小笠原　壽恵    Department   School of Medicine(Tokyo Women's Medical University Adachi Medical Center), School of Medicine    Position   Assistant Professor
Language	Japanese
Title	IVLBCL developed in bilateral adrenal gland, which represents a coexpression of BCL2, BCL6, and MYC
Conference	The 80th Annual Meeting of Japanese Society of Hematology
Conference Type	Nationwide Conferences
Presentation Type	Poster notice
Lecture Type	General
Publisher and common publisher	◎Nozomiko Ebata Toshie Ogasawara, Jyulia Hamasaki, Miki Shimada, Ai Kyono, Shoko Marshall , Kiyotaka Kawauchi1), Naoki Mori1), Hiroshi Sakura
Date	2018/10/14
Venue (city and name of the country)	Osaka, Japan
Society abstract	臨床血液 59(9),661 2018
Summary	Adrenal lymphoma is a rare lymphoma, accounting for less than 1% of malignant lymphomas and 3% of extranodal lymphomas. Most histopathological subtype of adrenal lymphomas is diffuse large B-cell lymphoma, while 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 of the biopsy specimen, indicating non-germinal center B-cell type lymphoma. Furthermore, triple expression of BCL2, BCL6, and MYC was demonstrated on the tumor cells. Bone marrow examination showed a normocellular marrow with large atypical lymphocytes in the absence of hemophagocytosis. Chromosomal analysis revealed complex karyotypic abnormalities, while rearrangement of BCL2 or MYC was not demonstrated by FISH analysis. Although the patient obtained a response to R-CHOP chemotherapy, its prognosis was predicted to be poor because of a non-GCB type lymphoma with bilateral adrenal tumor. Double-hit or triple-hit lymphomas are described as high grade B-cell lymphoma with poor prognosis in 2016 WHO classification. Although double expression of MYC and BCL2 protein without the gene translocations is inferred to be a prognostic indicator in DLBCL, the accurate significance of triple expression of BCL2, BCL6, and MYC without gene mutations 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.