Steinhardt Maximilian, Kortüm Martin, Einsele Hermann, Rasche Leo
Medizinische Klinik und Poliklinik II, Universitätsklinikum Würzburg.
Dtsch Med Wochenschr. 2022 Jun;147(11):675-682. doi: 10.1055/a-1664-7353. Epub 2022 May 30.
Monoclonal gammopathies are a frequently diagnosed entity. However, the diagnosis is not always clinically relevant. The diagnosis of a monoclonal gammopathy requires serum electrophoresis, immunofixation and free light chain measurement. Sometimes, monoclonal gammopathies occur in the course of transient or autoimmune inflammation. Further diagnostics should only be performed after risk assessment according to Mayo criteria. In non-low risk patients, a symptomatic myeloma has to be ruled out via SLiM-CRAB criteria. The diagnostic work-up should include whole-body MRI and a bone marrow puncture as well as a 24 h urine sample. If it does not imply myeloma, the diagnosis of MGUS is confirmed and a follow-up after 6 months is recommended. After that, low-risk patients only need SLiM-CRAB screening at clinical signs of progression. All other patients should receive serologic follow-ups once a year. Importantly, MGUS patients show higher morbidity. Amongst a higher prevalence of osteoporosis and immunodeficiency, a wide array of MGUS-associated diseases such as AL amyloidosis, deposition diseases and Fc binding-dependent effects can occur. This article gives an overview over the work-up, observation and caveats of monoclonal gammopathy of (un)known significance.
单克隆丙种球蛋白病是一种常被诊断出的病症。然而,该诊断在临床上并不总是具有相关性。单克隆丙种球蛋白病的诊断需要血清电泳、免疫固定和游离轻链检测。有时,单克隆丙种球蛋白病会在短暂性或自身免疫性炎症过程中出现。只有根据梅奥标准进行风险评估后,才应进一步开展诊断。对于非低风险患者,必须通过SLiM-CRAB标准排除症状性骨髓瘤。诊断检查应包括全身MRI、骨髓穿刺以及24小时尿液样本检测。如果排除了骨髓瘤,则确诊为意义未明的单克隆丙种球蛋白病(MGUS),建议6个月后进行随访。之后,低风险患者仅在出现病情进展的临床体征时需要进行SLiM-CRAB筛查。所有其他患者应每年接受血清学随访。重要的是,MGUS患者的发病率较高。除骨质疏松症和免疫缺陷的患病率较高外,还可能发生一系列与MGUS相关的疾病,如AL淀粉样变性、沉积性疾病和Fc结合依赖性效应。本文概述了意义未明(或已知)单克隆丙种球蛋白病的检查、观察要点及注意事项。