Microangiopathic hemolytic anemia due to ADAMTS‐13 loss in idiopathic systemic capillary leak syndrome

• Published in: Journal of thrombosis and haemostasis Vol. 14; no. 12; pp. 2353 - 2355
• Main Authors: Moreira, D. C., Ng, C. J., Quinones, R., Liang, X., Chung, D. W., Di Paola, J.
• Format: Journal Article
• Language: English
• Published: England Elsevier Limited 01.12.2016
• Subjects: ADAMTS13 Protein - blood; ADAMTS13 Protein - immunology; ADAMTS‐13 protein, human; Anemia; Anemia, Hemolytic - blood; Antibodies - blood; Capillary Leak Syndrome - blood; Child, Preschool; Circulatory system; Edema; Erythrocyte Transfusion; Female; hemolysis; Humans; microangiopathic hemolytic anemia; Platelet Transfusion; Recurrence; systemic capillary leak syndrome; thrombocytopenia; Time Factors; Treatment Outcome; ADAMTS-13 protein, human; microangiopathic hemolytic anemia; systemic capillary leak syndrome; thrombocytopenia; hemolysis
• ISSN: 1538-7933; 1538-7836; 1538-7836
• DOI: 10.1111/jth.13506

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.