Evaluation and Management of Coagulopathies and Thrombophilias in Pediatric Patients

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The diagnosis of coagulopathy or thrombophilia in pediatric patients can be challenging. Congenital coagulopathies often present in the pediatric period and require appropriate work-up for diagnosis and ongoing management. Acquired coagulopathies of childhood are frequently encountered in hospitalized children and warrant appropriate coagulation testing for goal-directed therapy. The incidence of thrombosis is increasing in pediatric patients. After identifying the presence of thrombus, acute management includes initiating therapeutic anticoagulation. Choice of anticoagulant depends on patient’s clinical status, along with availability of the anticoagulant. Thrombophilia evaluation is performed when children present with spontaneous thrombosis. Thrombophilia tests are inaccurate during acute illness.

Key points

• Coagulation screening for bleeding and thrombophilia disorders in infants and children provides critical clinical information but can be misleading because of an immature hemostatic system, preanalytical errors, interference, or the presence of acute phase response.
• Hemophilia and von Willebrand disease are congenital coagulopathies that require specialized testing for diagnosis and often require management with factor concentrate or recombinant factor replacement therapy.
• Multiple factor deficiencies can be seen in several acquired coagulopathies, such as disseminated intravascular coagulation, liver disease, vitamin K deficiency, and dilutional coagulopathy. These conditions may require treatment with blood components or prothrombin complex concentrates.
• Initial management of thrombosis is usually initiating therapeutic anticoagulation while laboratory or imaging evaluation is underway.
• Thrombophilia testing is performed in children or adolescents with spontaneous thrombus or unusual site of thrombus.

Introduction

Evaluation of coagulopathy and thrombophilia among pediatric patients poses significant and unique challenges compared with their adult counterparts. The immature hemostatic system in early childhood and lack of validated pediatric bleeding and thrombotic risk scores highlight part of these difficulties. An accurate personal and family hemostasis history can help guide the evaluation process; however, these limitations necessitate an algorithmic approach to the laboratory work-up. This article discusses the evaluation and management of coagulopathies and thrombophilias in pediatric patients.