Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorders, Diffuse Large B-Cell Lymphoma Type, in a Patient with Behçet's Disease Treated with Cyclosporine A

Yuka Urano; Rintaro Ohe; Akane Yamada; Takanobu Kabasawa; Kazushi Suzuki; Takumi Kitaoka; Nobuyuki Tamazawa; Naing Ye Aung; Aya Utsunomiya; Tomomi Toubai; Kenichi Ishizawa

doi:10.1159/000510362

Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorders, Diffuse Large B-Cell Lymphoma Type, in a Patient with Behçet's Disease Treated with Cyclosporine A

Case Rep Oncol. 2020 Sep 21;13(3):1145-1151. doi: 10.1159/000510362. eCollection 2020 Sep-Dec.

Affiliations

¹ Department of Pathological Diagnostics, Yamagata University Faculty of Medicine, Yamagata, Japan.
² Department of Neurology, Hematology, Metabolism, Endocrinology and Diabetology, Yamagata University Faculty of Medicine, Yamagata, Japan.

Abstract

A 40-year-old man had been treated for Behçet's disease (BD) with cyclosporine A (CsA) for 14 years. He presented multiple lymphadenopathies with fever. Histological examination of surgical biopsy showed other iatrogenic immunodeficiency-associated lymphoproliferative disorders, diffuse large B-cell lymphoma type with positivity for Epstein-Barr virus encoding RNA-1 (EBER-1). BCL2-IgH, BCL6-IgH, and MYC-IgH translocations were not detected. CsA was stopped, and R-CHOP therapy was initiated. However, his lymphoma was chemotherapy resistant and rapidly progressed. To the best of our knowledge, this is the first case of diffuse large B-cell lymphoma that occurred in a BD patient treated with CsA reported in English. Both BD and CsA are associated with the pathogenesis of lymphoma. We also describe extremely rare cases in the form of a literature review.

Keywords: Behçet's disease; Cyclosporine A; Diffuse large B-cell lymphoma; Epstein-Barr virus; Lymph node.

Publication types

Case Reports