presented two years following his initial asthma diagnosis with diplopia and mild ptosis, and was subsequently diagnosed with seropositive myasthenia gravis.     

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。     

Intractable ventricular tachycardia in a patient with giant cell myocarditis, thymoma and myasthenia gravis

A 48-year-old man presented with a malignant thymoma in combination with myositis, myasthenia gravis, a giant cell myocarditis and recurrent intractable ventricular tachycardias. Despite various therapies (chemical, electrical and surgical), arrhythmias supervened in the presence of a normal coronary arteriogram. Active myocarditis was believed to be the mechanism of the ventricular tachycardias.     

Thrombotic thrombocytopenic purpura associated with myasthenia gravis

A case of thrombotic thrombocytopenic purpura (TTP) associated with myasthenia gravis (MG) is reported. A 56-year-old woman was admitted to our hospital on April 27, 1988, because of easy fatigue and double vision. She was diagnosed as having myasthenia gravis from her neurological and laboratory findings. On the 14th hospital day, she developed fever, jaundice, hematuria, purpura and consciousness disturbance. Hematological examination revealed marked anemia, thrombocytopenia, fragmented red blood cells, elevated bilirubin and LDH level. Coagulation studies were almost normal. She was diagnosed as having TTP and treated with corticosteroid, antiplatelet agents and plasma exchange. Clinical condition and laboratory findings improved by the 23rd hospital day. It has been reported that TTP is associated with various autoimmune disease such as systemic lupus erythematosus, rheumatoid arthritis, Sj?gren's syndrome or idiopathic thrombocytopenic purpura. However TTP associated with MG appears the first report. This case may suggest that one of pathogenesis of TTP is autoimmune mechanism.     

Malignant thymoma associated with progressive systemic sclerosis

A 65-year-old man manifested certain features of scleroderma several years before discovery of malignant thymoma. Following tumor resection, the signs and symptoms of scleroderma did not improve, and the patient experienced the abrupt onset of renal failure with malignant hypertension 7 months after the operation. The scleroderma renal crisis caused terminal renal failure, which was treated by chronic hemodialysis. This is the second reported case of thymoma associated with progressive systemic sclerosis (PSS). The authors suggest that thymoma and various immunologic disorders have a common etiologic factor which has not yet been found. This case emphasizes that thymectomy frequently has little effect on the course of the immunologic disease. The implications of the association of thymoma and PSS are discussed.     

Malignant thymoma associated with mixed connective tissue disease: a case report

Many autoimmune diseases have been reported to be associated with malignancy. Mixed connective tissue disease (MCTD), however, has rarely been associated with malignancy. Thymoma is one of the neoplasms often reported to be related to various immunological disorders. Among the types of thymoma defined by WHO, malignant thymoma (thymoma type C) is the one least reported to be associated with autoimmune disease. Here, we report a case of malignant thymoma with concurrent MCTD, which manifested with acrosclerosis, Raynauds phenomenon, arthritis (synovitis), and a high titer of anti-ribonucleoprotein antibody.