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
杨艳 1刘玉秋 1王彬 1徐昌盛 2王凤梅 1张晓良 1刘必成1
作者信息
- 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
- 折叠
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.
Abstract
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Key words
Lymphohistiocytosis,hemophagocytic/Thrombotic microangiopathies/Acute kidney injury引用本文复制引用
基金项目
国家自然科学基金(82204839)
国家自然科学基金(82060132)
出版年
2024