Light Chain Amyloidosis (AL) Associated With B Cell Lymphoma a Single Center Experience
Light Chain Amyloidosis (AL) Associated With B Cell Lymphoma a Single Center Experience
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DOI：
10.1016/j.clml.2021.07.003
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发表时间：
2021-11-30
影响因子：
2.7
通讯作者：
Kukreti, Vishal
中科院分区：
文献类型：
--
作者：
De la Torre, Alfredo;Reece, Donna;Kukreti, Vishal
简介：轻链（AL）淀粉样变性和b细胞淋巴瘤占所有AL的5%至7%，系统性淀粉样变性，沉积在远离潜在淋巴瘤的部位，瘤周淀粉样变性沉积在邻近区域。材料和方法：我们进行了一项回顾性研究，以识别和描述1997年1月1日至2019年7月31日在玛格丽特公主癌症中心的AL伴B细胞淋巴瘤。结果：35例AL合并淋巴瘤患者，发病率为6.2%，中位诊断年龄66岁（47 ~ 86岁），以男性居多，多数有潜在的Waldestrom大球蛋白血症，21例为瘤周AL (PAL)， 15例为全身性AL， 42.8%的患者有主要器官受累。35%的患者接受利妥昔单抗联合烷基化剂治疗，20%的患者接受蛋白酶体抑制剂治疗，17%的患者采取观察等待方法，淀粉样蛋白反应显示非常好的部分反应45.8%，淋巴瘤ORR为42.8%，中位随访31.5个月。36个月的总生存期（OS）和无进展生存期（PFS）显示，心脏受累OS （P = 0.002）、PFS（0.057）和IgM亚型OS （P = 0.02）、PFS（0.01）的预后更差。结论：我们已经显示了IgM AL的不良结果，并记录了以前未报道的PAL的OS和PFS差异。(C) 2021 Elsevier Inc.。版权所有。
Introduction: Light chain (AL) amyloidosis and B-cell lymphoma represent 5% to 7% of all AL, Systemic amyloidosis, deposits in sites remote from the underlying lymphoma, and peritumoral amyloidosis deposition is within the immediate vicinity. Materials and Methods: We conducted a retrospectively study to identify and describe AL with B cell lymphoma at Princess Margaret Cancer Center from 01 January 1997 to 31 July 2019. Results: Thirty-five patients with AL and lymphoma, an incidence of 6, 2%, median age of diagnosis of 66 (range 47 to 86), majority male, most had underlying Waldestrom's Macroglobulinemia. 21 patients with peritumoral AL (PAL), and 15 with systemic AL. 42.8% of the patients had major organ involvement. 35% got treatment with Rituximab with alkylator, 20% received proteasome inhibitors, 17% patients were on a watch and wait approach, amyloid response showed very good partial response 45.8%, and lymphoma ORR was 42.8%, with a median follow up of 31.5 months. A 36 month overall survival (OS) and progression-free survival (PFS) showed worse outcomes for heart involvement OS (P = .002), PFS (0.057) and IgM subtype OS (P = .02), PFS (0.01). Conclusion: We have shown adverse outcome with IgM AL and to document a differences in OS and PFS not previously reported for PAL. (C) 2021 Elsevier Inc. All rights reserved.