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血清重链/轻链对在意义未明的单克隆丙种球蛋白病和冒烟型骨髓瘤患者中的预后影响:来自单一机构的长期结果

Prognostic Impact of Serum Heavy/Light Chain Pairs in Patients With Monoclonal Gammopathy of Undetermined Significance and Smoldering Myeloma: Long-Term Results From a Single Institution.

作者信息

Magnano Laura, Fernández de Larrea Carlos, Elena Montserrat, Cibeira María Teresa, Tovar Natalia, Aróstegui Juan I, Pedrosa Fabiola, Rosiñol Laura, Filella Xavier, Yagüe Jordi, Bladé Joan

机构信息

Department of Hematology, Amyloidosis and Myeloma Unit, Hospital Clínic, Barcelona, and Institut d'Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Barcelona, Spain.

Department of Hematology, Amyloidosis and Myeloma Unit, Hospital Clínic, Barcelona, and Institut d'Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Barcelona, Spain.

出版信息

Clin Lymphoma Myeloma Leuk. 2016 Jun;16(6):e71-7. doi: 10.1016/j.clml.2016.02.034. Epub 2016 Feb 27.

Abstract

BACKGROUND

Asymptomatic monoclonal gammopathies, such as monoclonal gammopathy of undetermined significance (MGUS) and smoldering myeloma (SMM), are clinical conditions that usually precede symptomatic multiple myeloma. Therefore, risk stratification is crucial owing to the heterogeneous progression rate among these patients. In previous years, suppression of the uninvolved chain of specific heavy/light chain (HLC) pairs in serum has been identified as a new risk factor in MGUS. The aim of the present study was to investigate the prognostic effect of involved and uninvolved HLC pairs and HLC ratios on progression in a series of patients with MGUS and SMM.

PATIENTS AND METHODS

All specific serum HLC pairs were measured in 114 patients diagnosed with SMM (n = 27) and MGUS (n = 87) from 1983 to 2003. Also, the HLC ratios were calculated.

RESULTS

Progression to symptomatic multiple myeloma was observed in 13 patients (8 with SMM and 5 with MGUS). The risk of progression was 6 times greater in those with SMM (P = .001) and 4 times greater for those with the IgA isotype (P = .01). The suppression of any IgM isotypes (IgMκ or IgMλ) in patients with IgA or IgG gammopathy or any IgA isotypes (IgAκ or IgAλ) in patients with IgG or IgM gammopathy was associated with a shorter time to progression to symptomatic gammopathy (P = .001 and P = .03, respectively). On multivariate analysis, the evolving pattern and suppression of any IgM HLC pair remained significant.

CONCLUSION

HLC ratios could be a valuable tool in the risk stratification of patients with SMM and MGUS, especially patients with IgG isotypes.

摘要

背景

无症状单克隆丙种球蛋白病,如意义未明的单克隆丙种球蛋白病(MGUS)和冒烟型骨髓瘤(SMM),通常是有症状的多发性骨髓瘤之前的临床状态。因此,由于这些患者的进展速度存在异质性,风险分层至关重要。在过去几年中,血清中特定重链/轻链(HLC)对的未受累链的抑制已被确定为MGUS中的一个新风险因素。本研究的目的是调查受累和未受累HLC对及HLC比值对一系列MGUS和SMM患者进展的预后影响。

患者和方法

对1983年至2003年诊断为SMM(n = 27)和MGUS(n = 87)的114例患者测定了所有特定血清HLC对。此外,还计算了HLC比值。

结果

13例患者(8例SMM和5例MGUS)进展为有症状的多发性骨髓瘤。SMM患者进展风险高6倍(P = .001),IgA同种型患者进展风险高4倍(P = .01)。IgA或IgG丙种球蛋白病患者中任何IgM同种型(IgMκ或IgMλ)的抑制,或IgG或IgM丙种球蛋白病患者中任何IgA同种型(IgAκ或IgAλ)的抑制,均与进展为有症状丙种球蛋白病的时间缩短相关(分别为P = .001和P = .03)。多变量分析显示,任何IgM HLC对的演变模式和抑制仍然具有显著性。

结论

HLC比值可能是SMM和MGUS患者风险分层的一个有价值的工具,尤其是IgG同种型患者。

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