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.