Northern Ireland Centre for Stratified Medicine, Ulster University, Derry/Londonderry, Northern Ireland.
Department of Haematology, North West Cancer Centre, Altnagelvin Area Hospital, Derry/Londonderry, Northern Ireland.
Haematologica. 2020 Jul;105(7):1780-1790. doi: 10.3324/haematol.2020.248518. Epub 2020 May 15.
Central nervous system involvement in multiple myeloma is a rare complication but carries a very poor prognosis. We provide a review of current literature, including presentation, treatment and survival data, and describe our experience in a regional hematologic malignancy diagnosis center where, over a 15-year period, ten cases were identified. Although the median age of onset, frequently between 50-60 years, is comparatively young, those diagnosed usually have a preceding diagnosis of multiple myeloma and often have had several lines of treatment. We discuss putative underlying factors such as prior treatment and associations including possible risk factors and features suggestive of a distinct biology. Central nervous system involvement may be challenging to diagnose in myeloma, displaying heterogeneous symptoms that can be confounded by neurological symptoms caused by the typical features of myeloma or treatment side-effects. We discuss the clinical features, imaging and laboratory methods used in diagnosis, and highlight the importance of considering this rare complication when neurological symptoms occur at presentation or, more commonly, during the disease pathway. In the absence of clinical trial data to inform an evidence-based approach to treatment, we discuss current and novel treatment options. Finally, we propose the establishment of an International Registry of such cases as the best way to collect and subsequently disseminate presentation, diagnostic and treatment outcome data on this rare complication of multiple myeloma.
多发性骨髓瘤累及中枢神经系统是一种罕见的并发症，但预后极差。我们对当前的文献进行了综述，包括发病表现、治疗和生存数据，并描述了我们在一个区域性血液恶性肿瘤诊断中心的经验，在 15 年的时间里，共发现了 10 例。尽管发病年龄中位数通常在 50-60 岁之间相对年轻，但确诊患者通常之前已经诊断为多发性骨髓瘤，并且经常接受了多种治疗方案。我们讨论了潜在的因素，如先前的治疗以及与多发性骨髓瘤的独特生物学相关的可能风险因素和特征。多发性骨髓瘤中中枢神经系统受累的诊断可能具有挑战性，其表现多样，可能会与多发性骨髓瘤的典型特征或治疗副作用引起的神经症状混淆。我们讨论了诊断中使用的临床特征、影像学和实验室方法，并强调了在出现神经症状时，或更常见于疾病进程中，考虑这种罕见并发症的重要性。由于缺乏临床试验数据来为治疗提供循证方法，我们讨论了当前和新型的治疗选择。最后，我们提出建立这样的病例国际登记处，是收集和随后传播多发性骨髓瘤这一罕见并发症的发病表现、诊断和治疗结果数据的最佳方法。
