Bagic Anto, Lupu Vitalie D, Kessler Craig M, Tornatore Carlo
Department of Neurology & Neurosurgery, University of Pittsburgh Medical School, Pittsburgh, PA 15213, USA.
J Neurooncol. 2007 Jul;83(3):325-8. doi: 10.1007/s11060-007-9334-2. Epub 2007 Feb 14.
Patients with B-cell chronic lymphocytic leukemia (CLL) have an increased risk of second malignancy and may develop diffuse large-cell non-Hodgkin's lymphoma (DLCL) also known as Richter's syndrome (RS). Only seven cases of isolated brain RS without evidence of systemic lymphoma have been reported to date. We describe a case of isolated DLCL of the brain in a 58-year-old female patient with a 3 year history of B-cell CLL. The patient presented with falls due to left leg paresis and showed non-specific neuroimaging findings. Brain biopsy confirmed the diagnosis of DLCL and CLL restaging failed to demonstrate evidence of RS outside the CNS. The patient was treated with whole brain radiation therapy and was discharged 4 weeks later on weekly rituximab infusions. This report extends the previous experience by providing a detailed clinical, neuroradiological and pathological description of isolated RS of the brain. Early identification and timely CNS directed treatment may alter morbidity and positively influence quality of life.
B 细胞慢性淋巴细胞白血病（CLL）患者发生第二原发恶性肿瘤的风险增加，可能会发展为弥漫性大细胞非霍奇金淋巴瘤（DLCL），也称为 Richter 综合征（RS）。迄今为止，仅报道了 7 例无系统性淋巴瘤证据的孤立性脑 RS 病例。我们描述了一例 58 岁女性患者，患有 3 年 B 细胞 CLL 病史，发生了孤立性脑 DLCL。患者因左腿无力而跌倒，神经影像学检查结果无特异性。脑活检确诊为 DLCL，CLL 再分期未能显示中枢神经系统外存在 RS 的证据。患者接受了全脑放射治疗，4 周后出院，每周输注利妥昔单抗。本报告通过提供孤立性脑 RS 的详细临床、神经放射学和病理学描述，扩展了以往的经验。早期识别和及时的中枢神经系统定向治疗可能会改变发病率，并对生活质量产生积极影响。
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