Gisriel Savanah D, Yuan Ji, Tang Haiming, Xu Jie, Fang Hong, Li Shaoying, McCracken Jenna, Li Peng, Braunberger Ryan C, Xu-Monette Zijun Y, Wu Xiaojun, Wang Endi, Zhang Qian-Yun, Soma Lorinda A, Katz Samuel G, Zhang Jing Jing, Matsumoto Nana P, Young Ken H, Xu Mina L, Pan Zenggang
Department of Pathology, Yale University School of Medicine, New Haven, CT, United States.
Department of Pathology and Laboratory Medicine, University of Wisconsin-Madison, Madison, WI, United States.
Am J Clin Pathol. 2025 Jun 29. doi: 10.1093/ajcp/aqaf057.
Rare large B-cell lymphomas (LBCLs) present with concurrent or subsequent lymphomatous effusion (solid-effusion LBCL, SE-LBCL), which may have an inferior prognosis compared with their noneffusion counterpart. In addition, the relationship between SE-LBCL and human herpesvirus 8-negative effusion-based LBCL (EB-LBCL) remains unclear.
We collected 141 cases of SE-LBCL and a control cohort of 101 cases of stage IV solid-only LBCL. The clinicopathologic features were analyzed and compared between SE-LBCL and solid-only LBCL.
Patients with SE-LBCL had a median age of 67 years with a male-to-female ratio of 1.3:1. Eighty-six patients had concurrent solid lymphoma and lymphomatous effusion, whereas 55 developed lymphomatous effusion subsequently. Most cases involved the pleural cavities (79%, 112/141), followed by the peritoneal (21%, 29/141) and pericardial (11%, 16/141) cavities. BCL6, CD10, and MUM1 were expressed in 77% (90/117), 46% (60/130), and 61% (58/95) of cases, respectively, and 58% (71/123) were subclassified into the germinal center B-cell (GCB) subtype. Rearrangements of BCL2, BCL6, and MYC were detected in 42% (31/73), 35% (22/63), and 40% (35/88), respectively, and 22% (19/87) had both MYC and BCL2 rearrangements. The patients with SE-LBCL had a dismal prognosis, with a median survival of 5.7 months, which was significantly worse than solid-only LBCL (147.5 months; P < .0001).
The pathologic features of SE-LBCL were similar to those of solid-only LBCL but distinct from those of EB-LBCL; in particular, lymphomatous effusion was an independently adverse prognostic factor in LBCL. Our study underscores the need for surveillance of lymphomatous effusion during LBCL staging and development of effective therapeutic regimens for SE-LBCL.
罕见的大B细胞淋巴瘤(LBCL)可伴有或随后出现淋巴瘤性积液(实体-积液LBCL,SE-LBCL),与无积液的同类淋巴瘤相比,其预后可能较差。此外,SE-LBCL与人类疱疹病毒8阴性的基于积液的LBCL(EB-LBCL)之间的关系仍不清楚。
我们收集了141例SE-LBCL病例和101例IV期单纯实体LBCL病例作为对照队列。对SE-LBCL和单纯实体LBCL的临床病理特征进行分析和比较。
SE-LBCL患者的中位年龄为67岁,男女比例为1.3:1。86例患者同时患有实体淋巴瘤和淋巴瘤性积液,而55例随后出现淋巴瘤性积液。大多数病例累及胸腔(79%,112/141),其次是腹腔(21%,29/141)和心包腔(11%,16/141)。BCL6、CD10和MUM1分别在77%(90/117)、46%(60/130)和61%(58/95)的病例中表达,58%(71/123)被分类为生发中心B细胞(GCB)亚型。BCL2、BCL6和MYC的重排分别在42%(31/73)、35%(22/63)和40%(35/88)的病例中检测到,22%(19/87)同时有MYC和BCL2重排。SE-LBCL患者预后不佳,中位生存期为5.7个月,明显差于单纯实体LBCL(147.5个月;P < .0001)。
SE-LBCL的病理特征与单纯实体LBCL相似,但与EB-LBCL不同;特别是,淋巴瘤性积液是LBCL的一个独立不良预后因素。我们的研究强调了在LBCL分期过程中监测淋巴瘤性积液以及为SE-LBCL制定有效治疗方案的必要性。