Viera Sandecká, Ludek Pour, Zdeněk Adam, Marta Krejčí, Martin Štork, Sabina Ševčíková, Zdeněk Král
Klin Onkol. 2018 Summer;31(4):270-276. doi: 10.14735/amko2018270.
Monoclonal gammopathy of undetermined significance (MGUS) is one of the most prevalent premalignant conditions associated with a risk of malignant transformation to multiple myeloma (MM) or other forms of lymphoproliferative disorders with risk of progression of approximately 1% per year. IgG and IgA MGUS are precursor conditions of multiple myeloma (MM), whereas light-chain MGUS is a precursor condition of light chain MM. IgM MGUS is a precursor condition of Waldenström macroglobulinemia (MW) or other lymphoproliferative diseases.
Assessment of the risk of progression of patients with asymptomatic monoclonal gammopathies (MG) is based on various factors, including the serum paraprotein (M protein) concentration, isotype of M protein, serum free light chain ratio, infiltration of bone marrow plasmocytes, reduction of one or two noninvolved immunoglobulin subtype levels (immunoparesis), evolving and non-evolving subtype of MGUS, ratio of normal/abnormal plasma cells in bone marrow identified by multiparametric flow cytometry techniques and number of circulating plasma cells in peripheral blood. Three risk stratification models have been constructed that are useful in daily practice for predicting risk of progression of MGUS into malignant forms of monoclonal gammopathy - MAYO, PETHEMA and CMG model. The goal of all three models is to identify correctly prognostic markers that can divide patients into low-risk MGUS and high-risk MGUS groups.
This review provides a look at the definition, pathogenesis, diagnostic algorithm, clinical significance and stratification of MGUS patients, followed by recommendations for patient risk dispensarisation intervals.
意义未明的单克隆丙种球蛋白病(MGUS)是最常见的癌前病变之一,有转化为多发性骨髓瘤(MM)或其他形式淋巴增殖性疾病的风险,每年进展风险约为1%。IgG和IgA MGUS是多发性骨髓瘤(MM)的前驱病变,而轻链MGUS是轻链MM的前驱病变。IgM MGUS是华氏巨球蛋白血症(WM)或其他淋巴增殖性疾病的前驱病变。
评估无症状单克隆丙种球蛋白病(MG)患者的进展风险基于多种因素,包括血清副蛋白(M蛋白)浓度、M蛋白的同种型、血清游离轻链比值、骨髓浆细胞浸润、一种或两种未受累免疫球蛋白亚型水平降低(免疫球蛋白缺乏)、MGUS的进展型和非进展型、通过多参数流式细胞术技术确定的骨髓中正常/异常浆细胞比例以及外周血中循环浆细胞数量。已经构建了三种风险分层模型,在日常实践中可用于预测MGUS进展为恶性单克隆丙种球蛋白病的风险——MAYO、PETHEMA和CMG模型。所有这三种模型的目标是正确识别可将患者分为低风险MGUS和高风险MGUS组的预后标志物。
本综述探讨了MGUS患者的定义、发病机制、诊断算法、临床意义和分层,随后给出了患者风险分层间隔的建议。