A patient with thymoma associated immunodeficiency syndrome (Good's syndrome) and bronchiectasis was retrospectively analyzed. Good's syndrome is a rare condition of immunodeficiency that is characterized by thymoma and hypogammaglobulinemia. It is important to bear in mind that Good's syndrome should be included in the differential diagnosis When patients repeatedly visited for bronchiectasis or infection, we should alert to their immune state and history of thymoma. Early screening of immunological status and aggressive correction of immune deficiency are beneficial to improving the prognosis to patients with Good's syndrome.
Agammaglobulinemia/complications* ; Bronchiectasis/complications* ; Humans ; Retrospective Studies ; Thymoma/complications* ; Thymus Neoplasms/complications*

Hypertrophic osteoarthropathy is characterized by clubbing and periosteal new bone formation along the shaft of the long bones of the extremities. Although various intrathoracic malignancies have been associated with the development of HOA, it has been extremely rare for HOA to occur in a patient with a thymic carcinoma. Recently, we experienced a 63-year-old woman diagnosed as a thymic carcinoma with hypertrophic osteoarthropathy. She had both digital clubbing and cortical thickening in her lower extremities identified radiologically. We herein describe this case with a review of the literature.
Carcinoma/complications/*diagnosis ; Female ; Human ; Middle Aged ; Osteoarthropathy, Secondary Hypertrophic/*etiology ; Thymus Neoplasms/complications/*diagnosis

Adult ; Aged ; Anemia ; etiology ; Diarrhea ; etiology ; Humans ; Male ; Paraneoplastic Syndromes ; etiology ; Thymoma ; complications ; Thymus Neoplasms ; complications

OBJECTIVEDiffuse panbronchiolitis, a distinct clinical entity of unknown etiology, has been reported originally and primarily in Japanese and rarely in non-Japanese populations. Macrolide therapy is effective for this once dismal disease. Diffuse panbronchiolitis complicated with thymoma is uncommon; only 2 cases have been reported to date. The aims of this study were to describe the clinical profiles, assess the response to macrolide therapy, and to discuss the possible pathogenesis of diffuse panbronchiolitis in this setting.

METHODSThe clinical profiles, macrolide therapy response of diffuse panbronchiolitis complicated with encapsulated thymoma in 2 histologically confirmed cases were described and discussed with the 2 cases reported in the literature: one complicated with encapsulated thymoma, another with invasive thymoma.

RESULTSOf the 2 cases, both had negative PPD skin testing and abnormal serum levels of various immunoglobulins, 1 had positive anti-nuclear antibody, but none had elevated cold hemagglutinin titers, and both had an excellent response to macrolide therapy. Of the 2 cases reported in the literature, both had negative PPD or tuberculin skin testing, 1 had severe hypogammaglobulinemia, 1 had elevated IgA, 1 had positive anti-DNA, 1 had elevated cold hemagglutinin titers, but both died of respiratory failure in spite of macrolide therapy in 1 case.

CONCLUSIONSPrognosis for diffuse panbronchiolitis complicated with thymoma may depend on the nature of the thymoma and on the disease course. Macrolide therapy is also effective if administered early in the disease course and if the thymoma is cured. Immunological factors may play an important role in the pathogenesis of diffuse panbronchiolitis in this setting.

Adult ; Bronchiolitis ; complications ; drug therapy ; mortality ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; Thymoma ; complications ; Thymus Neoplasms ; complications

OBJECTIVETo explore the clinical significance of serum anti-ryanodine receptor (RyR) antibody in the diagnosis of myasthenia gravis (MG).

METHODSThe crude sarcoplasmic reticulum was prepared from rabbit skeletal muscle, and then purified by differential centrifugation to produce the antigen. The serum anti-RyR antibody levels in 74 patients with MG (including 21 patients with comorbidic thymomas) were determined with ELISA.

RESULTSWestern blot demonstrated the presence of RyR in purified crude sarcoplasmic reticulum. The positive rate of anti-RyR antibody was significantly higher in MG patients who had comorbidic thymoma compared with those who had no such comorbidity (P < 0.01). Also, the positive rate was closely correlated with the severity of MG.

CONCLUSIONSerum anti-RyR antibody test is helpful in the diagnosis of MG associated with thymoma and can be used to judge the outcome of MG.

Autoantibodies ; blood ; Humans ; Myasthenia Gravis ; blood ; complications ; immunology ; Ryanodine Receptor Calcium Release Channel ; immunology ; Thymoma ; complications ; Thymus Neoplasms ; complications

Anesthesia ; methods ; Humans ; Male ; Middle Aged ; Muscle Hypotonia ; etiology ; Stiff-Person Syndrome ; complications ; physiopathology ; Thymoma ; surgery ; Thymus Neoplasms ; surgery

OBJECTIVETo explore the clinical characteristics and outcome of myasthenia gravis with and without thymoma after operation.

METHODSTwo hundred and forty-three patients with myasthenia gravis surgically treated in our department from 1978 to 2003 were studied retrospectively. The clinical characteristics, complication, remission and survival were compared between myasthenia gravis with and without thymoma.

RESULTSThe patients of myasthenia gravis with thymoma were significantly older (t = 6.138, P = 0.000), had shorter duration of symptom (t = 3.783, P = 0.000), and also had higher myasthenia crisis rates after operation (chi(2) = 64.77, P = 0.000) than those of myasthenia gravis without thymoma. No differences of Osserman classification was found between the two groups (chi(2) = 7.678, P = 0.104). The complete remission rates and partial remission rates of myasthenia gravis with thymoma were significantly lower than those of myasthenia gravis without thymoma at 1 and 3 years (P = 0.049, P = 0.000; P = 0.015, P = 0.010), but no differences at 5 year (P = 0.457; P = 0.699). The survival rates of MG with thymoma were lower than that of MG without thymoma (Log-rank = 18.58, P = 0.000).

CONCLUSIONSThe clinical characteristics are different between myasthenia gravis with and without thymoma. The remission of symptom of myasthenia gravis with thymoma is worse than that of myasthenia gravis without thymoma in the near future, but is similar in the long future. The death rates of MG with thymoma is significantly higher than that of MG without thymoma.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; mortality ; surgery ; Retrospective Studies ; Survival Rate ; Thymectomy ; Thymoma ; complications ; Thymus Neoplasms ; complications ; Treatment Outcome

OBJECTIVETo evaluate the different pathological and clinical characteristics of thymomas with and without myasthenia gravis (MG) and to determine whether the presence of MG influences the prognosis in thymoma patients.

METHODSThe clinical data from 228 consecutive patients (median sternotomy were used in 153, video-assisted thoracoscopic themectomy were used in 75) operated on from January 1992 to December 2007 was analyzed retrospectively. These thymoma patients had been subdivided into two groups: thymoma with MG (n = 125) and thymoma without MG (n = 103). All thymic epithelial tumors were classified according to the WHO histologic classification and the Masaoka clinical staging system. The result was evaluated according to the Myasthenia Gravis Foundation of America's criterion. The clinical features of the 2 test was compared between the two groups by χ² test, and the survival were compared between the two groups by Cox analysis.

RESULTSThere were no peri-operative deaths. 19 cases were inoperable (6 in the group with MG, 13 without MG (χ² = 4.52, P = 0.035)). The proportions of type A and thymic carcinoma were 0 in the group with MG, 10.5% (11/103) and 11.6% (12/103) respectively in the group without MG. According to the Masaoka's clinical staging, in the group MG, 24.8% (31/125) patients were stage III and IV; in the group without MG, 33.0% (34/103) patients were stage III and IV. There was a significant difference between hyperplastic paraneoplastic thymus coexisting in 28.8% (36/125) patients with MG and only 5.8% (6/103) in patients without MG (χ² = 20.91, P = 0.000) Microthymoma was identified in the paraneoplastic thymus of 3 patients with MG. There were 198 patients followed up, the rate was 86.8% (198/228). There was no recurrence in patients with type A and a few patients with type AB, B1, B2, B3 thymoma and thymic carcinoma recurred. The actuarial 5- and 10-year survival rates were 89.3% and 81.2% for patients with MG respectively, and 90.0% and 78.9% for patients without MG respectively. Within 5 years postoperatively, 6 of 9 patients with MG died of myasthenia crisis, while 6 out of 7 deaths in patients without MG were attributable to inoperable tumors (stage IV) and thymic carcinoma.

CONCLUSIONSThe existence of myasthenia gravis has little influence on the prognosis of thymomas, but it is good for early diagnosis and treatment. Extended thymectomy should be performed to all patients with thymoma, no matter they have myasthenia gravis or not. The main cause of death is myasthenia crisis for thymoma patients with MG and stage IV and (or) thymic carcinoma for patients without MG.

Humans ; Myasthenia Gravis ; complications ; pathology ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neoplasms, Glandular and Epithelial ; complications ; pathology ; surgery ; Postoperative Period ; Prognosis ; Retrospective Studies ; Sternotomy ; Survival Rate ; Thoracic Surgery, Video-Assisted ; Thymectomy ; Thymoma ; complications ; pathology ; surgery ; Thymus Neoplasms ; complications ; pathology ; surgery

Myasthenia gravis is an autoimmune disease that results from an antibody-mediated reaction and occurs with thymoma in 15+ACU- of patients. It is very rarely associated with autoimmune hepatitis. Four cases of myasthenia gravis with autoimmune hepatitis have been reported in the world. We recently experienced a case of 30-year-old man with myasthenia gravis associated with thymoma and autoimmune hepatitis. This condition is the first case that has not been reported previously in Korea. We report this rare condition along with a brief review of the literature.
Adult ; Case Report ; HLA-DR Antigens/genetics ; Hepatitis, Autoimmune/etiology+ACo- ; Human ; Male ; Myasthenia Gravis/complications+ACo- ; Thymoma/etiology+ACo- ; Thymus Neoplasms/etiology+ACo-

The association of thymoma with pure red cell aplasia has been well documented, but amegakaryocytic thrombocytopenia is not a recognized paraneoplastic syndrome complicating thymoma. We report a case of thymoma-complicated pure red cell aplasia and amegakaryocytic thrombocytopenia in a 73-yr-old woman. Pure red cell aplasia was diagnosed seven months after the detection of thymoma. One year after the diagnosis of pure red cell aplasia and seven months after thymectomy, bone marrow aspiration and biopsy showed an absence of megakaryocytes, marked erythroid hypoplasia with normal myeloid series. A diagnosis of amegakaryocytic thrombocytopenia and pure red cell aplasia was made. Oral steroid maintenance therapy resulted in recovery of platelet count. She has still transfusion-dependant anemia but platelet and neutrophil counts had been maintained in normal range for more than five months, until the last follow-up. We think that autoreactive T cells may induce a clinical autoimmune response even after eradication of thymoma, and aplastic anemia as a late complication following thymectomy was described in previous cases. This patient also has to be under a close observation because of the possibility to evolve into aplastic anemia.
Aged ; Bone Marrow/pathology ; Female ; Humans ; Imidazoles/therapeutic use ; Megakaryocytes/pathology ; Pregnadienetriols/therapeutic use ; Red-Cell Aplasia, Pure/complications/*diagnosis ; Thrombocytopenia/*diagnosis/drug therapy/*etiology ; Thymectomy/*adverse effects ; Thymoma/*complications/diagnosis/surgery ; Thymus Neoplasms/*complications/diagnosis/surgery