A prospective cohort study followed 40 evaluable children (<18 years) with persistent antiphospholipid antibodies (aPL) and hematologic involvement (thrombocytopenia, autoimmune hemolytic anemia [AIHA], or Evans syndrome) at a single tertiary center from 1995–2024 (up to 29 years), assessing progression to classifiable APS and/or SLE.
27.5% (11/40) progressed to APS and/or SLE. Evans syndrome at presentation carried the highest hazard of progression vs. isolated thrombocytopenia (HR 6.21, 95% CI 1.46–26.41); isolated thrombocytopenia followed a largely indolent course; AIHA was intermediate but did not independently predict progression.
Single tertiary center limits generalizability; small sample (n=40) reduces statistical power, particularly for aPL profile analyses; lupus anticoagulant was nearly universal, limiting its discriminatory value as a predictor.
In aPL-positive children, Evans syndrome — at presentation or emerging during follow-up — should prompt heightened surveillance for APS and SLE. Isolated thrombocytopenia with persistent aPLs can be monitored with a lower level of concern for near-term autoimmune progression.
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