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中国癌症防治杂志

中国癌症防治杂志 ›› 2021, Vol. 13 ›› Issue (3): 277-282.doi: 10.3969/j.issn.1674-5671.2021.03.10

• 临床研究 • 上一篇    下一篇

伴MYD88 L265P突变的脾边缘区淋巴瘤临床特征分析

  

  1. 中国医学科学院/北京协和医院血液内科
  • 出版日期:2021-06-25 发布日期:2021-07-08
  • 通讯作者: 张薇， E-mail：vv1223@vip.sina.com
  • 基金资助:
    北京市自然科学基金项目（7202154）

Clinical features of splenic marginal zone lymphoma with MYD88 L265P mutation

  • Online:2021-06-25 Published:2021-07-08

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摘要： 目的 分析伴MYD88 L265P突变的脾边缘区淋巴瘤（splenic marginal zone lymphoma，SMZL）患者的临床特征。方法 回顾性分析本中心SMZL患者队列，将MYD88 L265P突变型患者的临床特征与野生型以及华氏巨球蛋白血症 (Waldenstr-m macroglobulinemia，WM)的患者进行比较。结果 本中心SMZL患者队列中，MYD88 L265P突变率为10.3%（7/68），与该基因野生型的SMZL患者相比，突变型均为老年患者（100.0% vs 56.3%，P=0.038），更易出现B症状（85.7% vs 42.1%，P=0.046），M蛋白阳性率更高（85.7% vs 46.2%，P=0.043）。而相较WM患者，SMZL患者淋巴结受累相对少见（28.6% vs 61.3%），无高黏滞症状，M蛋白阳性率较低（85.7% vs 100.0%），且约1/3为非IgM型M蛋白。结论 伴MYD88 L265P突变的SMZL患者具有一定临床特征，确诊需依据典型的形态学结合免疫分型/免疫组化，确诊困难的患者进一步检查7q31缺失、IGHV突变状态、NOTCH2和KLF2突变有助于辅助诊断。

关键词: 脾边缘区淋巴瘤, 淋巴浆细胞淋巴结, 华氏巨球蛋白血症, MYD88 L265P突变

Abstract: Objective To analyze the clinical features of patients in splenic marginal zone lymphoma (SMZL) with MYD88 L265P mutation. Methods The cases of SMZL patients in Chinese Academy of Medical Sciences were retrospectively reviewed, and the features of MYD88 L265P mutation patients were compared with those of the wild-type and Waldenstr-m macroglobulinemia (WM) patients. Results In the SMZL patient cohort of Chinese Academy of Medical Sciences, the rate of MYD88 L265P mutation was 10.3% (7/68). Compared with the wild-type SMZL patients of the same gene, the mutants were all elderly patients (100.0% vs 56.3%, P=0.038), and B symptoms were more likely to occur (85.7% vs 42.1%, P=0.046), and the positive rate of M protein was higher (85.7% vs 46.2%, P=0.043). In contrast to WM patients, SMZL patients were rarely lymph node-involved (28.6% vs 61.3%), and had no hyperviscosity and a relatively low positive rate of monoclonal protein (85.7% vs 100.0%), one third of which had non-IgM type. Conclusions The SMZL patients with MYD88 L265P mutation shows some clinical features, and the diagnosis should be based on morphological classification combined immunological/histoimmunochemistry. Patients with difficulty in diagnosis further examined for 7q31 deletion, IGHV mutation status, NOTCH2 and KLF2 mutation may help for diagnosis.

Key words: Splenic marginal zone lymphoma, Lymphoplasmacytic lymphoma, Waldenstr-m macroglobulinemia, MYD88 L265P mutation

中图分类号: 

  • R733.4