Clinical significance of lupus anticoagulants in children

Objectives: To determine the spectrum of associated clinical manifestations and time course of lupus anticoagulants (LA) in children. Study design: Retrospective study of 95 consecutive children (46 boys and 4 7 girls), with a median age of 5.3 years (range, 1.7 to 17.1 years), diagno sed with presence of LA at a hemostasis referral center; 83 were followed u p over a median of 2.9 years (range, 6 weeks to 21.6 years). Results: At diagnosis, 80 of 95 (84%) children were free of symptoms, and p resence of LA was found incidentally. Nine children (10%) had bleeding symp toms, 5 (5%) had thrombotic events, and 1 had systemic lupus erythematosus. Among the patients with bleeding, 5 had transient severe hypoprothrombinem ia after adenovirus infections, and 3 had thrombocytopenia. None of the chi ldren who were initially free of symptoms had bleeding, thrombotic complica tions, or autoimmune disease subsequently. At followup, 48 of 83 (58%) pati ents had normal activated partial thromboplastin time values after 1.9 year s (5 weeks to 19.1 years). Thirty-two (38%) still had activated partial thr omboplastin time elevations but did not fulfill all criteria for presence o f LA after 3.2 years (7.4 months to 9.3 years). Three (4%) patients, who ha d presented with thrombosis, had persistent positive LA, anti-cardiolipin, and antinuclear antibodies after 1.4, 2.8, and 7.5 years, respectively. One of these had recurrent thrombosis. Conclusions: In most children the presence of LA did not lead to clinical c omplications and was transient. Bleeding occurred with additional hypoproth rombinemia or thrombocytopenia. Thrombosis was rare and strongly associated with persistently positive LA.