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意义未明的单克隆丙种球蛋白病的自然史。对263例患者进行的5至20年随访。

The natural history of monoclonal gammopathy of undetermined significance. A 5- to 20-year follow-up of 263 cases.

作者信息

Pasqualetti P, Festuccia V, Collacciani A, Casale R

机构信息

Department of Internal Medicine and Public Health, School of Medicine and Surgery, University of L'Aquila, Italy.

出版信息

Acta Haematol. 1997;97(3):174-9. doi: 10.1159/000203676.

DOI:10.1159/000203676
PMID:9066713
Abstract

Patients with monoclonal gammopathy of undetermined significance (MGUS) have a serum monoclonal component (M-component), but no evidence of multiple myeloma, macroglobulinaemia, amyloidosis or other plasma cell proliferative disease. A long-term follow-up study (median 11.5 years) has been carried out in 263 cases of MGUS, 159 males (60.5%) and 104 females (39.5%), aged 40-89 years (median 66.5 years). The actuarial probability for malignant transformation was 6.1, 15.4 and 31.3% at 5, 10 and 20 years, respectively. At the final evaluation, 157 patients (59.7%), 119 (45.3%) of whom with no increase and 38 (14.4%) with an increase in serum M-component, died of causes unrelated to MGUS and without development of any plasma cell proliferative disease; 47 patients (17.9%) were still alive without increase in M-component; 11 patients (4.1%) were still alive and at follow-up presented values of serum M-component > 30 g/l without any evidence of plasma cell proliferative or lymphoproliferative disease; 48 patients (18.3%) developed multiple myeloma (35 cases, 13.1%), solitary plasmacytoma of the bone (2 cases, 0.8%), macroglobulinaemia (4 cases, 1.6%), malignant lymphoma (3 cases, 1.2%), amyloidosis (2 cases, 0.8%), chronic lymphocytic leukaemia (1 case, 0.4%), and plasma cell leukaemia (1 case, 0.4%). The patients developing multiple myeloma, solitary plasmacytoma, macroglobulinaemia and plasma cell leukaemia had an increase in serum M-component, whereas no increase was found in malignant lymphoma, amyloidosis and chronic lymphocytic leukaemia. These findings and the data in the literature suggest that MGUS could be considered a preneoplastic condition; since no clinical and laboratory features are able to identify in advance the patients at high risk of disease progression, each patient must be followed up indefinitely.

摘要

意义未明的单克隆丙种球蛋白病(MGUS)患者血清中有单克隆成分(M成分),但无多发性骨髓瘤、巨球蛋白血症、淀粉样变性或其他浆细胞增殖性疾病的证据。对263例MGUS患者进行了一项长期随访研究(中位随访时间11.5年),其中男性159例(60.5%),女性104例(39.5%),年龄40 - 89岁(中位年龄66.5岁)。5年、10年和20年时恶性转化的精算概率分别为6.1%、15.4%和31.3%。在最终评估时,157例患者(59.7%)死亡,其中119例(45.3%)血清M成分无增加,38例(14.4%)血清M成分增加,均死于与MGUS无关的原因,且未发生任何浆细胞增殖性疾病;47例患者(17.9%)仍存活,M成分无增加;有11例患者(4.1%)仍存活,随访时血清M成分值>30 g/l,无任何浆细胞增殖性或淋巴细胞增殖性疾病的证据;48例患者(18.3%)发生了多发性骨髓瘤(35例,13.1%)、骨孤立性浆细胞瘤(2例,0.8%)、巨球蛋白血症(4例,1.6%)、恶性淋巴瘤(3例,1.2%)、淀粉样变性(2例,0.8%)、慢性淋巴细胞白血病(1例,0.4%)和浆细胞白血病(1例,0.4%)。发生多发性骨髓瘤、孤立性浆细胞瘤、巨球蛋白血症和浆细胞白血病的患者血清M成分增加,而恶性淋巴瘤、淀粉样变性和慢性淋巴细胞白血病患者血清M成分无增加。这些发现以及文献中的数据表明,MGUS可被视为一种肿瘤前状态;由于没有临床和实验室特征能够提前识别疾病进展高危患者,因此必须对每位患者进行无限期随访。

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