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t(11;14) 多发性骨髓瘤:一种与独特免疫特征、免疫表型特征相关但结局不同的亚型。

t(11;14) multiple myeloma: a subtype associated with distinct immunological features, immunophenotypic characteristics but divergent outcome.

机构信息

Department of Lymphoma & Myeloma, Institute of Hematology & Blood Diseases Hospital, State Key Laboratory of Experimental Hematology, Chinese Academy of Medical Sciences & Peking Union of Medical College, Tianjin, People's Republic of China.

出版信息

Leuk Res. 2013 Oct;37(10):1251-7. doi: 10.1016/j.leukres.2013.06.020. Epub 2013 Aug 5.

Abstract

UNLABELLED

t(11;14)(q13;q32) is the most common chromosome translocation in multiple myeloma (MM), but a consensus of clinicopathological features and impact on survival is yet to be reached. We analyzed a cohort of 350 patients with various plasma cell malignancies, including newly diagnosed MM (NDMM, n=253), relapsed/refractory MM (RRMM, n=77), as well as primary and secondary plasma cell leukemia (PCL, n=10 and n=10, respectively).

RESULTS

A remarkably higher frequency of t(11;14) was observed in the PCL than in the NDMM. A high incidence of t(11;14) was detected in the IgD, IgM, and nonsecretory MM. The t(11;14) MM group was associated with a significantly higher positive rate of B-lineage associated antigens CD20 and CD79a as well as the lack of CD56 expression. t(11;14) was less likely to be accompanied by 13q14 deletion than 13q14 deletion frequency in non-t(11;14) population (p=0.026), and fewer patients displaying t(11;14) were identified as belonging to the high-risk cytogenetic group due to the extremely low incidence of t(4;14) and t(14;16). As a whole, patients exhibiting t(11;14) had a comparable outcome with the control cohort in NDMM, but CD20 was able to identify two subsets of the disease with dissimilar outcomes. Among patients receiving bortezomib-based treatment, patients harboring t(11;14) without CD20 expression had a significantly shortened PFS (11.0 versus 43.0 months, p=0.005) and OS (16.5 versus 54.0 months, p=0.016) compared with patients displaying t(11;14) with CD20. Our findings suggest that although the t(11;14) plasma cell disorder displayed distinct biological, clinical and laboratory features, it was a heterogeneous disease with divergent outcome.

摘要

未标记

t(11;14)(q13;q32) 是多发性骨髓瘤 (MM) 中最常见的染色体易位,但尚未达成关于临床病理特征和对生存影响的共识。我们分析了一组 350 名患有各种浆细胞恶性肿瘤的患者,包括新诊断的 MM (NDMM,n=253)、复发/难治性 MM (RRMM,n=77) 以及原发性和继发性浆细胞白血病 (PCL,n=10 和 n=10)。

结果

在 PCL 中观察到 t(11;14)的频率明显高于 NDMM。在 IgD、IgM 和非分泌性 MM 中检测到高发生率的 t(11;14)。t(11;14) MM 组与 B 细胞相关抗原 CD20 和 CD79a 的阳性率显著升高以及 CD56 表达缺失有关。与非 t(11;14)人群相比,t(11;14) 较少伴有 13q14 缺失(p=0.026),由于 t(4;14)和 t(14;16) 的发生率极低,显示 t(11;14)的患者较少被归类为高危细胞遗传学组。总的来说,在 NDMM 中,表现出 t(11;14)的患者与对照组的结果相当,但 CD20 能够识别疾病的两个亚组,其结果不同。在接受硼替佐米治疗的患者中,不表达 CD20 的 t(11;14)患者的 PFS(11.0 与 43.0 个月,p=0.005)和 OS(16.5 与 54.0 个月,p=0.016)明显缩短,与显示 t(11;14)和 CD20 的患者相比。我们的研究结果表明,尽管 t(11;14)浆细胞疾病表现出独特的生物学、临床和实验室特征,但它是一种具有不同结局的异质性疾病。

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