Li Xiang-Yang, Mai Yan-Fen, Huang Jing, Pai Pearl
Department of Nephrology, The University of Hong Kong-Shenzhen Hospital, Shenzhen 518053, Guangdong Province, China.
Department of Hematology, The University of Hong Kong-Shenzhen Hospital, Shenzhen 518053, Guangdong Province, China.
World J Clin Cases. 2019 Jul 6;7(13):1660-1670. doi: 10.12998/wjcc.v7.i13.1660.
Thrombocytopenia associated with acute kidney injury is a challenging disorder. Thrombotic microangiopathy (TMA) is a potentially life- or organ-threatening syndrome that can be induced by several disorders or medical interventions. There is overlap between the clinical presentation and pathophysiology of thrombotic thrombocytopenia purpura and hemolytic uremic syndrome (HUS), and to a lesser extent, disseminated intravascular coagulation (DIC). We describe a case to illustrate the potential diagnostic difficulty, especially at initial presentation.
We reported a case of a 44-year-old woman that presented with diarrhea, thrombocytopenia, schistocytes, elevated serum lactate dehydrogenase (LDH) level and acute kidney injury. While the clinical presentation resembled that of Shiga toxin-induced HUS, the disease course was more consistent with gastrointestinal infection-related DIC. To aid in the accurate diagnosis of TMA and other associated disorders, we have undertaken a review and provided a clear interpretation of some typical biomarkers including schistocytes, LDH and platelet count, coagulation profile and more specific indexes of ADAMTS13, complement profile, and the isolation of Shiga toxin-producing (commonly referred to as STEC).
The use and correct interpretation of classical indexes of schistocyte, LDH, and platelet count is vital in diagnosing TMA and associated disorders. Understanding the characteristics of these biomarkers in the context of thrombocytopenia purpura, HUS and DIC will facilitate the accurate diagnosis and early initiation of appropriate treatment.
与急性肾损伤相关的血小板减少症是一种具有挑战性的疾病。血栓性微血管病(TMA)是一种可能危及生命或器官的综合征,可由多种疾病或医疗干预诱发。血栓性血小板减少性紫癜和溶血性尿毒症综合征(HUS)的临床表现和病理生理学存在重叠,在较小程度上,与弥散性血管内凝血(DIC)也存在重叠。我们描述了一个病例以说明潜在的诊断困难,尤其是在初次就诊时。
我们报告了一例44岁女性,表现为腹泻、血小板减少、裂体细胞、血清乳酸脱氢酶(LDH)水平升高及急性肾损伤。虽然临床表现类似于志贺毒素诱导的HUS,但病程更符合胃肠道感染相关的DIC。为了有助于准确诊断TMA及其他相关疾病,我们进行了综述,并对一些典型的生物标志物进行了清晰的解读,包括裂体细胞、LDH和血小板计数、凝血指标以及ADAMTS13更特异的指标、补体指标,以及产志贺毒素菌(通常称为STEC)的分离。
裂体细胞、LDH和血小板计数等经典指标的应用及正确解读对于诊断TMA及相关疾病至关重要。了解这些生物标志物在血小板减少性紫癜、HUS和DIC背景下的特征将有助于准确诊断并尽早开始适当治疗。