Lymphomatoid granulomatosis involving central nervous system successfully treated with rituximab alone
Lymphomatoid granulomatosis involving central nervous system successfully treated with rituximab alone
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DOI：
10.1001/archneur.65.5.662
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发表时间：
2008-05-01
影响因子：
--
通讯作者：
Tsuji, Shoji
中科院分区：
文献类型：
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作者：
Ishiura, Hiroyuki;Morikawa, Masato;Tsuji, Shoji
目的：报告使用利妥昔单抗（抗CD 20单克隆抗体）成功治疗1例淋巴瘤样肉芽肿病（LYG），这是一种罕见的EB病毒阳性淋巴组织增生性疾病。据报道，LYG的预后很差，目前还没有令人满意的治疗方法。由于已知LYG累及中枢神经系统（CNS）预后较差，因此需要建立一种有效治疗中枢神经系统LYG且副作用轻微的治疗方法。设计：病例报告。设定：大学医院，病人：一名48岁的日本男性，表现为缓慢进行性痉挛性下肢轻瘫，诊断为累及中枢神经系统和肺部的LYG。干预措施：患者接受利妥昔单抗治疗（375 mg/m2，每周一次，持续1个月）。主要结果测量：影像学上病变的改善。结果：利妥昔单抗单药治疗后，神经系统体征消退，CNS和肺部病变大部分减轻，无任何不良反应。患者在缓解18 months.Conclusion：利妥昔单抗单药治疗患者是有效的，因此，利妥昔单抗应被视为对LYG涉及中枢神经系统的初始治疗。
Objective: To report the successful treatment of a patient with lymphomatoid granulomatosis (LYG), a rare Epstein-Barr virus - positive lymphoproliferative disorder, using rituximab (anti-CD20 monoclonal antibody). The prognosis for LYG has been reported to be poor, and no satisfactory treatment has been established. Because central nervous system (CNS) involvement of LYG has been known to show poor prognosis, the establishment of an effective treatment for CNS LYG with mild adverse effects is desired.Design: Case report. Setting: University hospital.Patient: A 48-year-old Japanese man presenting with slowly progressive spastic paraparesis diagnosed as LYG involving the CNS and lungs.Interventions: The patient was treated with rituximab (375 mg/m(2), once weekly for 1 month) alone.Main Outcome Measure: Improvement of the lesions on imaging.Results: The neurological signs resolved and the lesions in the CNS and lungs were mostly diminished after the rituximab monotherapy without any adverse effects. The patient stayed in remission for 18 months.Conclusion: Rituximab monotherapy was effective in treating the patient; hence, rituximab should be considered as the initial treatment against LYG involving the CNS.