Elsevier

The Journal of Pediatrics

Volume 134, Issue 2, February 1999, Pages 199-205
The Journal of Pediatrics

Clinical significance of lupus anticoagulants in children,☆☆

https://doi.org/10.1016/S0022-3476(99)70416-6Get rights and content

Abstract

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 47 girls), with a median age of 5.3 years (range, 1.7 to 17.1 years), diagnosed with presence of LA at a hemostasis referral center; 83 were followed up 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 presence of LA was found incidentally. Nine children (10%) had bleeding symptoms, 5 (5%) had thrombotic events, and 1 had systemic lupus erythematosus. Among the patients with bleeding, 5 had transient severe hypoprothrombinemia after adenovirus infections, and 3 had thrombocytopenia. None of the children who were initially free of symptoms had bleeding, thrombotic complications, or autoimmune disease subsequently. At follow-up, 48 of 83 (58%) patients had normal activated partial thromboplastin time values after 1.9 years (5 weeks to 19.1 years). Thirty-two (38%) still had activated partial thromboplastin time elevations but did not fulfill all criteria for presence of LA after 3.2 years (7.4 months to 9.3 years). Three (4%) patients, who had 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 complications and was transient. Bleeding occurred with additional hypoprothrombinemia or thrombocytopenia. Thrombosis was rare and strongly associated with persistently positive LA. (J Pediatr 1999;134:199-205)

Section snippets

Patients

Ninety-five consecutive children, 47 girls and 48 boys, in whom LA had been identified at our hemostasis referral center between 1969 and 1996, were studied. Median age at diagnosis of presence of LA was 5.3 years (range, 1.7 to 17.1 years). Most patients had been followed up at variable intervals after diagnosis. Eighty-three patients could be studied longitudinally, having had at least one follow-up examination (mean, 2; range, 1 to 5). Only patients who were followed up for more than 6

Clinical Symptoms at Diagnosis of Presence of LA

Eighty of 95 (84%) children were free of symptoms, and presence of LA was diagnosed incidentally. In most cases (n = 69) coagulation tests had been done for preoperative screening, mainly before planned adenotonsillectomy (n = 54). Only 15 patients (16%) had specific symptoms leading to the diagnosis of presence of LA: 9 (10%) patients were referred because of bleeding symptoms (Table I). Five of these had bleeding symptoms of acute onset associated with severe hypoprothrombinemia, 3 had mild

DISCUSSION

Our study provides evidence that the presence of LA does not lead to clinically relevant complications and is transient in most children. LA were found incidentally in 84% of our children; 68% were identified at screening before adenotonsillectomy, representing a selected population with recurrent sinus, tonsillar, and upper respiratory tract infections. A relationship between bacterial or viral infections and LA in these children has previously been suggested.21, 24, 27, 28 Our series had an

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    Reprint requests: Christoph Male, MD, Department of Paediatrics, University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria.

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