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Thrombocytopenia and thromboembolism in pediatric systemic lupus erythematosus
Markus Schmugge1, Shoshana Revel-Vilk, Linda Hiraki
1Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
In children with systemic lupus erythematosus (SLE), thrombocytopenia affects 50%. Lupus anticoagulant, not sustained thrombocytopenia, is linked to a higher risk of thromboembolic events (TEs) in pediatric SLE patients.
Area of Science:
- Pediatric Rheumatology
- Hematology
- Immunology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect multiple organ systems.
- Thrombocytopenia, a condition characterized by low platelet counts, is a common hematologic manifestation in SLE.
- The relationship between thrombocytopenia, its severity, and the risk of thromboembolic events (TEs) in pediatric SLE requires further elucidation.
Purpose of the Study:
- To determine the incidence and outcome of thrombocytopenia in children diagnosed with SLE.
- To investigate the association between thrombocytopenia and other SLE manifestations, particularly TEs.
- To identify risk factors for TEs in pediatric patients with SLE.
Main Methods:
- Retrospective review of 106 pediatric patients diagnosed with SLE between 1992 and 2001.
- Analysis of patient data including incidence of thrombocytopenia, its severity and duration, and occurrence of TEs.
- Statistical assessment of the association between thrombocytopenia, lupus anticoagulant, anticardiolipin antibodies, and TEs.
Main Results:
- Thrombocytopenia was present in 50% of pediatric SLE patients, with 34% experiencing moderate or severe degrees.
- Sustained thrombocytopenia occurred in 29% of the cohort.
- Lupus anticoagulant was significantly associated with an increased risk of TEs, whereas anticardiolipin antibodies and sustained thrombocytopenia were not.
Conclusions:
- Thrombocytopenia is a frequent finding in pediatric SLE, with a notable proportion experiencing sustained low platelet counts.
- The presence of lupus anticoagulant is a key predictor of thromboembolic events in this population.
- Further research is warranted to understand the complex interplay between hematologic abnormalities and thrombotic risk in pediatric SLE.
Abstract:
To define in children with systemic lupus erythematosus (SLE) the incidence, outcome, and association of thrombocytopenia with other SLE manifestations, specifically thromboembolic events (TEs), we retrospectively reviewed 106 pediatric patients with SLE diagnosed between 1992 and 2001. Thrombocytopenia was found in 50%, which was of a moderate or severe degree in 34%. The thrombocytopenia was sustained in 29% of all patients. Twelve patients were diagnosed with autoimmune thrombocytopenic purpura 2 to 69 months before the diagnosis of SLE. Of them, 10 children required treatment, and three patients underwent splenectomy. TEs occurred in 10.4% of the total cohort of 106. Lupus anticoagulant, but not anticardiolipin antibodies and sustained thrombocytopenia, were associated with a higher risk for TEs.
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