Taktak Aysel, Köksoy Adem, Başaran Özge, Kiremitçi Saba, Acar Banu, Uncu Nermin, Çakar Nilgün
Department of Pediatric Nephrology and Rheumatology, Ankara Child Health, Hematology, Oncology Training and Research Hospital, Ankara, Turkey.
Department of Pathology, Ankara University Faculty of Medicine, Ankara, Turkey.
Turk J Pediatr. 2016;58(2):223-226. doi: 10.24953/turkjped.2016.02.018.
Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures. In addition, his symptoms fulfilled the 2012 revised criteria for the classification of SLE. He was treated with plasma infusions and methylprednisolone/prednisone. At follow up his laboratory findings and general condition improved and no relapse was seen.
血栓性微血管病(TM),尤其是血栓性血小板减少性紫癜(TTP),在系统性红斑狼疮(SLE)中被描述为一种严重的血液系统受累情况。然而,溶血性尿毒症综合征(HUS)在SLE中较少见,尤其是作为首发表现。在此,我们报告一名15岁男孩,表现为肉眼血尿、尿量减少和瘀点性皮损。根据TM的经典三联征,同时观察到补体降低和粪便培养阴性,他被诊断为非典型HUS。此外,他的症状符合2012年修订的SLE分类标准。他接受了血浆输注和甲泼尼龙/泼尼松治疗。随访时,他的实验室检查结果和一般状况有所改善,未见复发。