小笠原 壽恵
   Department   School of Medicine(Tokyo Women's Medical University Adachi Medical Center), School of Medicine
   Position   Assistant Professor
Article types Case report
Language Japanese
Peer review Peer reviewed
Title BCL2, BCL6, and MYC-positive intravascular large B-cell lymphoma presenting with bilateral adrenal gland lesions
Journal Formal name:The Japanese Journal of Clinical Hematology
Abbreviation:Rinsho Ketsueki
ISSN code:04851439/18820824
Domestic / ForeginDomestic
Volume, Issue, Page pp.570-576
Author and coauthor Toshie Ogasawara†*, Nozomiko Ebata, Jyulia Hamasaki, Shoko Marshall, Kiyotaka Kawauchi, Koichi Ohshima, Naoki Mori, Hiroshi Sakura
Authorship Lead author,Corresponding author
Publication date 2019/06
Summary Primary adrenal lymphoma is a rare lymphoma, accounting for less than 0.2% of non-Hodgkin lymphomas. Most histopathological subtype of adrenal lymphomas is diffuse large B-cell lymphoma, and intravascular large B-cell lymphoma (IVLBCL) is rare. We report a case of IVLBCL occurred as bilateral adrenal gland tumor, which was diagnosed by CT-guided biopsy. Tumor cells were positive for CD20, MUM-1 but not for CD10 on an immunostaining, indicating non-germinal center B-cell subtype lymphoma. Furthermore, triple expression of BCL2, BCL6, and MYC was demonstrated on the tumor cells. Bone marrow examination showed an involvement of lymphoma cells but not hemophagocytosis. Chromosomal analysis revealed complex karyotypic abnormalities without a rearrangement of BCL2 or MYC by FISH analysis. Although the patient obtained a response to R-CHOP chemotherapy, he developed central nervous system involvement by lymphoma. The significance of triple expression of BCL2, BCL6, and MYC without gene translocation is not well defined. For clarifying the pathogenesis and clinical significance of the triple expression of these oncoproteins, an accumulation of the similar cases is needed.
DOI 10.11406/rinketsu.60.570