Sorrentino Anthony D, Kelkar Amar H, Dang Nam H
Internal Medicine, Northwestern University Feinberg School of Medicine, Chicago, USA.
Oncology, University of Florida College of Medicine, Gainesville, USA.
Cureus. 2021 Apr 20;13(4):e14590. doi: 10.7759/cureus.14590.
A 68-year-old man presented with a two-week history of ascending, symmetric, sensory neuropathy concerning an acute inflammatory demyelinating polyneuropathy that briefly responded to intravenous immunoglobulin (IVIg) therapy. The initial workup was negative for acquired causes. After three months of poor response to standard therapies, he was hospitalized for severe disability, unintentional weight loss, and additional, unexplained neurologic symptoms including cerebellar ataxia, dysarthria, and muscle twitching. Positron emission tomography revealed hypermetabolism isolated to the bone marrow. Bone marrow biopsy confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL). Due to rapidly worsening performance status, plasmapheresis was initiated prior to treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy. His symptoms initially improved following plasmapheresis and resolved with chemotherapy. One year following treatment, he remains in complete remission. This case describes a unique paraneoplastic neurologic syndrome involving the central and peripheral nervous system that responded well to plasmapheresis and systemic chemotherapy.
一名68岁男性，有两周进行性、对称性感觉神经病病史，怀疑为急性炎症性脱髓鞘性多发性神经病，静脉注射免疫球蛋白（IVIg）治疗后曾有短暂缓解。初始检查未发现后天性病因。在对标准治疗反应不佳三个月后，他因严重残疾、非故意体重减轻以及包括小脑共济失调、构音障碍和肌肉抽搐等其他无法解释的神经症状而住院。正电子发射断层扫描显示仅骨髓代谢亢进。骨髓活检确诊为弥漫性大B细胞淋巴瘤（DLBCL）。由于患者的功能状态迅速恶化，在使用利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松（R-CHOP）化疗之前先进行了血浆置换。血浆置换后他的症状最初有所改善，并在化疗后得到缓解。治疗一年后，他仍处于完全缓解状态。本病例描述了一种累及中枢和周围神经系统的独特副肿瘤性神经综合征，对血浆置换和全身化疗反应良好。
