Schnitzler综合征进展为惰性小B细胞淋巴瘤1例  

Schnitzler Syndrome with Subsequent Development of Indolent Small Bcell Lymphoma:A Case Report

作　　者：胡影 田美伊 丁秋玲[2] 刘坚 HU Ying;TIAN Mei-yi;DING Qiuling;LIU Jian(Department of Hematology,Aerospace Center Hospital,Beiing 100049,China;Department of Rheumatology and immunology,Aerospace Center Hospital,Beiing 100049,China)

机构地区：[1]航天中心医院血液科,北京100049 [2]航天中心医院免疫科,北京100049

出　　处：《中国合理用药探索》2023年第4期7-10,共4页Chinese Journal of Rational Drug Use

摘　　要：患者男性,46岁,2015年5月开始反复出现发热和皮肤荨麻疹,先后被诊断为结节性红斑、结缔组织病和炎症反应综合征。实验室检查示炎症标志物显著增加,皮肤活检示嗜中性粒细胞性荨麻疹。甲泼尼龙片、硫酸羟氯喹片、白芍总苷胶囊、依巴斯汀片治疗无效。之后检查发现存在单克隆免疫球蛋白IgM,骨髓穿刺及正电子发射计算机断层成像(PET)未见异常,诊断为嗜中性荨麻疹性皮病、意义未明的单克隆免疫球蛋白血症、Schnitzler综合征。给予硼替佐米治疗无效,之后给予托珠单抗治疗有效,患者体温恢复正常,皮疹消退。1年后患者出现腰骶部明显疼痛,核磁共振示腰椎多发病变及压缩骨折,骨活检提示小B细胞淋巴瘤,骨髓免疫表型显示为单克隆B细胞和单克隆浆细胞,诊断为惰性小B细胞淋巴瘤,给予利妥昔单抗联合苯达莫司汀化疗。A 46-year-old man complained of a history of recurrent fever and chronic urticaria since May 2015.He was diagnosed with erythema nodosum,connective tissue disease,and inflammatory response syndrome.Laboratory tests showed a significant increase in inflammatory markers,and skin biopsy showed neutrophilic urticaria.The patient was unresponsive to treatment with methylprednisolone,hydroxychloroquine sulfate and total glucosides of paeony capsules.The monoclonal immunoglobulin IgM was detected at a later time.Bone marrow examination and positron emission tomography(PET)showed no abnormalities.The patient was finally diagnosed with neutrophilic urticarial dermatosis,monoclonal immunoglobulinemia of undetermined significance,Schnitzler syndrome.Bortezomib was initially given which was ineffective,and then tocilizumab was given,after which the patient’s body temperature returned to normal with rash subsided.However,one year later,he developed pain in lumbosacral region.Magnetic resonance imaging suggested multiple lumbar spine lesions and compression fractures.Bone biopsy indicated small Bcell lymphoma.Bone marrow immunophenotyping revealed monoclonal B cells and monoclonal plasma cells,which confirmed the diagnosis of indolent small B cell lymphoma.Rituximab plus bendamustine chemotherapy regimen was administered.

关 键 词：Schnitzler综合征 托珠单抗 惰性小B细胞淋巴瘤 单克隆免疫球蛋白 荨麻疹 

分 类 号：R593.2[医药卫生—内科学]