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无症状及需治疗的多发性骨髓瘤——来自捷克单克隆丙种球蛋白病登记处的数据

Asymptomatic and Treatment-requiring Multiple Myeloma - Data from the Czech Registry of Monoclonal Gammopathies.

作者信息

Brozova L, Jarkovský J, Pour L, Minárik J, Jungová A, Gregora E, Spicka I, Maisnar V, Hájek R

出版信息

Klin Onkol. 2017 Summer;30(Supplementum2):51-59. doi: 10.14735/amko20172S51.

Abstract

BACKGROUND

Monoclonal gammopathy of undetermined significance (MGUS) and smouldering multiple myeloma (SMM) are premalignant stages of multiple myeloma (MM). MM is a malignancy of plasma cells, which is associated with a median overall survival of 5 to 7 years. MM accounts for approximately 10% of hematological malignancies.

PATIENTS AND METHODS

Descriptive analysis of data from 19 Czech centres collected in the Registry of Monoclonal Gammopathies (RMG) was performed.

RESULTS

Over the last 10 years of prospective collection of data, together with retrospectively recorded data on patients diagnosed before the registry establishment, data on 7,467 patients with either asymptomatic or symptomatic form of MM have been gathered. Validation criteria for the analysis were met by 2,506 MGUS patients, 400 SMM patients and 4,738 MM patients. The median duration of follow-up was 4.3 years in MGUS patients and 2.4 years in SMM patients. The overall risk of progression from MGUS to malignancy was 1.7% per year. The risk of progression from SMM to MM was highest in the 1st years after diagnosis: overall, this risk was 16.6% per year. The median duration of follow-up was 2.8 years in MM patients. The median overall survival from the diagnosis was 5.7 years. The median OS from treatment initiation/progression-free survival decreased from 60.5/21.0 months in the 1st line therapy to 34.3/12.4 months in the 2nd line therapy, 22.6/8.9 months in the 3rd line therapy and 13.8/5.8 months in the 4th or higher line therapies. Thanks to the availability of novel drugs for MM treatment in the Czech Republic, treatment strategies have changed dramatically over the last decade.

CONCLUSION

RMG is a registry designated for the collection of data on diagnosis, treatment, treatment results and survival of patients with monoclonal gammopathies in the long-term follow-up. RMG is a valuable source of data from real clinical practice.Key words: registries - monoclonal gammopathy of undetermined significance - smouldering multiple myeloma - multiple myeloma - progression - treatment - survival.

摘要

背景

意义未明的单克隆丙种球蛋白病(MGUS)和冒烟型多发性骨髓瘤(SMM)是多发性骨髓瘤(MM)的癌前阶段。MM是一种浆细胞恶性肿瘤,其总体中位生存期为5至7年。MM约占血液系统恶性肿瘤的10%。

患者与方法

对在单克隆丙种球蛋白病登记处(RMG)收集的来自19个捷克中心的数据进行描述性分析。

结果

在过去10年的前瞻性数据收集过程中,连同登记处建立之前诊断患者的回顾性记录数据,已收集到7467例无症状或有症状形式MM患者的数据。2506例MGUS患者、400例SMM患者和4738例MM患者符合分析的验证标准。MGUS患者的中位随访时间为4.3年,SMM患者为2.4年。MGUS进展为恶性肿瘤的总体风险为每年1.7%。SMM进展为MM的风险在诊断后的第1年最高:总体而言,该风险为每年16.6%。MM患者的中位随访时间为2.8年。自诊断起的中位总生存期为5.7年。从治疗开始/无进展生存期的中位总生存期从一线治疗的60.5/21.0个月降至二线治疗的34.3/12.4个月、三线治疗的22.6/8.9个月以及四线或更高线治疗的13.8/5.8个月。由于捷克共和国有用于MM治疗的新药,过去十年治疗策略发生了巨大变化。

结论

RMG是一个用于长期随访收集单克隆丙种球蛋白病患者诊断、治疗、治疗结果和生存数据的登记处。RMG是真实临床实践中有价值的数据来源。关键词:登记处 - 意义未明的单克隆丙种球蛋白病 - 冒烟型多发性骨髓瘤 - 多发性骨髓瘤 - 进展 - 治疗 - 生存

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