Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Marfan syndrome (MFS) is caused by mutations in the gene encoding fibrillin. A 35-year-old man with MFS visited a local physician because of a sore throat. His left tonsil gradually became swollen and he was referred to our department. Histopathological examination of tonsil biopsy specimens showed diffuse proliferation of lymphoma cells with large nuclei. The tumor cells showed CD5+, CD10+, CD20+, BCL-6+, and MUM-1-. Based on these findings, the patient was diagnosed with CD5+ CD10+ diffuse large B-cell lymphoma (DLBCL). Chemotherapy combined with rituximab was administered and complete response was achieved. CD5+ DLBCL comprises approximately 5 approximately 10% of DLBCLs. In addition, CD5+ CD10+ DLBCL comprises about 5% of CD5+ DLBCLs. There may be a relationship between MFS and B-cell lymphoma because mutations in the gene encoding the receptor of transforming growth factor-beta (TGF-beta) have been implicated in the pathogenesis of MFS and downregulation of TGF-beta receptor expression has been described in the pathology of B-cell lymphoma.
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PMID:[Marfan syndrome complicated with CD5+ CD10+ diffuse large B-cell lymphoma]. 2037 14

We describe the case of a 70-year-old man with Marfan Syndrome who was found to have Diffuse Large B Cell Lymphoma (DLBCL) within an ascending aortic graft, his native aortic arch and arch vessels. Ten years previously he had undergone a Bentall's Procedure for a dilated aortic root. He presented with a left middle cerebral artery stroke. Computed tomography (CT) scan revealed multiple filling defects within the aortic arch and branch vessels suggestive of thrombus. Following the stroke, his neurological deficit improved. A decision was made to perform an aortic arch replacement to reduce the risk of a further embolic event. Subsequent histopathology confirmed DLBCL. Intravascular DLBCL is an extremely rare entity but an important diagnosis to consider in patients with intra-aortic thrombus in proximity to prosthetic graft material.
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PMID:Diffuse Large B Cell Lymphoma in a Prosthetic Aortic Graft. 2764 Oct 96