Yunoki Masatoshi, Suzuki Kenta, Uneda Atsuhito, Yoshino Kimihiro
Department of Neurosurgery, Kagawa Rosai Hospital, Kagawa, Japan.
Surg Neurol Int. 2015 Aug 20;6(Suppl 13):S367-70. doi: 10.4103/2152-7806.163316. eCollection 2015.
Intravascular lymphoma (IVL) is a rare subtype of non-Hodgkin lymphoma with exclusively or predominantly intravascular proliferation. Without therapeutic intervention, the neurologic involvement is rapidly progressive and inevitably fatal. Most of the IVL patients have prominent or exclusive manifestations in the nervous system and there are several reports of patients presenting with spinal symptoms.
A 68-year-old male patient admitted with the complaints of progressive paraparesis. T2-weighted magnetic resonance imaging (MRI) of the spinal cord showed hyperintense lesions in the thoracic cord. A diagnosis of myelitis of unknown etiology was assumed, and steroid pulse therapy was administered, which temporarily improved the patient's symptoms. However, the paraparesis recurred, and other symptoms, such as vertigo, psychosis, and seizures, developed 1-month after the initial treatment. Multiple high-intensity lesions were detected in the bilateral subcortical white matter on DW MRI. Based on the patient's clinical course, IVL was suspected; however, obtaining histological confirmation was not possible, as no Gd-enhanced brain or spinal lesions were identified and repeated cerebrospinal fluid examinations were negative for tumor cells. Therefore, a random skin biopsy was performed, and IVL was diagnosed. Obtaining a comparatively favorable outcome was possible owing to the subsequent administration of R-CHOP chemotherapy.
IVL should be included in the differential diagnosis of atypical case of presumed myelitis. An early diagnosis and chemotherapy is crucial for improving the patient's outcome. When obtaining a diagnosis based on tissues other than skin is difficult, a random skin biopsy should be considered in patients with suspected IVL.
血管内淋巴瘤(IVL)是非霍奇金淋巴瘤的一种罕见亚型,具有完全或主要的血管内增殖特征。若不进行治疗干预,神经系统受累会迅速进展且必然致命。大多数IVL患者在神经系统有突出或唯一表现,且有几例患者表现出脊髓症状的报道。
一名68岁男性患者因进行性双下肢轻瘫入院。脊髓的T2加权磁共振成像(MRI)显示胸段脊髓有高信号病变。初步诊断为病因不明的脊髓炎,并给予类固醇冲击治疗,患者症状暂时改善。然而,双下肢轻瘫复发,且在初始治疗1个月后出现了眩晕、精神病和癫痫等其他症状。扩散加权成像(DW MRI)显示双侧皮质下白质有多个高强度病变。根据患者的临床病程,怀疑为IVL;然而,由于未发现钆增强的脑部或脊髓病变,且多次脑脊液检查肿瘤细胞均为阴性,无法获得组织学确诊。因此,进行了随机皮肤活检,确诊为IVL。由于随后给予了R-CHOP化疗,患者获得了相对较好的预后。
IVL应纳入疑似脊髓炎非典型病例的鉴别诊断。早期诊断和化疗对改善患者预后至关重要。当难以通过皮肤以外的组织进行诊断时,对于疑似IVL的患者应考虑进行随机皮肤活检。