Chak W K, Lam D S Y, Lo W H, Hui C M, Wong S N
Department of Paediatrics and Adolescent Medicine, Tuen Mun Hospital, Tsing Chung Koon Road, Tuen Mun, Hong Kong, ROC.
Hong Kong Med J. 2003 Oct;9(5):363-8.
Thrombotic thrombocytopenic purpura is a rare but serious condition in childhood. It can be idiopathic or a complication of other diseases or drug therapy. We report on a 12-year-old Chinese girl who presented with fulminant systemic lupus erythematosus with progressive renal failure, pancytopenia, and cerebral dysfunction due to thrombotic thrombocytopenic purpura. The patient also had Pneumocystis carinii pneumonia, Pseudomonas septicaemia, and Herpes zoster infections as a result of immunosuppressive treatment. She responded to combined therapy with pulse methylprednisolone, cyclophosphamide, plasmapheresis, and intensive care support, and completely recovered renal and neurological function. A review of the English-language medical literature since 1968 identified 20 other paediatric cases of systemic lupus erythematosus and thrombotic thrombocytopenic purpura. Clinical features, treatment, and outcome of these cases are presented and discussed. Early recognition is important, and although plasmapheresis is not of proven benefit in severe cases of systemic lupus erythematosus, it is life-saving in lupus-related thrombotic thrombocytopenic purpura and must be instituted early to avoid a poor outcome.
血栓性血小板减少性紫癜是一种儿童期罕见但严重的疾病。它可以是特发性的，也可以是其他疾病或药物治疗的并发症。我们报告了一名12岁的中国女孩，她因血栓性血小板减少性紫癜出现暴发性系统性红斑狼疮，伴有进行性肾衰竭、全血细胞减少和脑功能障碍。由于免疫抑制治疗，该患者还患有卡氏肺孢子虫肺炎、铜绿假单胞菌败血症和带状疱疹感染。她对脉冲甲基强的松龙、环磷酰胺、血浆置换和重症监护支持的联合治疗有反应，肾脏和神经功能完全恢复。对1968年以来的英文医学文献进行回顾，发现了另外20例系统性红斑狼疮和血栓性血小板减少性紫癜的儿科病例。本文介绍并讨论了这些病例的临床特征、治疗方法和结果。早期识别很重要，虽然血浆置换在严重系统性红斑狼疮病例中尚未证实有益，但在狼疮相关的血栓性血小板减少性紫癜中是救命的，必须尽早进行以避免不良后果。
