Features of Extramedullary Disease of Multiple Myeloma: High Frequency of P53 Deletion and Poor Survival: A Retrospective Single-Center Study of 834 Cases
Features of Extramedullary Disease of Multiple Myeloma: High Frequency of P53 Deletion and Poor Survival: A Retrospective Single-Center Study of 834 Cases
复制标题
多发性骨髓瘤髓外病特点：p53缺失频率高、生存率低：834例单中心回顾性研究
DOI：
10.1016/j.clml.2014.12.013
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发表时间：
2015-05-01
影响因子：
2.7
通讯作者：
Qiu, Lugui
中科院分区：
文献类型：
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作者：
Deng, Shuhui;Xu, Yan;Qiu, Lugui
多发性骨髓瘤（MM）的髓外疾病（EMD）可在诊断时或随访期间发生，并表现出不同的临床特征。我们对834例连续MM患者的分析表明，EMD与P53缺失显著相关，采用荧光原位杂交分析确定，预后差。背景：多发性骨髓瘤（MM）是一种异质性疾病，大多数患者的骨髓瘤仅限于骨髓，一些患者在诊断时或随访期间出现髓外疾病（EMD），并显示出不同的临床特征和令人沮丧的预后。患者和方法：我们在中国的一个中心研究了834例连续的MM患者，并比较了有和没有EMD的患者的临床特征。结果：一般情况下，EMD的患病率在诊断时为4.8%，在随访期间为3.4%，近年来有显著增加。与未诊断EMD的患者相比，诊断时伴有EMD的MM患者P53缺失的发生率明显更高（34.5% vs. 11.9%; P = 0.037），乳酸脱氢酶（LDH）水平更高（P = 0.003）。随访期间的EMD复发/进展与诊断时的EMD表现、免疫球蛋白（IG）D亚型和FISH分析中的P53缺失相关，但与既往治疗（沙利度胺、硼替佐米或移植）无关。预后方面，多因素分析显示EMD是独立的不良预后因素。诊断时有和无EMD患者的总生存期分别为16.5和40个月（P < .001），两组的疾病进展时间分别为11.5和25个月（P < .001）。结论：MM患者在诊断时伴有EMD，在FISH分析中表现出更高的P53缺失率和更高的LDH水平。EMD复发/进展与诊断时的EMD表现、IgD亚型和FISH分析中的P53缺失相关，但与既往暴露于新药或移植无关。EMD参与的存在对生存率有负面影响。(C)2015 Elsevier Inc. All rights reserved.
Extramedullary disease (EMD) of multiple myeloma (MM) can develop at the time of diagnosis or during follow-up, and exhibits different clinical features. Our analysis of 834 consecutive MM patients showed that EMD was remarkably related with P53 deletion, determined using fluorescence in situ hybridization analysis, and a dismal prognosis.Background: Multiple myeloma (MM) is a heterogeneous disease in which most patients have myeloma restricted to the bone marrow, and some patients develop extramedullary disease (EMD) at the time of diagnosis or during follow-up, and show different clinical characteristics and a dismal prognosis. Patients and Methods: We studied 834 consecutive MM patients in a single center in China and compared clinical features of patients with and without EMD. Results: In general, the prevalence of EMD was 4.8% at the time of diagnosis and 3.4% during follow-up, with a significant increase in recent years. MM patients with EMD at the time of diagnosis had remarkably greater prevalence of P53 deletion determined using fluorescence in situ hybridization (FISH) analysis (34.5% vs. 11.9%; P = .037) and higher level of lactate dehydrogenase (LDH) (P = .003) compared with patients without EMD. EMD relapse/progression during follow-up was correlated with EMD presentation at diagnosis, immunoglobulin (Ig)D subtype and P53 deletion in FISH analysis, but not previous treatment (thalidomide, bortizomib, or transplantation). With respect to prognosis, multivariate analysis showed that EMD was an independent adverse prognostic factor. The overall survival of patients with and without EMD at diagnosis were 16.5 and 40 months, respectively (P < .001), and the time to disease progression of the 2 groups was 11.5 and 25 months, respectively (P < .001). Conclusion: MM patients with EMD at the time of diagnosis showed remarkably greater prevalence of P53 deletion in FISH analysis and higher LDH levels. EMD relapse/progression was correlated with EMD presentation at diagnosis, IgD subtype, and P53 deletion in FISH analysis, but not previous exposure to new drugs or transplantation. The presence of EMD involvement negatively affected survival. (C) 2015 Elsevier Inc. All rights reserved.