Hashimoto Norisato, Ueda Tomoki, Hiraiwa Shinichiro, Tajiri Takuma, Nakamura Naoya, Yokoyama Kenji
Department of Hematology/Oncology, Tokai University Hachioji Hospital, 1838 Ishikawa-machi, Hachioji 1920032, Japan.
Department of Pathology, Tokai University Hachioji Hospital, 1838 Ishikawa-machi, Hachioji 1920032, Japan.
Case Rep Hematol. 2020 Dec 1;2020:8876567. doi: 10.1155/2020/8876567. eCollection 2020.
Plasmablastic lymphoma (PBL) is a rare aggressive lymphoma. Although it was first described in HIV- (human immunodeficiency virus-) infected patients, PBL has been diagnosed in patients with other immunodeficiencies as well as in immunocompetent patients. PBL immunohistochemically expresses plasmacytic markers and lacks pan B-cell markers. The cells of origin of PBL are considered to be plasmablasts. MYC gene rearrangement and MYC overexpression are frequently found in PBL, but the pathogenesis of PBL is yet to be elucidated. Here, we report a case of composite lymphoma of PBL and diffuse large B-cell lymphoma (DLBCL); that is, PBL in the urinary bladder and DLBCL in the nasal cavity occurred simultaneously. We extracted DNA from the two lymphomas for polymerase chain reaction and sequenced the amplified immunoglobulin heavy variable genes and the complementarity-determining region- (CDR-) 3. The sequence of the CDR3 region of both tumors matched. MYC rearrangement was found in the bladder tumor but not in the nasal tumor. The patient was treated with R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisone), and durable remission had been obtained. The results of the DNA analysis indicated that both PBL and DLBCL emerged from common postgerminal B cells. This case may help to elucidate the pathogenesis of PBL.
浆母细胞淋巴瘤（PBL）是一种罕见的侵袭性淋巴瘤。尽管它最初是在感染人类免疫缺陷病毒（HIV）的患者中被描述的，但PBL也在其他免疫缺陷患者以及免疫功能正常的患者中被诊断出来。PBL免疫组化表达浆细胞标志物且缺乏泛B细胞标志物。PBL的起源细胞被认为是浆母细胞。MYC基因重排和MYC过表达在PBL中经常被发现，但PBL的发病机制尚未阐明。在此，我们报告一例PBL与弥漫性大B细胞淋巴瘤（DLBCL）的复合淋巴瘤病例；也就是说，膀胱中的PBL和鼻腔中的DLBCL同时发生。我们从这两种淋巴瘤中提取DNA进行聚合酶链反应，并对扩增的免疫球蛋白重链可变基因和互补决定区（CDR）-3进行测序。两种肿瘤的CDR3区域序列匹配。在膀胱肿瘤中发现了MYC重排，而在鼻腔肿瘤中未发现。该患者接受了R-CHOP（利妥昔单抗、环磷酰胺、长春新碱、阿霉素和泼尼松）治疗，并获得了持久缓解。DNA分析结果表明，PBL和DLBCL均起源于共同的生发后B细胞。该病例可能有助于阐明PBL的发病机制。
