Wavre Aurelia, Baur Audrey S, Betz Michael, Mühlematter Dominique, Jotterand Martine, Zaman Khalil, Ketterer Nicolas
Department of Oncology, University Hospital CHUV , Lausanne, Switzerland.
Neuro Oncol. 2007 Jul;9(3):370-2. doi: 10.1215/15228517-2007-008. Epub 2007 May 23.
Cerebral involvement is an uncommon complication of multiple myeloma. We report on a 64-year-old man hospitalized for a partial seizure. MRI showed two intracerebral lesions, which proved to be plasmacytomas. After complete staging, we retained the diagnosis of immunoglobulin G lambda-type multiple myeloma with CNS involvement. Cytogenetic analysis of plasma cells detected a deletion in the p53 gene at 17p13.1. Despite cranial radiotherapy and systemic chemotherapy, the patient's disease progressed rapidly and he died five months after diagnosis. What makes this case unusual is that overt multiple myeloma had been absent before cerebral involvement was discovered. It confirms the extremely poor prognosis of patients with CNS myeloma even in the presence of aggressive treatment. Cytogenetic abnormalities could be a marker of chromosomal and genetic instability, conferring to multiple myeloma a more aggressive profile.
脑部受累是多发性骨髓瘤一种不常见的并发症。我们报告一名64岁男性因部分性癫痫发作住院。磁共振成像(MRI)显示两个脑内病变,经证实为浆细胞瘤。全面分期后,我们确诊为免疫球蛋白G λ型多发性骨髓瘤伴中枢神经系统受累。对浆细胞进行的细胞遗传学分析检测到17号染色体短臂13.1位置的p53基因缺失。尽管进行了颅脑放疗和全身化疗,患者病情仍迅速进展,确诊后五个月死亡。该病例的不同寻常之处在于,在发现脑部受累之前并无明显的多发性骨髓瘤。这证实了即使接受积极治疗,中枢神经系统骨髓瘤患者的预后仍极差。细胞遗传学异常可能是染色体和基因不稳定的一个标志物,使多发性骨髓瘤具有更具侵袭性的特征。