原发性脊髓髓内淋巴瘤病例分析及文献复习

发布时间：2018-01-20 10:27

本文关键词： 淋巴瘤脊髓核磁共振成像脑脊液治疗　出处：《浙江大学》2017年硕士论文　论文类型：学位论文

【摘要】：目的:报告我科诊断的原发性脊髓髓内淋巴瘤(primary intramedullary spinal cord lymphoma,PISCL)1例,并结合国内外PISCL相关病例报道综合分析PISCL的临床表现、影像学检查、脑脊液检查、病理组织学特点,提高对PISCL的认识,为临床早期识别及诊断提供帮助。方法:报告我科确诊的PISCL 1例,同时对相关期刊论文和pubmed数据库自1976年至今收录发表的经病理确诊且有相关临床资料的PISCL的病例进行回顾性研究,分析其临床表现、影像学检查、脑脊液检查、病理组织学等特点。结果:病例:一位47岁男性因肩颈部酸痛1周来我院就诊,入院后进行性出现左侧肢体无力,左侧周围性面瘫,尿便障碍,左眼视觉障碍等症状。头颅及颈椎增强MRI提示C2～T1水平脊髓、双侧丘脑、侧脑室旁、基底节区、大脑脚多发异常信号灶。经激素、免疫球蛋白、免疫抑制剂及血浆二次分离治疗后效果不佳,最终行脑组织活检病理提示弥漫大B细胞型淋巴瘤。患者随后接受3次大剂量甲氨蝶呤化疗,但疗效欠佳。文献复习:经病理确诊的27例PISCL患者,男女比例14:13,中位数年龄52岁;67%的患者出现双下肢或四肢无力,22%出现偏侧或单个肢体无力;85%的患者出现感觉障碍包括肢体、会阴部麻木及肩背部疼痛,41%的患者出现尿便障碍。89%的患者脊髓病灶位于颈胸段,11%位于腰段或胸腰段;91%的患者病灶在T2WI表现为高信号,76%伴随脊髓肿胀,增强后100%患者病灶出现强化。91%患者脑脊液蛋白水平升高,83%患者有核细胞数升高。85%患者经手术活检确诊,48%为B细胞来源淋巴瘤,其中弥漫大B细胞型占26%。各病例报道采用的化疗方案及放疗剂量各不相同。结论:PISCL为罕见病,多以脊髓病变症状起病,症状无特异性,早期影像学表现不典型,容易误诊。对激素、免疫球蛋白治疗效果不佳或短时间缓解后复发,病灶持续强化的脊髓病变患者应警惕PISCL的可能。
[Abstract]:Objective: to report primary intramedullary spinal cord lymphoma diagnosed in our department. Cases of PISCL)1, and combined with domestic and foreign cases of PISCL related cases of comprehensive analysis of PISCL clinical manifestations, imaging examination, cerebrospinal fluid examination, histopathological characteristics. To improve the understanding of PISCL and to provide help for early clinical identification and diagnosis. Methods: 1 case of PISCL confirmed in our department was reported. At the same time, a retrospective study was made on the cases of PISCL with pathologically confirmed and relevant clinical data published in the full text database of Chinese periodicals and pubmed database since 1976. The clinical manifestation, imaging examination, cerebrospinal fluid examination and histopathology were analyzed. Results: a 47-year-old male was admitted to our hospital for one week due to shoulder and neck soreness. Progressive left limb weakness, left peripheral facial paralysis, urinary defecation, visual impairment of left eye and so on. Enhanced MRI of head and cervical spine indicated C2T 1 level spinal cord, bilateral thalamus, lateral ventricle. Basal ganglia, multiple abnormal signal foci in the foot of brain. After treatment with hormone, immunoglobulin, immunosuppressant and plasma, the effect is not good. The final biopsy showed diffuse large B-cell lymphoma. The patient received three times of high dose methotrexate chemotherapy, but the efficacy was not good. Literature review: 27 cases of PISCL confirmed by pathology. Male to female ratio 14: 13, median age 52 years; 67% of the patients developed bilateral lower limb or limb weakness. 22% of the patients developed unilateral or single limb weakness. 85% of the patients had sensory disorders, including limbs, perineal numbness, shoulder and back pain, and 41% of the patients had urinary defecation disorder .89% of the spinal cord lesions were located in the cervical and thoracic segments, 11% were located in the lumbar or thoracolumbar segments. 91% of the lesions showed hyperintense spinal cord swelling in 76% of the lesions on T2WI, and increased CSF protein level in 100% patients after enhancement. 83% patients with increased number of nucleated cells. 85% of the patients were diagnosed as B-cell-derived lymphoma by biopsy. The proportion of diffuse large B cell type is 26%. The dosage of chemotherapy and radiotherapy are different in each case. Conclusion: the disease is rare, most of which are caused by the symptoms of spinal cord disease, and the symptoms are not specific. [WT5HZ] [WT5 "HZ] [WT5" HZ] [WT5 "BZ]. Early imaging findings were atypical and easy to misdiagnose. Patients with spinal cord lesions with persistent enhancement of lesions should be alert to the possibility of PISCL if the effect of hormone and immunoglobulin therapy is not good or relapse after short time remission.
【学位授予单位】：浙江大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R739.42

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