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



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.
Department of Pediatrics, University of Colorado Anschutz Medical Campus (AMC), Aurora, CO, USA.
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DC
Moreira
Department of Pediatrics, University of Colorado Anschutz Medical Campus (AMC), Aurora, CO, USA.
C J
CJ
Ng
Department of Pediatrics, University of Colorado Anschutz Medical Campus (AMC), Aurora, CO, USA.
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R
Quinones
Department of Pathology, University of Colorado, AMC, Aurora, CO, USA.
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X
Liang
Bloodworks Research Institute -Puget Sound, Seattle, WA, USA.
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DW
Chung
Department of Pediatrics, University of Colorado Anschutz Medical Campus (AMC), Aurora, CO, USA.|Human Medical Genetics and Genomics Program, University of Colorado, Aurora, CO, USA.
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Di Paola
D000071120:ADAMTS13 Protein / Q000097:blood* / Q000276:immunology*; D000743:Anemia, Hemolytic / Q000097:blood*; D000906:Antibodies / Q000097:blood; D019559:Capillary Leak Syndrome / Q000097:blood*; D002675:Child, Preschool; D004487:Edema; D017707:Erythrocyte Transfusion; D005260:Female; D006801:Humans; D017713:Platelet Transfusion; D012008:Recurrence; D013997:Time Factors; D016896:Treatment Outcome
D000906:Antibodies; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
ADAMTS-13 protein, human; hemolysis; microangiopathic hemolytic anemia; systemic capillary leak syndrome; thrombocytopenia