Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe 18 cases of a distinctive morphologic variant of primary thymic epithelial neoplasm characterized by a micronodular growth pattern associated with florid lymphoid follicular hyperplasia of the stroma. The tumors occurred in seven women and 11 men aged 41 to 76 years (mean, 58 years). All cases were asymptomatic and discovered incidentally on routine chest radiograph or during coronary artery bypass surgery. The tumors measured from 3 to 10 cm in greatest dimension and were well circumscribed and encapsulated. In seven cases, the lesions were grossly described as cystic or partially cystic masses. Histologically, they were characterized by a proliferation of small tumor nodules separated by abundant lymphoid stroma with prominent germinal centers. The nodules were composed of spindle cells containing oval nuclei devoid of atypia or mitotic activity. Immunohistochemical studies showed strong positivity of the spindle tumor cells for CAM 5.2 and broad spectrum keratin antibodies. The surrounding lymphoid cell population was strongly positive for LCA and L26 and showed a polyclonal pattern of staining for kappa and lambda. Stains for UCHL-1, CD1a, CD3, CD5, and CD99 were negative in the stromal lymphoid cell population. The tumor in one of the patients was associated with active pulmonary tuberculosis, and in another with anemia and splenomegaly of unknown etiology. None of the patients had clinical signs or history of myasthenia gravis or other autoimmune disorders. The present cases are interpreted as an unusual morphologic variant of spindle cell thymoma with prominent B-cell lymphoid hyperplasia. The possible significance of this phenomenon is discussed.
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PMID:Micronodular thymoma with lymphoid B-cell hyperplasia: clinicopathologic and immunohistochemical study of eighteen cases of a distinctive morphologic variant of thymic epithelial neoplasm. 1043 66

A 62-year-old female with chest and right shoulder pain admitted to a hospital. The chest computed tomography (CT) scan showed a large anterior mediastinum mass with pleural effusion. Thymoma was suspected by percutaneous aspiration biopsy of it. She was referred to our hospital for operation with decreased serum platelet count (7.0 x 10(4)/microliter) and high platelet associated IgG (PA IgG) level (119.5 ng/10(7) cells). Result of bone marrow examination was normal, splenomegaly was not seen and no other autoimmune disease was not detected, so she was thought to have ITP. After giving her intravenous high-dose gamma-globulin and her platelet count increased to normal, operation was done. Extended thymectomy was performed and postoperative course was uneventful. Pathological finding showed the mass mediastinum hematoma in thymus and there found a small thymoma. The hemorrhage was thought to occur from the thymoma. After the operation, her platelet count has not been changed to normal but remained lower level. Though myasthenia gravis, one of the autoimmune disease, improve after total thymectomy, it is thought that idiopathic thrombocytopenic purpura (ITP) could not improve after thymectomy.
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PMID:[Thymoma with idiopathic thrombocytopenic purpura preceded by mediastinal hemorrhage; report of a case]. 1223 13

Myasthenia gravis (MG), a neuromuscular junction autoimmune disease, sometimes complicates second malignancies; however, T-cell lymphoproliferative disorders have rarely been reported. A 55-year-old man, who received oral tacrolimus and prednisolone for MG for 16 years after thymectomy, presented with left abdominal pain, lymphadenopathy, and splenomegaly. A lymph node biopsy revealed peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). This is the first report of oral tacrolimus leading to a T-cell lymphoproliferative disorder in patient without a history of transplantation. Physicians should be aware of the possibility of rare T-cell lymphoproliferative disorders, such as PTCL-NOS, occurring as complications in MG patients on immunosuppressive regimens after thymectomy.
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PMID:Myasthenia Gravis Complicated with Peripheral T-cell Lymphoma, Not Otherwise Specified (PTCL-NOS), Following Thymectomy and Longstanding Tacrolimus Therapy. 2926 44

Autoimmune polyglandular syndrome (APS) is a rare disease that is characterized by autoimmune reactions to multiple endocrine and non-endocrine organs, which can be divided into four main types. The principal manifestations of APS-3 are autoimmune thyroid disease and other autoimmune diseases, such as type 1 diabetes, atrophic gastritis, pernicious anemia, vitiligo, alopecia, and myasthenia gravis, but not Addison's disease or hypoparathyroidism. Here we report a case demonstrating the rare coexistence of growth hormone deficiency and hyperthyroidism with sexual dysgenesis, secondary amenorrhea, cardiomegaly, splenomegaly, hypoproteinemia, pleural effusion, seroperitoneum, pericardial effusion, anasarca, osteoporosis, vitamin D deficiency, iron-deficiency anemia, poor blood coagulation, leucocytopenia, peripheral neuropathy, hyperuricemia, ichthyosis, tinea cruris, and onychomycosis.
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PMID:A rare case of coexistence of autoimmune polyglandular syndrome type 3 with growth hormone deficiency and hyperthyroidism in a patient with pseudo-Turner's syndrome. 3304 97