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意义未明的单克隆丙种球蛋白病和冒烟型多发性骨髓瘤

Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma.

作者信息

Kyle R A

出版信息

Eur J Haematol Suppl. 1989;51:70-5. doi: 10.1111/j.1600-0609.1989.tb01496.x.

DOI:10.1111/j.1600-0609.1989.tb01496.x
PMID:2516809
Abstract

Patients with monoclonal gammopathy of undetermined significance (MGUS) have a monoclonal protein but no evidence of multiple myeloma, macroglobulinemia, amyloidosis, or related disorders. In a long-term follow-up (median, 19 years) of 241 patients with MGUS, 53 (22%) developed multiple myeloma (36 patients), macroglobulinemia (7 patients), primary systemic amyloidosis (7 patients), or malignant lymphoproliferative disease (3 patients). Fifty-seven patients (24%) remained stable and were alive, while 124 patients (51%) died of causes unrelated to the monoclonal gammopathy. Multiple myeloma was diagnosed in the 36 patients 23 to 251 months (median, 9.6 years) after recognition of the M-protein. Smoldering multiple myeloma (SMM) is characterized by a serum M-protein level greater than 30 g/l and greater than 10% plasma cells in the bone marrow but no anemia, renal insufficiency, or lytic lesions. Differentiation of MGUS and SMM from multiple myeloma and macroglobulinemia is difficult at the time of diagnosis of the M-protein, and the patients must be followed up indefinitely.

摘要

意义未明的单克隆丙种球蛋白病(MGUS)患者有单克隆蛋白,但无多发性骨髓瘤、巨球蛋白血症、淀粉样变性或相关疾病的证据。在对241例MGUS患者进行的长期随访(中位时间为19年)中,53例(22%)发展为多发性骨髓瘤(36例)、巨球蛋白血症(7例)、原发性系统性淀粉样变性(7例)或恶性淋巴增殖性疾病(3例)。57例患者(24%)病情稳定且存活,而124例患者(51%)死于与单克隆丙种球蛋白病无关的原因。在识别出M蛋白后23至251个月(中位时间为9.6年),36例患者被诊断为多发性骨髓瘤。冒烟型多发性骨髓瘤(SMM)的特征是血清M蛋白水平大于30 g/l,骨髓中浆细胞大于10%,但无贫血、肾功能不全或溶骨性病变。在诊断M蛋白时,将MGUS和SMM与多发性骨髓瘤和巨球蛋白血症区分开来很困难,必须对患者进行无限期随访。

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