Jurczyszyn Artur, Grzasko Norbert, Gozzetti Alessandro, Czepiel Jacek, Cerase Alfonso, Hungria Vania, Crusoe Edvan, Silva Dias Ana Luiza Miranda, Vij Ravi, Fiala Mark A, Caers Jo, Rasche Leo, Nooka Ajay K, Lonial Sagar, Vesole David H, Philip Sandhya, Gangatharan Shane, Druzd-Sitek Agnieszka, Walewski Jan, Corso Alessandro, Cocito Federica, Vekemans Marie-Christine M, Atilla Erden, Beksac Meral, Leleu Xavier, Davila Julio, Badros Ashraf, Aneja Ekta, Abildgaard Niels, Kastritis Efstathios, Fantl Dorotea, Schutz Natalia, Pika Tomas, Butrym Aleksandra, Olszewska-Szopa Magdalena, Usnarska-Zubkiewicz Lidia, Usmani Saad Z, Nahi Hareth, Chim Chor S, Shustik Chaim, Madry Krzysztof, Lentzsch Suzanne, Swiderska Alina, Helbig Grzegorz, Guzicka-Kazimierczak Renata, Lendvai Nikoletta, Waage Anders, Andersen Kristian T, Murakami Hirokazu, Zweegman Sonja, Castillo Jorge J
Jagiellonian University Medical College, Cracow, Poland.
Department of Hematooncology and Bone Marrow Transplantation, Medical University of Lublin, Lublin, Poland.
Am J Hematol. 2016 Jun;91(6):575-80. doi: 10.1002/ajh.24351. Epub 2016 Apr 24.
The multicenter retrospective study conducted in 38 centers from 20 countries including 172 adult patients with CNS MM aimed to describe the clinical and pathological characteristics and outcomes of patients with multiple myeloma (MM) involving the central nervous system (CNS). Univariate and multivariate analyses were performed to identify prognostic factors for survival. The median time from MM diagnosis to CNS MM diagnosis was 3 years. Thirty-eight patients (22%) were diagnosed with CNS involvement at the time of initial MM diagnosis and 134 (78%) at relapse/progression. Upon diagnosis of CNS MM, 97% patients received initial therapy for CNS disease, of which 76% received systemic therapy, 36% radiotherapy and 32% intrathecal therapy. After a median follow-up of 3.5 years, the median overall survival (OS) from the onset of CNS involvement for the entire group was 7 months. Untreated and treated patients had median OS of 2 and 8 months, respectively (P < 0.001). At least one previous line of therapy for MM before the diagnosis of CNS disease and >1 cytogenetic abnormality detected by FISH were independently associated with worse OS. The median OS for patients with 0, 1 and 2 of these risk factors were 25 months, 5.5 months and 2 months, respectively (P < 0.001). Neurological manifestations, not considered chemotherapy-related, observed at any time after initial diagnosis of MM should raise a suspicion of CNS involvement. Although prognosis is generally poor, the survival of previously untreated patients and patients with favorable cytogenetic profile might be prolonged due to systemic treatment and/or radiotherapy. Am. J. Hematol. 91:575-580, 2016. © 2016 Wiley Periodicals, Inc.
一项在来自20个国家的38个中心开展的多中心回顾性研究纳入了172例成年中枢神经系统多发性骨髓瘤（CNS MM）患者，旨在描述累及中枢神经系统（CNS）的多发性骨髓瘤（MM）患者的临床和病理特征及预后。进行单因素和多因素分析以确定生存的预后因素。从MM诊断到CNS MM诊断的中位时间为3年。38例患者（22%）在初始MM诊断时被诊断为CNS受累，134例（78%）在复发/进展时被诊断为CNS受累。在诊断为CNS MM时，97%的患者接受了CNS疾病的初始治疗，其中76%接受了全身治疗，36%接受了放疗，32%接受了鞘内治疗。中位随访3.5年后，整个队列从CNS受累开始的中位总生存期（OS）为7个月。未治疗和已治疗患者的中位OS分别为2个月和8个月（P＜0.001）。在CNS疾病诊断前至少接受过一线MM治疗以及通过荧光原位杂交（FISH）检测到＞1种细胞遗传学异常与较差的OS独立相关。具有0、1和2个这些危险因素的患者的中位OS分别为25个月、5.5个月和2个月（P＜0.001）。在MM初始诊断后的任何时间观察到的与化疗无关的神经学表现应引起对CNS受累的怀疑。尽管总体预后较差，但由于全身治疗和/或放疗，既往未治疗患者和具有良好细胞遗传学特征患者的生存期可能会延长。《美国血液学杂志》91:575 - 580，2016年。©2016威利期刊公司
