Marfan syndrome complicated with CD5+ CD10+ diffuse large B-cell lymphoma

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  • YOSHITAKE Kumiko
    Department of Hematology, International Medical Center, Saitama Medical University
  • HAGIWARA Yuki
    Department of Hematology, International Medical Center, Saitama Medical University
  • TANAE Ken
    Department of Hematology, International Medical Center, Saitama Medical University
  • TAKAHASHI Naoki
    Department of Hematology, International Medical Center, Saitama Medical University
  • KOHRI Mika
    Department of Hematology, International Medical Center, Saitama Medical University
  • TAMARU Jun-ichi
    Department of Pathology, Saitama Medical University
  • BESSHO Masami
    Department of Hematology, International Medical Center, Saitama Medical University
  • NIITSU Nozomi
    Department of Hematology, International Medical Center, Saitama Medical University

Bibliographic Information

Other Title
  • Marfan症候群に合併したCD5陽性CD10陽性びまん性大細胞型B細胞リンパ腫
  • 症例報告 Marfan症候群に合併したCD5陽性CD10陽性びまん性大細胞型B細胞リンパ腫
  • ショウレイ ホウコク Marfan ショウコウグン ニ ガッペイ シタ CD5 ヨウセイ CD10 ヨウセイビマンセイ ダイ サイボウガタ B サイボウ リンパ シュ

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Description

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∼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-β) have been implicated in the pathogenesis of MFS and downregulation of TGF-β receptor expression has been described in the pathology of B-cell lymphoma.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 51 (3), 196-200, 2010

    The Japanese Society of Hematology

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