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Catastrophic antiphospholipid syndrome in childhood: presentation with an inferior caval vein mass

Abdul Alim Abdul Haium1, Mary Sheppard, Michael Rubens

  • 1Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.

BMJ Case Reports
|July 18, 2013
PubMed

Insights

Pulmonary hypertension in children can stem from venous thromboembolism, a condition linked to antiphospholipid antibody syndrome (APS). This case highlights primary catastrophic APS presenting as a tumor with pulmonary hypertension.

Area of Science:

  • Pediatric Cardiology
  • Hematology
  • Rheumatology

Background:

  • Venous thromboembolism (VTE) is a significant cause of pediatric pulmonary hypertension.
  • Antiphospholipid antibody syndrome (APS) is a known, albeit less common, cause of VTE.
  • Catastrophic APS (CAPS) is a severe, life-threatening variant of APS characterized by widespread thrombosis and organ damage.

Observation:

  • A pediatric patient presented with symptoms suggestive of pulmonary hypertension.
  • Imaging revealed a mass in the inferior vena cava, initially mimicking a tumor.
  • Further investigation indicated the mass was related to VTE secondary to primary CAPS.

Findings:

  • The patient was diagnosed with primary catastrophic antiphospholipid antibody syndrome (CAPS).
  • The presentation of pulmonary hypertension and an inferior vena cava mass was attributed to CAPS-induced thrombosis.
  • This case underscores the diverse clinical manifestations of CAPS in children.

Implications:

  • Early recognition of CAPS is crucial for timely intervention in pediatric patients with unexplained pulmonary hypertension and vascular masses.
  • This case expands the differential diagnosis for pediatric pulmonary hypertension and inferior vena cava masses.
  • Understanding the link between CAPS and VTE can improve diagnostic and therapeutic strategies in pediatric hematology and rheumatology.

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