Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eight cases of AIL-type T-cell malignant lymphoma are reported. The clinical symptoms are the same as those described in AIL: fever, malaise, weight loss, skin rashes, polyadenopathy, and splenomegaly. However, some differences can be noted: the absence of hepatomegaly in all cases but one, the absence of polyclonal hypergammapathy in all cases but one, and predominance in females. The lymph node modifications comprise diffuse infiltrations of lymphoid cells with irregular nuclei and pale cytoplasm, associated with a large number of immunoblasts and plasma cells. Some eosinophilic granulocytes and epithelioid cells can be seen. Hyperplasia of the vessels and remnants of follicles, sometimes with proliferation of follicular dendritic cells, are prominent features. The immunolabelling study demonstrates the presence of an important T-cell population all expressing a high predominance of CD 4 phenotype. These findings are in accordance with those published in Europe and in contrast with those of some of the Japanese cases, particularly the first patients published by Shimoyama et al. The differential diagnosis with AIL is based on the presence of clusters of mainly large cells with a pale cytoplasm, on the loss of expression of one T cell marker, as in 3 cases of our series, and on the presence of a high percentage of lymphoid cells engaged in the mitotic cycle as demonstrated with the Ki 67 monoclonal antibody. However, to draw a clear cut difference between AIL-type T-cell lymphoma and AIL considered as a prelymphomatous dysimmune lymphadenopathy, only the demonstration of cytogenetic abnormalities, as in one of our cases or of rearrangement of the genes coding for beta and/or gamma chain of the antigen receptor of T-cell are valuable criteria. The follow-up of our series is not long enough to appreciate the prognosis. Three patients died, one from a glioma. All the other cases, treated with polychemotherapy show total remission with an evolution of 10 to 39 months.
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PMID:Angio-immunoblastic lymphadenopathy (AIL) or T-cell malignant lymphoma of AIL-type. A histopathological, immunohistochemical and ultrastructural study of 8 cases. 326 11

Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated multisystem lymphoproliferative and granulomatous disease histologically characterized by angiocentric and angiodestructive cellular infiltrate. LYG usually involves the lungs, skin, kidneys and brain, but isolated central nervous system LYG has been reported rarely. We describe a 57-year-old man with histologically proved LYG confined to the brain. The patient complained of visual disturbance, and was admitted to our hospital. Gadolinium-enhanced T1-weighted MRI showed irregular, partly cystic and lobulated enhancement in the left occipital lobe, suggesting the presence of possible neoplastic lesions such as glioma, malignant lymphoma or metastatic brain tumor. We performed surgical resection of the lesion. Histological and immunohistochemical examinations of the tumor showed angiocentric polymorphic infiltration and granulomatous reaction, consisting of CD3-positive small lymphocytes and CD20-positive large atypical cells. Subsequently, in situ hybridization study for EBER (Epstein-Barr virus encoded small RNA) was performed and proved that the large atypical cells were positive for EBER. These findings were compatible with LYG. We emphasize that the possibility of LYG should be considered in the differential diagnosis of any ring-like enhancing mass lesions on MR images, though less specific for LYG. Histopathological examination is indispensable for the final diagnosis and the choice of an optimal treatment strategy.
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PMID:[Primary central nervous system lymphomatoid granulomatosis: a case report]. 2187 44