How is pediatric antiphospholipid antibody syndrome diagnosed?

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
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Answer

Classification criteria for pediatric antiphospholipid antibody syndrome includes the following clinical criteria for vascular thrombosis: One or more clinical episodes of arterial, venous, or small-vessel thrombosis, in any tissue or organ. Thrombosis must be confirmed by objective validated criteria (ie, unequivocal findings of appropriate imaging studies or histopathology). For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation in the vessel wall.

The laboratory criteria include the following:

  • Anticardiolipin antibody of immunoglobulin G (IgG) and/or immunoglobulin M (IgM) isotype in serum or plasma - Must be present in medium or high titre (ie, >40 GPL or MPL, or >99th percentile) on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA

  • Anti-β2 glycoprotein-I antibody of IgG and/or IgM isotype in serum or plasma - Must be present in titre >99th percentile, on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA

  • Lupus anticoagulant in plasma - Must be present on two or more occasions at least 12 weeks apart, detected according to the guidelines of the International Society on Thrombosis and Hemostasis

Pediatric antiphospholipid syndrome is considered to be present if the clinical criterion and at least I of the laboratory criteria are met.


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