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意义未明的单克隆丙种球蛋白病后发生的多发性骨髓瘤。10例患者的研究

[Multiple myeloma after monoclonal gammopathy of uncertain significance. Study of 10 patients].

作者信息

Salgado C, Bladé J, López-Guillermo A, Cervantes F, Montserrat E, Rozman C

机构信息

Escuela de Hematología Farreras Valenti, Hospital Clínico y Provincial, Universidad de Barcelona.

出版信息

Sangre (Barc). 1993 Oct;38(5):371-4.

PMID:8140498
Abstract

PURPOSE

To analyse the clinical, biological and evolutive characteristics of 10 patients with multiple myeloma (MM) previously diagnosed of monoclonal gammopathy of uncertain significance (MGUS).

PATIENTS AND METHODS

Ten patients with diagnostic criteria of MM, included in a series of 114 subjects with MGUS, IgG (92 cases), IgA (21 cases) or biclonal IgG+IgA (1 case), diagnosed between 1970 and 1990, comprise this study group.

RESULTS

The actuarial risk of developing MM in the series of 114 MGUS was 7.25% (95% CI: 1.5-13) and 16.1% (05% CI: 4.7-27.5) at 5 and 10 years of follow-up, respectively. MM occurred suddenly, both on clinical and biological grounds, in 8 of the 10 patients. The median period between the diagnosis of MGUS and the onset of MM was 48 months (range, 12-153). IgG MM appeared in 5 instances and IgA MM in 5 others. Bone pain (7 cases) and hypercalcaemic encephalopathy (2 cases) were the commonest clinical manifestations. Osteolytic lesions were found in the x-ray scan in 9 patients. Eight patients received chemotherapy, objective response being seen in five of them. The median survival since the diagnosis of MM was 18 months (range, 0.1-95). The causes of death in 8 deceased patients include disease progression (4 cases), pneumonia (3 cases) and neoplasm of the bladder (1 case).

CONCLUSIONS

This analysis shows the difficulties in predicting the outcome of MGUS, since most of the MM seen here occurred abruptly and after a highly variable length of time. Thus, patients with MGUS must be followed-up for undetermined time.

摘要

目的

分析10例先前诊断为意义未明的单克隆丙种球蛋白病(MGUS)的多发性骨髓瘤(MM)患者的临床、生物学及病情演变特征。

患者与方法

本研究组包括10例符合MM诊断标准的患者,这些患者来自于1970年至1990年间诊断的114例MGUS患者系列,其中IgG型92例、IgA型21例、双克隆IgG + IgA型1例。

结果

在114例MGUS患者系列中,5年和10年随访时发生MM的精算风险分别为7.25%(95%CI:1.5 - 13)和16.1%(95%CI:4.7 - 27.5)。10例患者中有8例MM的发生在临床和生物学方面均很突然。MGUS诊断至MM发病的中位时间为48个月(范围12 - 153个月)。出现5例IgG型MM和5例IgA型MM。骨痛(7例)和高钙血症性脑病(2例)是最常见的临床表现。9例患者X线扫描发现溶骨性病变。8例患者接受化疗,其中5例有客观反应。自MM诊断后的中位生存期为18个月(范围0.1 - 95个月)。8例死亡患者的死亡原因包括疾病进展(4例)、肺炎(3例)和膀胱肿瘤(1例)。

结论

本分析显示预测MGUS预后存在困难,因为此处所见的大多数MM发病突然且间隔时间长短不一。因此,MGUS患者必须进行不确定时长的随访。

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