Cho Sung Bin, Park Jun Su, Kim Hyun Ok, Chung Kee Yang
Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemun- gu, Seoul 120-752, Korea.
Yonsei Med J. 2006 Oct 31;47(5):737-40. doi: 10.3349/ymj.2006.47.5.737.
A 54-year-old Korean male with scleroderma-like manifestation of primary systemic amyloidosis presented with firm cutaneous induration of face and distal extremities, subcutaneous induration of the trunk and proximal extremities, limited range of motion in all joints, hoarseness, and dysphagia. Monthly high-dose intravenous immunoglobulin (hdIVIg) was given (three treatments, each time administering 0.4 g/kg per day for five days), and both signs and symptoms began to improve. However, the quantitative analyses of serum protein did not improve. Therapeutic plasma exchange (TPE) was performed monthly to clear the elevated serum immunoglobulin, and after several treatments, their levels normalized and symptoms were maintained in the improved state for more than two years. To summarize, hdIVIg and TPE combination therapy may be used as a safe first-line treatment for patients with primary systemic amyloidosis presenting with symptomatic monoclonal gammopathy.
一名54岁的韩国男性，患有原发性系统性淀粉样变性的硬皮病样表现，出现面部和远端肢体皮肤硬结、躯干和近端肢体皮下硬结、所有关节活动范围受限、声音嘶哑和吞咽困难。每月给予大剂量静脉注射免疫球蛋白（hdIVIg）（共三次治疗，每次每天给予0.4 g/kg，持续五天），症状和体征开始改善。然而，血清蛋白的定量分析并未改善。每月进行治疗性血浆置换（TPE）以清除升高的血清免疫球蛋白，经过几次治疗后，其水平恢复正常，症状维持改善状态超过两年。总之，hdIVIg和TPE联合治疗可作为原发性系统性淀粉样变性伴症状性单克隆丙种球蛋白病患者的安全一线治疗方法。
