李园园1，谢瑞坤2，张允1，肖正光3，都爱莲1
1.上海交通大学医学院附属同仁医院神经内科，200336；2.台北马偕纪念医院血液肿瘤科；3. 上海交通大学医学院附属同仁医院放射科
通讯作者：都爱莲，Email：lotusdu@126.com
【摘要】目的 回顧性分析一例難治性淋巴瘤樣肉芽腫（LYG）患者的臨床、病理和影像學特點，並報導應用苯達莫司汀（Bendamustine）聯合利妥昔單抗(Rituximab)成功治療的過程。方法分析患者的病史、體格檢查、影像學特點，肺部結節穿刺活檢經HE染色、腫瘤和炎症相關免疫組化染色、Epstein-Barr病毒原位雜交等染色，報導治療過程。結果 患者女性，67歲，主因“雙下肢無力3月伴眼球活動受限3天“就診。體格檢查：時間空間定向力減退，雙眼水平眼震伴外展受限，雙手指鼻不准，雙下肢肌力3-4級，雙下肢腱反射亢進，雙側巴氏徵陽性，EDSS 8分。頭及脊髓MR增強：雙側大腦半球及腦幹、頸段脊髓多發散在顆粒狀異常信號伴強化。 PET-CT見兩肺多發結節伴放射性攝取增高。肺穿刺活檢符合淋巴瘤樣肉芽腫病理表現。給予甲強龍(Methylprednisolone)衝擊治療明顯好轉但減量到40mg就復發，給環磷酰胺(Cyclophosphamide)未見好轉，苯達莫司汀（Bendamustine）100mg/月+利妥昔單抗(Rituximab) 500mg/月連續治療6個月，患者的臨床症狀和影像學增強病灶均得到明顯緩解。結論：淋巴瘤樣肉芽腫可以出現腦、脊髓、肺結節等多系統損害，苯達莫司汀（Bendamustine）聯合利妥昔單抗(Rituximab)可有效治療難治性淋巴瘤樣肉芽腫。
【關鍵詞】淋巴瘤樣肉芽腫；腦和脊髓；肺結節；苯達莫司汀（Bendamustine）；利妥昔單抗(Rituximab)；治療。
基金项目：国家自然科学基金（81971181）；长宁区科学技术委员会基金（CNKW2017Y02, CNKW2018Y01）；上海市同仁医院院级课题（TRYJ201705, TRYJ(JC)201803）
Clinical characteristics and successful treatment of 1 case of refractory lymphomatoid granulomatosis with bendamoxetine and rituximab.
Li Yuanyuan 1, Xie Ruikun 2, Zhang Yun 1, Xiao Zhengguang 3, Du Ailian 1
- Department of Neurology, Tongren Hospital, School of medicine, Shanghai Jiaotong University, 200336; 2. Department of hematologic oncology, mackay memorial hospital, Taipei; 3. Department of Radiology, Tongren Hospital, School of medicine, Shanghai Jiaotong University
Corresponding author: Du Ailian, email: lotusdu@126.com
【Abstract】 Objective To analyze the clinical, pathological and imaging characteristic of a patient with refractory lymphomatoid granulomatosis (LYG), and to report the successful treatment with bendamostine combined rituximab. Methods The medical history, physical examination and imaging of the patients were analyzed. The lung nodule biopsy was stained by H＆E staining, tumor and inflammation related immunohistochemistry, Epstein Barr virus in situ hybridization. The treatment process was reported. Results The patient was 67 years old female patient. She came with the complain of “weakness of both legs for 3 months and limitation of eye movement for 3 days”. Physical examination: decreased time and space orientation, horizontal nystagmus of both eyes with limited abduction, inaccurate fingers and nose test, muscle strength grade 3-4 on both lower limbs, hyperreflexia and positive Babinski sign on both sides. EDSS score 8 points. Brain and spinal cord MR show granular abnormal signals with enhancement on bilateral hemisphere, brainstem and cervical spinal cord. PET-CT showed multiple pulmonary nodules with increased radiation uptake. Biopsy of pulmonary nodules showed pathological findings of lymphomatoid granulomatosis. The symptoms improved with the treatment of high dose methylprednisolone (500mg/day for 5 days) relapsed with the dose reduction of prednisone to 40 mg/day. Cyclophosphamide was used for 3 months but failed. Then treatment was switched to Bendamoxetine 100mg/ month + rituximab 500mg / month and lasted 6 months. The clinical symptoms and imaging were significantly relieved. Conclusion Lymphomatoid granulomatosis involving multiple systems such as brain, spinal cord and pulmonary was reported. Bendamoxetine combined with rituximab was effective on the treatment of refractory lymphomatous granuloma.
【Key words】lymphomatoid granulomatosis; brain and spinal cord; pulmonary nodules; bendamostine; rituximab; treatment.
Funding: National Natural Science Foundation of China (81971181); The Science and Technology Commission Foundation of Shanghai Changning District (CNKW2017Y02, CNKW2018Y01) and Foundation of Shanghai Tongren Hospital (TRYJ201705, TRYJ(JC)201803)