Transformation of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia into diffuse large B-cell lymphoma: five cases report and literature review

TL;DR

This paper reports on five rare cases where lymphoplasmacytic lymphoma/Waldenström macroglobulinemia transformed into diffuse large B-cell lymphoma, highlighting clinical features and outcomes.

Contribution

The study provides new clinical insights into the rare transformation of LPL/WM into DLBCL through a multi-center case series and literature review.

Findings

Five patients with LPL/WM developed DLBCL, showing common features like β2-microglobulin elevation and MYD88 mutations.

Transformation occurred after a median of 11.8 months, with symptoms like weight loss and lymphadenopathy.

R-CHOP treatment achieved partial responses, but overall survival remained poor with a median of 16.8 months.

Abstract

分析淋巴浆细胞淋巴瘤/华氏巨球蛋白血症（LPL/WM）转化为弥漫大B细胞淋巴瘤（DLBCL）患者的临床特征及预后情况。

回顾性分析湖南省多中心自2020年12月至2023年4月期间诊治的5例LPL/WM转化为DLBCL患者的临床资料，比较转化前后的临床表现、治疗方案及疗效。

5例患者中男4例，女1例，中位年龄64（57～80）岁，诊断时均可见β2微球蛋白水平异常升高，2例合并乳酸脱氢酶升高。4例患者检测到MYD88L265P突变，1例携带FAT1与NOTCH1突变，5例均未检出CXCR4突变。3例患者TP53突变阴性，余2例未检测。转化前，3例患者接受布鲁顿酪氨酸激酶抑制剂治疗，1例接受BR方案治疗。所有患者均转化为非生发中心来源型DLBCL，中位转化时间为11.8（4.0～19.0）个月，多伴体重下降、淋巴结肿大、脾肿大和结外受累等表现。转化后主要采用R-CHOP方案治疗，最佳疗效为部分缓解。其中4例患者出现疾病进展，中位总生存期为16.8（10.0～26.0）个月。

LPL/WM向DLBCL的转化罕见，若患者出现淋巴结迅速增大和（或）新出现淋巴结受累、全身症状进行性加重、体质下降时需高度警惕转化可能。R-CHOP方案在该类患者中可取得一定缓解，但总体预后仍不理想。