噬血细胞综合征合并血栓性微血管病1例  

Hemophagocytic syndrome combined with thrombotic microangiopathy: a case report

作　　者：杨艳 刘玉秋 王彬 徐昌盛[2] 王凤梅 张晓良[1] 刘必成[1] Yang Yan;Liu Yuqiu;Wang Bin;Xu Changsheng;Wang Fengmei;Zhang Xiaoliang;Liu Bicheng(Department of Nephrology,Zhongda Hospital,Southeast University,Nanjing 210009,China;Department of Emergency Medicine,Zhongda Hospital,Southeast University,Nanjing 210009,China)

机构地区：[1]东南大学附属中大医院肾脏科,东南大学医学院,南京210009 [2]东南大学附属中大医院急诊科,东南大学医学院,南京210009

出　　处：《中华肾脏病杂志》2024年第7期558-561,共4页Chinese Journal of Nephrology

基　　金：国家自然科学基金(82204839, 82060132)。

摘　　要：该文报道1例以不明原因发热为首发表现、较为罕见的噬血细胞综合征合并血栓性微血管病的诊治病例。患者女性, 37岁, 以发热、溶血性贫血、血小板下降及肾功能进行性下降为主要表现。经双重血浆置换后输注新鲜冰冻血浆和大剂量糖皮质激素治疗后, 患者病情明显好转, 未再有发热、溶血, 血小板及肾功能恢复正常。急性期后肾活检病理诊断为急性肾小管损伤。随访期间患者病情无复发, 肾功能正常。The paper reports a rarely case of hemophagocytic syndrome complicated with thrombotic microangiopathy,first presented with fever of unknown origin.A 37-year-old female patient mainly presented with fever,hemolytic anemia,thrombocytopenia,and progressive decline in renal function.After infusion of fresh frozen plasma and high dose of glucocorticoid after double plasma exchange,the patient showed good prognosis,no further fever or hemolysis occurred,recovered platelet and renal function.After acute episode phase,kidney biopsy was performed and acute tubular necrosis was diagnosed.During the follow-up period,the disease did not recur,and the renal function was normal.

关 键 词：淋巴组织细胞增多症 噬血细胞性 血栓性微血管病 急性肾损伤 

分 类 号：R55[医药卫生—血液循环系统疾病]