多发性骨髓瘤髓外疾病的发病情况、表现特征和转归：对 1003 例连续患者的纵向研究。

Incidence, presenting features and outcome of extramedullary disease in multiple myeloma: a longitudinal study on 1003 consecutive patients.

机构信息

Division of Hematology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, University of Pavia, Pavia, Italy.

出版信息

Ann Oncol. 2010 Feb;21(2):325-330. doi: 10.1093/annonc/mdp329. Epub 2009 Jul 24.

DOI:10.1093/annonc/mdp329

PMID:19633044

Abstract

BACKGROUND

There are few data on the incidence and prognosis of extramedullary (EM) multiple myeloma (MM). There are concerns about a possible increase of EM relapses with the expanding use of high-dose therapy (HDT) and biological agents.

PATIENTS AND METHODS

The incidence of EM disease, its relationship with prior exposure to HDT or novel agents, and its prognostic impact were analyzed in 1003 MM patients. Based on the different therapies available, three periods were considered: 1971-1993, conventional-dose chemotherapy; 1994-1999, HDT for younger patients; and 2000-2007, introduction of novel agents.

RESULTS

Overall, 13% of patients had EM disease, 7% at diagnosis and 6% later. In the 2000-2007 period, there was a significant increase of EM involvement, at diagnosis (P = 0.02) and during follow-up (P = 0.03). The risk of EM spread was not significantly increased after HDT [hazard ratio (HR 0.6)], bortezomib (HR 1.62), or thalidomide/lenalidomide (HR 1.07). EM disease was associated with shorter overall (HR 3.26, P < 0.0001) and progression-free (HR 1.46, P = 0.04) survival.

CONCLUSIONS

The incidence of EM disease has increased, probably due to the availability of more sensitive imaging techniques and the prolongation of patients' survival. HDT or novel agents seem not to increase the risk of EM disease. EM involvement confers a poor prognosis.

摘要

背景

有关髓外（EM）多发性骨髓瘤（MM）的发病和预后数据较少。人们担心随着大剂量治疗（HDT）和生物制剂的广泛应用，EM 复发的可能性会增加。

患者和方法

分析了 1003 例 MM 患者的 EM 疾病的发病率、与先前接受 HDT 或新型药物治疗的关系及其对预后的影响。根据不同的治疗方法，将研究分为三个时期：1971-1993 年，常规剂量化疗；1994-1999 年，年轻患者的 HDT；2000-2007 年，引入新型药物。

结果

总体而言，13%的患者患有 EM 疾病，7%在诊断时，6%在诊断后。在 2000-2007 年期间，EM 受累的发生率在诊断时（P=0.02）和随访期间（P=0.03）均显著增加。HDT[风险比（HR）0.6]、硼替佐米（HR 1.62）或沙利度胺/来那度胺（HR 1.07）后 EM 扩散的风险没有显著增加。EM 疾病与总生存期（HR 3.26，P<0.0001）和无进展生存期（HR 1.46，P=0.04）较短有关。