Aasfara Jehanne, Guessous Fadila, Al Bouzidi Abderahmane, Ouhabi Hamid, Schiff David
Neurology, Mohammed VI University of Health Sciences (UM6SS) / Cheikh Khalifa International University Hospital, Casablanca, MAR.
Biological Sciences, Faculty of Medicine, Mohammed VI University of Health Sciences (UM6SS), Casablanca, MAR.
Cureus. 2021 Jan 17;13(1):e12749. doi: 10.7759/cureus.12749.
Intravascular large B cell lymphoma (IVLBCL) is a rare form of extranodal non-Hodgkin's lymphoma, usually of B-cell lineage. Several organs are affected, most commonly the skin and the nervous system. We report a case of a 52-year-old man, with no medical history admitted with a five-month history of back pain with lower extremity numbness and tingling evolved to weakness associated with urinary retention, constipation and abdominal pain. Spinal magnetic resonance imaging (MRI) showed a gadolinium-enhancing lesion in the conus medullaris (CM). Electromyography (EMG) and nerve conduction velocity (NCV) test was consistent with demyelinating polyradiculoneuropathy in lower limbs. Slight clinical improvement with corticosteroids was observed. Three months after discharge, he presented a generalized tonic-clonic seizure. Cerebral MRI showed patchy lesions in the subcortical white matter with infiltration of the internal table of the skull with elevated serum lactate dehydrogenase (LDH). Calvarial biopsy revealed an intravascular large B-cell lymphoma. Treatment with cyclophosphamide and high-dose corticosteroids was initiated but the patient developed impaired consciousness and died of respiratory and circulatory failure six weeks after his readmission. Intravascular large B cell lymphoma should be considered in patients with a rapidly progressive severe encephalomyeloradiculoneuropathy. A biopsy of involved organs including the brain should not be delayed when IVLBCL is suspected, to initiate prompt systemic therapy.
血管内大B细胞淋巴瘤(IVLBCL)是一种罕见的结外非霍奇金淋巴瘤,通常为B细胞谱系。多个器官会受到影响,最常见的是皮肤和神经系统。我们报告一例52岁男性病例,该患者无病史,因背痛伴下肢麻木和刺痛5个月入院,后发展为伴有尿潴留、便秘和腹痛的肌无力。脊髓磁共振成像(MRI)显示圆锥马尾(CM)有钆增强病变。肌电图(EMG)和神经传导速度(NCV)测试结果与下肢脱髓鞘性多发性神经根神经病相符。观察到使用皮质类固醇后临床症状略有改善。出院3个月后,他出现全身性强直阵挛发作。脑部MRI显示皮质下白质有斑片状病变,颅骨内板有浸润,血清乳酸脱氢酶(LDH)升高。颅骨活检显示为血管内大B细胞淋巴瘤。开始使用环磷酰胺和大剂量皮质类固醇进行治疗,但患者意识障碍,再次入院6周后死于呼吸和循环衰竭。对于快速进展的严重脑脊髓神经根神经病患者,应考虑血管内大B细胞淋巴瘤。当怀疑IVLBCL时,不应延迟对包括脑在内的受累器官进行活检,以便及时开始全身治疗。