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

D C Moreira1, C J Ng1, R Quinones1

  • 1Department of Pediatrics, University of Colorado Anschutz Medical Campus (AMC), Aurora, CO, USA.

Journal of Thrombosis and Haemostasis : JTH
|September 14, 2016
PubMed

Insights

Systemic capillary leak syndrome (SCLS) can lead to microangiopathic hemolytic anemia (MAHA). This rare anemia may result from ADAMTS-13 enzyme loss into extravascular fluid spaces during SCLS episodes.

Area of Science:

  • Hematology
  • Vascular Biology
  • Rare Diseases

Background:

  • Idiopathic systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent episodes of severe vascular leakage, leading to hypotension, hypoalbuminemia, hemoconcentration, and edema.
  • While anemia and thrombocytopenia are not typical features of SCLS, this case highlights a potential complication.

Observation:

  • A pediatric patient presenting with SCLS developed microangiopathic hemolytic anemia (MAHA) during a phase of significant fluid extravasation and anasarca.
  • Serial measurements of ADAMTS-13 activity were performed during the clinical course.

Findings:

  • The study proposes a novel mechanism for acquired MAHA in SCLS.
  • ADAMTS-13 loss into the extravascular 'third-space' fluid is hypothesized to cause MAHA in this patient.
  • This suggests that significant protein extravasation in SCLS can lead to conditions conducive to MAHA.

Implications:

  • This finding expands the spectrum of potential complications associated with SCLS.
  • It suggests a new pathway for the development of acquired MAHA, linked to severe protein leakage.
  • Further research may elucidate the precise mechanisms and clinical significance of ADAMTS-13 deficiency in SCLS-associated MAHA.
Keywords:
ADAMTS-13 protein, humanhemolysismicroangiopathic hemolytic anemiasystemic capillary leak syndromethrombocytopenia

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