多发性骨髓瘤 (MM) 是一种浆细胞恶液质，其典型特征是血液或尿液中可识别的副蛋白。然而，少数无法鉴定副蛋白的患者被指定为非分泌型 MM (NSM)。由于无法跟踪副蛋白水平，因此评估这些患者的治疗反应更加困难。由于无法通过经典的血清和尿液测量机制来测量反应，并且与分泌型 MM 相比总体生存率似乎降低，因此缺乏包括这些患者的临床试验。 NSM 分为四个子组：“非生产者”、“真正的非分泌者”、“寡分泌者”和“假非分泌者”。 “非生产者”表型与更具侵袭性的病程相关。诸如涉及原癌基因 c-MYC（8 号染色体）和 lambda 轻链基因 IGL（22 号染色体）的易位（更常见与伯基特淋巴瘤相关）在 MM 中很少见。我们描述了一名患有 NSM 的 60 岁男性，他被确定具有多种高风险特征，包括复杂的细胞遗传学和非生产者表型，这些特征在传统 MM 分期和风险分层中未考虑。该病例强调需要认识与较高临床风险相关的表型和细胞遗传学，这些风险未包含在修订后的国际分期系统中。版权所有 2024，Jiwani 等人。

Multiple myeloma (MM) is a plasma cell dyscrasia which is typically characterized by identifiable paraprotein in the blood or urine. However, the minority of patients in whom paraprotein cannot be identified are designated non-secretory MM (NSM). Evaluation of treatment response is more difficult in these patients as paraprotein levels cannot be followed. A dearth of clinical trials including these patients exists because of an inability to measure response by classical serum and urine measurement mechanisms as well as seemingly decreased overall survival compared to secretory MM. NSM is subdivided into four subgroups: "non-producers", "true non-secretors", "oligosecretors" and "false non-secretors". The "non-producers" phenotype is associated with more aggressive disease course. Translocations such as those involving the proto-oncogene c-MYC (chromosome 8) and the lambda light chain gene IGL (chromosome 22) - more commonly associated with Burkitt lymphoma - are rare in MM. We describe a 60-year-old male with NSM who was identified as having multiple high-risk features including complex cytogenetics and a non-producer phenotype, which are features not considered in conventional MM staging and risk stratification. This case highlights the need for awareness of phenotypes and cytogenetics associated with higher clinical risk that are not included in the revised International Staging System.Copyright 2024, Jiwani et al.