双克隆淋巴浆细胞淋巴瘤5例报道并文献复习

黄燕姗; 于颖; 熊文婕; 阎禹廷; 王婷玉; 吕瑞; 王齐; 邱录贵; 易树华

doi:10.12354/j.issn.1000-8179.2023.20230198

双克隆淋巴浆细胞淋巴瘤5例报道并文献复习

doi: 10.12354/j.issn.1000-8179.2023.20230198

黄燕姗^{1, 2,},
于颖^{1, 2},
熊文婕^{1, 2},
阎禹廷^{1, 2},
王婷玉^{1, 2},
吕瑞^{1, 2},
王齐^{1, 2},
邱录贵^{1, 2},
易树华^{1, 2, ,}

1.
中国医学科学院血液病医院（中国医学科学院血液学研究所）, 实验血液学国家重点实验室, 国家血液系统疾病临床医学研究中心, 细胞生态海河实验室（天津市300020）
2.
天津医学健康研究院

基金项目: 本文课题受国家自然科学基金项目（编号：81970187，82170193，81900203）和中国医学科学院医学与健康科技创新工程项目（编号：2021-I2M-C&T-B-081）资助

详细信息

作者简介:
黄燕姗：专业方向为B细胞淋巴瘤的相关研究

通讯作者:
易树华　yishuhua@ihcams.ac.cn

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- 文章访问数: 95
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- 被引次数: 0
出版历程
- 收稿日期: 2023-03-06
- 录用日期: 2023-05-15
- 修回日期: 2023-05-13
- 网络出版日期: 2023-05-29

Bi-clonal lymphoplasmacytic lymphoma: five cases report and a literature review

Yanshan Huang^{1, 2
,},
Ying Yu^{1, 2},
Wenjie Xiong^{1, 2},
Yuting Yan^{1, 2},
Tingyu Wang^{1, 2},
Rui Lv^{1, 2},
Qi Wang^{1, 2},
Lugui Qiu^{1, 2},
Shuhua Yi^{1, 2
, ,}

1.
State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China
2.
Tianjin Institutes of Health Science, Tianjin 301600, China

Funds: This work was supportrd by the National Natural Science Foundation of China (No. 81970187，No. 82170193，No. 81900203) and Medical and Health Science and Technology Innovation Project of Chinese Academy of Medical Sciences（No. 2021-I2M-C&T-B-081）

More Information

Corresponding author: Shuhua Yi; E-mail: yishuhua@ihcams.ac.cn

摘要

摘要: 目的探讨双克隆型淋巴浆细胞淋巴瘤（lymphoplasmacytic lymphoma，LPL）的临床特征及诊疗疗效。方法收集1993年6月至2017年10月就诊于中国医学科学院血液病医院5例双克隆型LPL患者的临床资料并进行分析。结果 5例患者中男性4例、女性1例。3例为华氏巨球蛋白血症国际预后指数（ISSWM）分期高危患者，4例患者表达免疫球蛋白G（IgG）和免疫球蛋白M（IgM），3例患者表达不同类型轻链。5例患者的血清免疫固定电泳均为双克隆，骨髓均可见异常淋巴细胞及浆细胞浸润。5例患者确诊双克隆LPL后，2例CD20阳性者接受利妥昔单抗为基础的化疗方案、1例伊布替尼口服治疗、1例苯达莫司汀治疗效果不佳改用沙利度胺+地塞米松+环磷酰胺（TCD）治疗、1例使用司莫司汀+环磷酰胺+马法兰+地塞米松+长春新碱（M2）化疗。5例患者中4例达到微小缓解（minimal response，MR）及以上疗效，中位随访时间为44.1个月，中位无进展生存期（median progression-free survival，mPFS）为52.4个月，中位总生存期（median overall survival，mOS）为 52.4个月。截至 2022 年5月随访结束，2例患者部分缓解（partial response，PR），2例患者死亡。结论双克隆LPL非常罕见，对该疾病的临床特征、诊断、治疗转归及预后尚缺乏深刻一致的认识，仍需更多的临床病例总结分析。
- 淋巴浆细胞淋巴瘤 /
- 双克隆淋巴浆细胞淋巴瘤 /
- 华氏巨球蛋白血症 /
- 免疫固定电泳
Abstract: Objective To investigate the clinical features and treatment efficacy of bi-clonal lymphoplasmacytic lymphoma (LPL). Methods We collected clinical data from five patients with bi-clonal LPL who visited Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College from 1993 to 2017 and reviewed the relevant literature. Results Four of the five patients were male, and three were high-risk patients according to ISSWM staging. Bi-clonal serum immunofixation electrophoresis was performed. Abnormal lymphocytes and plasma cell infiltration were observed in the bone marrow of all five patients. Additionally, four patients expressed IgG and IgM antibodies, while three patients expressed different types of light chains. Serum immunofixation electrophoresis was biclonal in all five patients, and abnormal lymphocytes and plasma cell infiltration were observed in the bone marrow of all five patients. After bi-clonal LPL was confirmed in all patients, varied treatment strategies were employed. Two CD20-positive patients received rituximab-based chemotherapy regimens, one was treated with ibrutinib, one was treated with TCD because of the poor effect of bendamustine, and one was treated with M2. Four of the five patients achieved minimal response (MR) or greater. After a median follow-up time of 44.1 months, a median progression-free survival (mPFS) of 52.4 months, and a median overall survival (mOS) of 52.4 months were observed. At the end of the follow-up, two patients maintained partial response (PR) while two passed away. Conclusions Occurrence of bi-clonal LPL is very rare, and deep and consistent understanding of the clinical features, diagnosis, treatment regression, and prognosis of this disease is lacking. Therefore, more summary and analysis of clinical cases is required.
- lymphoplasmacytic lymphoma (LPL) /
- bi-clonal lymphoplasmacytic lymphoma /
- Waldenstrom macroglobulinemia (WM) /
- immunofixation electrophoresis (IFE)

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图 1 患者血清免疫固定电泳图

A：患者1；B：患者2；C：患者3；D：患者5

下载: 全尺寸图片幻灯片

图 2 骨髓形态图

A：患者1；B：患者2

下载: 全尺寸图片幻灯片

表 1 患者M蛋白类型分析

患者编号	性别	年龄（岁）	血红蛋白（g/L）	血小板（×10⁹/L)	M蛋白类型	初诊IgM（g/L）	初诊IgG（g/L）
1	男	80	117	216	IgGκ和IgMκ	10.10	8.70
2	女	77	98	182	IgGκ和IgMκ	8.12	25.40
3	男	70	47	62	IgGλ和IgMκ	5.89	14.20
4	男	73	83	22	IgMλ和IgMκ	51.50	5.45
5	男	45	61	51	IgGκ和IgMλ	4.58	41.00
6^[4]	男	86	36	142	IgAλ和IgMκ	5.71	6.14
7^[5]	男	72	75	正常	IgGκ和IgAκ	0.97	51.90
8^[6]	男	66	81	84	IgGκ和IgMκ	36.60	14.50
9^[7]	女	58	58	148	IgMκ和IgAκ	10.00	13.30
10^[8]	男	83	100	正常	IgMλ和IgMκ	34.07	—

下载: 导出CSV

表 2 5例患者骨髓异常肿瘤细胞比例及免疫表型

患者编号	骨髓流式异常淋巴细胞比例（%）	骨髓流式异常浆细胞比例（%）	免疫表型
1	0.68	0.49	CD20个别+，PAX5少数+，CD3少数+，CD5少数+，CD10-， CD38浆细胞+，Cyclin D1-
2	10.87	6.81	CD20+，PAX5+，CD3-，CD5-，CD10-，CD23-，Cyclin D1-， CD138+，Kappa+，Lambda-
3	12.66	19.29	PAX5+，CD20-，CD3少数+，CD5少数+，CD11c-，CD103-， CD38少数+，Kappa-，Lambda+，CD23少数+
4	0.48	2.94	CD34+，CD19+，HLA-DR+，CD19+，CD20+，CD22+，Kappa+
5	79.50	19.50	—

下载: 导出CSV

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