Ríos-Tamayo Rafael, Paiva Bruno, Lahuerta Juan José, López Joaquín Martínez, Duarte Rafael F
Hospital Universitario Puerta de Hierro, Fundación para la Investigación Biomédica del Hospital Universitario Puerta de Hierro-Majadahonda, 28222 Majadahonda, Spain.
Centro de Investigación Médica Aplicada, Instituto de Investigación Sanitaria de Navarra, Clínica Universidad de Navarra, 31008 Pamplona, Spain.
Cancers (Basel). 2022 Oct 26;14(21):5247. doi: 10.3390/cancers14215247.
Monoclonal gammopathies of clinical significance (MGCSs) represent a group of diseases featuring the association of a nonmalignant B cells or plasma cells clone, the production of an M-protein, and singularly, the existence of organ damage. They present a current framework that is difficult to approach from a practical clinical perspective. Several points should be addressed in order to move further toward a better understanding. Overall, these entities are only partially included in the international classifications of diseases. Its definition and classification remain ambiguous. Remarkably, its real incidence is unknown, provided that a diagnostic biopsy is mandatory in most cases. In fact, amyloidosis AL is the final diagnosis in a large percentage of patients with renal significance. On the other hand, many of these young entities are syndromes that are based on a dynamic set of diagnostic criteria, challenging a timely diagnosis. Moreover, a specific risk score for progression is lacking. Despite the key role of the clinical laboratory in the diagnosis and prognosis of these patients, information about laboratory biomarkers is limited. Besides, the evidence accumulated for many of these entities is scarce. Hence, national and international registries are stimulated. In particular, IgM MGCS deserves special attention. Until now, therapy is far from being standardized, and it should be planned on a risk and patient-adapted basis. Finally, a comprehensive and coordinated multidisciplinary approach is needed, and specific clinical trials are encouraged.
具有临床意义的单克隆丙种球蛋白病（MGCSs）是一组疾病，其特征为非恶性B细胞或浆细胞克隆、M蛋白的产生，以及器官损害的存在。它们呈现出一个从实际临床角度难以处理的当前框架。为了进一步加深理解，应解决几个要点。总体而言，这些实体仅部分包含在国际疾病分类中。其定义和分类仍不明确。值得注意的是，由于大多数情况下必须进行诊断性活检，其实际发病率尚不清楚。事实上，在很大比例具有肾脏意义的患者中，AL型淀粉样变性是最终诊断。另一方面，这些年轻的实体中有许多是基于一系列动态诊断标准的综合征，对及时诊断构成挑战。此外，缺乏用于评估病情进展的特定风险评分。尽管临床实验室在这些患者的诊断和预后中起着关键作用，但关于实验室生物标志物的信息有限。此外，针对这些实体中许多疾病积累的证据很少。因此，鼓励建立国家和国际登记系统。特别是，IgM MGCS值得特别关注。到目前为止，治疗远未标准化，应根据风险和患者情况进行个体化规划。最后，需要一种全面且协调的多学科方法，并鼓励开展特定的临床试验。
