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不一致性淋巴瘤,其特征为脑内弥漫性大B细胞淋巴瘤与结肠、直肠和骨髓中的套细胞淋巴瘤共存。

Discordant lymphoma characterized by the coexistence of diffuse large B-cell lymphoma in the brain and mantle cell lymphoma in the colon, rectum, and bone marrow.

作者信息

Yamaguchi Kyosuke, Yamamoto Go, Watanabe Otoya, Kageyama Kosei, Kaji Daisuke, Taya Yuki, Nishida Aya, Ishiwata Kazuya, Takagi Shinsuke, Yamamoto Hisashi, Asano-Mori Yuki, Uruga Hironori, Ito Shinji, Takazawa Yutaka, Wake Atsushi, Uchida Naoyuki, Taniguchi Shuichi

机构信息

Department of Hematology, Toranomon Hospital, Tokyo, Japan.

Department of Hematology, Toranomon Hospital Kajigaya, Kanagawa, Japan.

出版信息

Brain Tumor Pathol. 2025 Apr;42(2):26-32. doi: 10.1007/s10014-025-00499-y. Epub 2025 Mar 18.

Abstract

We describe a rare case of discordant lymphoma characterized by the coexistence of diffuse large B-cell lymphoma (DLBCL) in the brain and mantle cell lymphoma (MCL) in the colon, rectum, and bone marrow. A 63-year-old male patient with consciousness impairment and gait disturbance was admitted to our institution. Head computed tomography scan and contrast-enhanced magnetic resonance imaging showed a mass in the right temporal lobe and rectal wall thickening. Brain biopsy revealed DLBCL, and bone marrow and rectum biopsy showed MCL. According to a polymerase chain reaction analysis of immunoglobulin heavy-chain gene rearrangements using brain and bone marrow specimens, the two lesions were clonally unrelated lymphomas. After five cycles of R-MPV (rituximab, methotrexate, procarbazine, vincristine) therapy and three cycles of R-ESHAP (rituximab, etoposide, cytarabine, cisplatin, methylprednisolone) therapy, the patient received autologous hematopoietic stem cell transplantation using R-MEAM (rituximab, ranimustine, etoposide, cytarabine, melphalan) regimen after bridging therapy with ibrutinib. In addition, he received whole-brain irradiation at a dose of 40 Gy in 20 fractions as consolidation therapy. He did not relapse within 3 years of transplantation. To the best of our knowledge, this is the first case report of DLBCL and MCL coexistence.

摘要

我们描述了一例罕见的异源性淋巴瘤,其特征为脑内弥漫性大B细胞淋巴瘤(DLBCL)与结肠、直肠及骨髓中的套细胞淋巴瘤(MCL)共存。一名63岁男性患者因意识障碍和步态不稳入住我院。头部计算机断层扫描及增强磁共振成像显示右侧颞叶有一肿块及直肠壁增厚。脑活检显示为DLBCL,骨髓及直肠活检显示为MCL。根据使用脑和骨髓标本进行的免疫球蛋白重链基因重排的聚合酶链反应分析,这两个病灶是克隆无关的淋巴瘤。经过5个周期的R-MPV(利妥昔单抗、甲氨蝶呤、丙卡巴肼、长春新碱)治疗和3个周期的R-ESHAP(利妥昔单抗、依托泊苷、阿糖胞苷、顺铂、甲泼尼龙)治疗后,患者在使用伊布替尼进行桥接治疗后,采用R-MEAM(利妥昔单抗、司莫司汀、依托泊苷、阿糖胞苷、美法仑)方案接受了自体造血干细胞移植。此外,他接受了总量40 Gy分20次的全脑照射作为巩固治疗。移植后3年内他未复发。据我们所知,这是DLBCL与MCL共存的首例病例报告。

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