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Published on: November 26, 2013
Treatment, survival, and thromboembolic outcomes of thrombotic storm in children
Marilyn J Manco-Johnson1, Michael Wang, Neil A Goldenberg
1Department of Pediatrics, The Children's Hospital, University of Colorado School of Medicine, Aurora, CO 80045, USA. marilyn.manco-johnson@ucdenver.edu
Insights
Thrombotic storm, a rare but serious condition in children, involves widespread blood clots despite anticoagulation. Treatment with direct thrombin inhibitors and immune modulation can control clots, but long-term complications remain a concern.
Area of Science:
- Pediatric Hematology
- Thrombosis Research
- Vascular Medicine
Background:
- Thrombotic storm is a severe form of pediatric venous thromboembolism (VTE).
- Characterized by multisite VTE with acute thrombus progression despite anticoagulation.
- Understanding its course and management is critical for improving outcomes.
Observation:
- Eight children (6 months to 21 years) were diagnosed with thrombotic storm.
- Antiphospholipid antibodies were present in 6 children; 3 had heparin-induced thrombocytopenia.
- One child died; 7 survivors experienced VTE recurrence or postthrombotic syndrome.
Findings:
- Direct thrombin inhibitors and immunomodulatory therapy were used to manage thrombotic storm.
- Treatment aimed to normalize elevated D-dimer levels and prevent clot progression.
- Despite initial management, 3 of 7 survivors had recurrent VTE, and 4 developed postthrombotic syndrome.
Implications:
- Thrombotic storm is an infrequent yet potentially fatal pediatric VTE presentation.
- Effective management involves direct thrombin inhibitors and immune modulation.
- Long-term adverse outcomes necessitate ongoing monitoring and research in pediatric thrombosis.
Objective:
To describe the course and management of thrombotic storm in 8 children.
Study Design:
Clinical data were collected and analyzed for consecutive children diagnosed with thrombotic storm, aged 6 months to 21 years inclusive, in the context of a single-institution prospective inception cohort study. Thrombotic storm was defined as newly diagnosed multisite venous thromboembolism (VTE) with acute thrombus progression despite conventional or higher than conventional dosing of heparin or low molecular weight heparin. All evaluations and therapies were ordered by the treating physicians in the context of clinical decision making.
Results:
Eight of the 178 children with VTE enrolled in the cohort between March 2006 and November 2009 were diagnosed with thrombotic storm. Antiphospholipid antibodies were acutely positive in 6 children, of whom heparin-induced thrombocytopenia was confirmed by serotonin release assay in 2 and atypical in 1. One child died. Five children received a direct thrombin inhibitor, titrated to achieve normalization of markedly elevated D-dimer levels. All children were transitioned to fondaparinux or enoxaparin before receiving extended anticoagulation with warfarin. Immunomodulatory therapy was instituted in all children. During follow-up (median duration, 3 years; range, 2-6 years), 3 of the 7 surviving children experienced recurrent VTE, and 4 children had clinically significant postthrombotic syndrome.
Conclusion:
Thrombotic storm is an infrequent but potentially fatal presentation of VTE in children. Administration of direct thrombin inhibitors and immune modulation can achieve quiescence, although long-term adverse outcomes are common.
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