特发性系统性毛细血管渗漏综合征中因ADAMTS - 13缺乏导致的微血管病性溶血性贫血。
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome.
作者信息
Moreira D C, Ng C J, Quinones R, Liang X, Chung D W, Di Paola J
机构信息
Department of Pediatrics, University of Colorado Anschutz Medical Campus (AMC), Aurora, CO, USA.
Department of Pathology, University of Colorado, AMC, Aurora, CO, USA.
出版信息
J Thromb Haemost. 2016 Dec;14(12):2353-2355. doi: 10.1111/jth.13506. Epub 2016 Oct 24.
UNLABELLED
Essentials Idiopathic systemic capillary leak syndrome (SCLS) is characterized by episodes of vascular leakage. We present the case of a patient with SCLS who developed microangiopathic hemolytic anemia (MAHA). We propose that this anemia is the result of ADAMTS-13 loss in the third-space fluid. This suggests that MAHA can occur in patients with significant extravasation of proteins.
SUMMARY
Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema. Anemia and thrombocytopenia are not part of this syndrome, but here we present the case of a pediatric patient with a clinical presentation consistent with SCLS who subsequently developed microangiopathic hemolytic anemia at a time when she had significant fluid loss and anasarca. Based on serial ADAMTS-13 levels, we propose that the anemia in this patient developed as a result of ADAMTS-13 loss in the third-space fluid, a novel mechanism for acquired microangiopathic hemolytic anemia.
未标注
要点 特发性系统性毛细血管渗漏综合征（SCLS）的特征为血管渗漏发作。我们报告了一例患有SCLS且发生微血管病性溶血性贫血（MAHA）的患者。我们认为这种贫血是由于第三间隙液中ADAMTS - 13缺失所致。这表明在蛋白质大量外渗的患者中可发生MAHA。
总结
特发性系统性毛细血管渗漏综合征（SCLS）是一种罕见病症，其特征为急性和复发性血管渗漏发作，并伴有严重低血压、低白蛋白血症、血液浓缩和水肿。贫血和血小板减少并非该综合征的组成部分，但在此我们报告了一例儿科患者，其临床表现与SCLS相符，随后在她有大量液体丢失和全身性水肿时发生了微血管病性溶血性贫血。基于连续的ADAMTS - 13水平，我们认为该患者的贫血是由于第三间隙液中ADAMTS - 13缺失所致，这是获得性微血管病性溶血性贫血的一种新机制。
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