title: [Marfan syndrome complicated with CD5+ CD10+ diffuse large B-cell lymphoma].
abstract: 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.
authors.affiliation: Department of Hematology, International Medical Center, Saitama Medical University.
authors.forename: Kumiko
authors.initials: K
authors.lastname: Yoshitake
authors.forename: Yuki
authors.initials: Y
authors.lastname: Hagiwara
authors.forename: Ken
authors.initials: K
authors.lastname: Tanae
authors.forename: Naoki
authors.initials: N
authors.lastname: Takahashi
authors.forename: Mika
authors.initials: M
authors.lastname: Kohri
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Tamaru
authors.forename: Masami
authors.initials: M
authors.lastname: Bessho
authors.forename: Nozomi
authors.initials: N
authors.lastname: Niitsu
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage; D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018956:CD5 Antigens; D015536:Down-Regulation; D000071837:Fibrillins; D015870:Gene Expression; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000235:genetics; D008840:Microfilament Proteins / Q000235:genetics; D009154:Mutation; D015260:Neprilysin; D014066:Palatine Tonsil / Q000473:pathology; D018125:Receptors, Transforming Growth Factor beta / Q000235:genetics; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018956:CD5 Antigens; D000071837:Fibrillins; D008840:Microfilament Proteins; D018125:Receptors, Transforming Growth Factor beta; D000069283:Rituximab; D015260:Neprilysin