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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。 -
关键词:
- 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. -
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 — 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 — -
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