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[单克隆丙种球蛋白病的疾病分类学与管理]

[Nosology and management of monoclonal gammapathy].

作者信息

Grosbois Bernard, Decaux Olivier, Guenet Lucienne, Goasguen Jean, Jego Patrick

机构信息

Médecine Interne, Hôpital Sud, CHU, 16 boulevard de Bulgarie, 35203, Rennes.

出版信息

Bull Acad Natl Med. 2009 May;193(5):1069-85; discussion 1085-7.

Abstract

Monoclonal gammapathy (MG) affects about 1% of the general population, and its prevalence is higher in elderly subjects. Monoclonal gammapathy of undetermined significance (MGUS), the most common disorder, is asymptomatic and associated with normal hemoglobin, calcium and creatinine levels and a monoclonal component of less than 15 g/l. A B cell neoplasm should be suspected in patients with clinical manifestations and/or abnormal hemoglobin, calcium or creatinine levels, and/or a monoclonal component >15 g/l. Multiple myeloma tends to be associated with IgG or IgA MG, and Waldenstrom's macroglobulinemia with IgM MG Patients with MGUS do not need treatment but only yearly follow-up (symptoms, protein electrophoresis, hemoglobin, calcium and creatinine assay), as the estimated annual risk of malignant transformation is about 1 %. Factors predictive of malignant transformation include the type of serum monoclonal protein, the monoclonal protein concentration, bone marrow plasmocytosis, and the serum free light chain ratio.

摘要

单克隆丙种球蛋白病(MG)影响约1%的普通人群,在老年人群中的患病率更高。意义未明的单克隆丙种球蛋白病(MGUS)是最常见的类型,无症状,且血红蛋白、钙和肌酐水平正常,单克隆成分低于15 g/l。有临床表现和/或血红蛋白、钙或肌酐水平异常,和/或单克隆成分>15 g/l的患者应怀疑为B细胞肿瘤。多发性骨髓瘤往往与IgG或IgA MG相关,而华氏巨球蛋白血症与IgM MG相关。MGUS患者无需治疗,仅需每年随访(症状、蛋白电泳、血红蛋白、钙和肌酐检测),因为估计每年恶变风险约为1%。预测恶变的因素包括血清单克隆蛋白类型、单克隆蛋白浓度、骨髓浆细胞增多症和血清游离轻链比值。

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