Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome.

Research paper by Daniel C DC Moreira, Christopher J CJ Ng, Ralph R Quinones, Xiayuan X Liang, Dominic W DW Chung, Jorge J Di Paola

Indexed on: 14 Sep '16Published on: 14 Sep '16Published in: Journal of Thrombosis and Haemostasis


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 patent developed as a result of ADAMTS-13 loss in the third-space fluid, a novel mechanism for acquired microangiopathic hemolytic anemia. This article is protected by copyright. All rights reserved.