Rahim A Jiwani, Joseph R Liput, Attah Abraham, Khaled Alhamad, Mukta Kapdi, Renan Mota, Kayla Forte, John R McGill, Jasper C Acer, Palgun Nisarga, Nicholas R Jaeger, Santhosh Sadashiv, Prerna Mewawalla
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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 <i>c-MYC</i> (chromosome 8) and the lambda light chain gene <i>IGL</i> (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. 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引用次数: 0
摘要
多发性骨髓瘤(MM)是一种浆细胞障碍性疾病,其典型特征是血液或尿液中存在可识别的副蛋白。然而,少数无法识别副蛋白的患者被称为非分泌型多发性骨髓瘤(NSM)。由于无法跟踪副蛋白水平,因此对这些患者的治疗反应评估更为困难。由于无法通过传统的血清和尿液测量机制来衡量这些患者的反应,而且与分泌型 MM 相比,这些患者的总生存期似乎更短,因此包括这些患者在内的临床试验非常少。NSM 又分为四个亚组:"非分泌型"、"真性非分泌型"、"少分泌型 "和 "假性非分泌型"。非分泌型 "表型与更具侵袭性的病程有关。在 MM 中,涉及原癌基因 c-MYC(8 号染色体)和λ轻链基因 IGL(22 号染色体)的易位比较罕见,这些易位通常与伯基特淋巴瘤(Burkitt lymphoma)有关。我们描述了一名患有 NSM 的 60 岁男性患者,经鉴定,他具有多种高危特征,包括复杂的细胞遗传学和非生产者表型,而这些特征在传统的 MM 分期和风险分层中并未被考虑在内。该病例强调,我们需要认识到与较高临床风险相关的表型和细胞遗传学,但这些表型和细胞遗传学并未纳入修订后的国际分期系统。
Non-Secretory Multiple Myeloma Associated With High-Risk Phenotype and Complex Cytogenetics Including t(8;22).
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.