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Antiphospholipid syndrome and recurrent thrombosis in children

Y Berkun1, S Padeh, J Barash

  • 1Safra Children's Hospital, Sheba Medical Center, Tel-Hashomer, Israel.

Arthritis and Rheumatism
|December 2, 2006
PubMed

Insights

Pediatric antiphospholipid syndrome (APS) presents uniquely, with venous thrombosis and stroke common. Anticoagulant therapy after initial events may prevent recurrence in children with APS.

Area of Science:

  • Pediatric Rheumatology
  • Hematology
  • Autoimmune Diseases

Background:

  • Antiphospholipid syndrome (APS) is rare in children, with limited data on its clinical features and outcomes.
  • Understanding pediatric APS is crucial for early diagnosis and management.

Purpose of the Study:

  • To analyze clinical and laboratory manifestations in a pediatric APS cohort.
  • To assess the influence of inherited thrombophilia on outcomes in pediatric APS.

Main Methods:

  • Multicenter retrospective study of pediatric patients with APS without prior autoimmune disease.
  • Review of clinical data, laboratory findings, hereditary thrombophilia, and patient outcomes.

Main Results:

  • The cohort included 28 children (mean age 10.6 years); common initial symptoms were venous thrombosis, stroke, and thrombocytopenia.
  • Long-term follow-up revealed significant central nervous system (57.1%) and hematologic involvement (9%). Systemic lupus erythematosus (SLE) developed in 5 female patients.
  • Hereditary thrombophilia did not predict recurrence, but anticoagulant treatment after the first thrombotic event was associated with fewer recurrences.

Conclusions:

  • Pediatric APS exhibits distinct characteristics compared to adult forms.
  • Systemic lupus erythematosus (SLE) can develop in a notable proportion of girls with pediatric APS.
  • Preventive anticoagulant therapy following the initial thrombotic event appears crucial in managing pediatric APS and preventing recurrences.
Abstract

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