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Neurolymphomatosis, a rare manifestation of peripheral nerve involvement in lymphomas: Suggestive features and diagnostic challenges.
Journal of the Peripheral Nervous System ( IF 3.8 ) Pub Date : 2020-07-27 , DOI: 10.1111/jns.12401
Marta Campagnolo 1 , Mario Cacciavillani 2 , Tiziana Cavallaro 3 , Sergio Ferrari 3 , Roberto Gasparotti 4 , Renato Zambello 5 , Chiara Briani 1
Affiliation  

Neurolymphomatosis, the infiltration of the peripheral nervous system from lymphoid cells, represents an uncommon manifestation of lymphomas. We describe the challenging diagnostic work‐up in a patient with neurolymphomatosis. A 58‐year‐old woman with previous breast diffuse large B‐cell lymphoma treated with chemo‐ and radiation‐therapy, presented with dysesthesias, neuropathic pain at left abdomen and thigh, and weakness at left lower limb 9 years after disease remission. Neurophysiology revealed left T10‐L4 radiculo‐plexopathy with no abnormalities at cerebrospinal fluid (CSF), nerve ultrasound, and 18fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT). MR‐neurography disclosed left rectus abdominis muscle atrophy, neurogenic edema, and denervation. Radiation‐induced damage, paraneoplastic, infectious radiculo‐plexopathies, and atypical chronic inflammatory demyelinating polyradiculoneuropathy were ruled out. Neurolymphomatosis was suspected, and the patient treated with rituximab with improvement. Despite treatment, the radiculo‐plexopathy eventually extended to the right side and sacral roots. Later in the disease course, sural nerve biopsy confirmed the diagnosis. Maintenance therapy was continued, until cutaneous localizations occurred, requiring salvage therapy and autologous stem cell transplant. Although rare, neurolymphomatosis should be considered in all patients with lymphomas and unexplained peripheral nervous system involvement. Hematological, CSF, and neuroimaging findings may be unremarkable, and a high index of suspicion required in order to achieve the diagnosis.

中文翻译:

神经淋巴瘤病,淋巴瘤中外周神经受累的一种罕见表现:提示特征和诊断挑战。

神经淋巴瘤病是淋巴样细胞浸润周围神经系统,是淋巴瘤的一种罕见表现。我们描述了一名神经淋巴瘤患者的具有挑战性的诊断检查。一名 58 岁女性,既往患有乳腺弥漫性大 B 细胞淋巴瘤,接受化疗和放疗,在疾病缓解 9 年后出现感觉迟钝、左腹部和大腿神经性疼痛以及左下肢无力。神经生理学显示左侧 T10-L4 神经根丛病,脑脊液 (CSF)、神经超声和18氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(FDG PET/CT)。MR 神经造影显示左腹直肌萎缩、神经源性水肿和去神经支配。排除了辐射引起的损伤、副肿瘤性、感染性神经根神经病和非典型慢性炎症性脱髓鞘性多发性神经根神经病。怀疑有神经淋巴瘤病,患者用利妥昔单抗治疗后好转。尽管进行了治疗,但神经根神经丛病最终会扩展到右侧和骶骨根部。在病程后期,腓肠神经活检证实了诊断。继续维持治疗,直到发生皮肤定位,需要挽救治疗和自体干细胞移植。虽然很少见,所有患有淋巴瘤和不明原因周围神经系统受累的患者都应考虑神经淋巴瘤病。血液学、脑脊液和神经影像学检查结果可能不显着,需要高度怀疑才能做出诊断。
更新日期:2020-07-27
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