The clinical features of hematologic diseases in childhood vary significantly with the underlying disorder and are best subdivided into disorders of white blood cells, disorders of red blood cells, disorders of platelets, and disorders of the hemostatic system. As disorders of platelets and the hemostatic syndrome are likely to have the greatest impact when considering surgical procedures in children with hematologic disorders, they will be discussed first.
Disorders of Platelets and the Hemostatic System
The properly functioning hemostatic system requires an orchestrated interaction between coagulation factors, Von Willebrand Factor, platelets, endothelial cells of blood vessels, and the extracellular matrix (Fig.
1). Dysfunctions of any step of this process can lead to recurrent abnormal bleeding, including abnormal surgical bleeding, or thromboembolic events.
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1.Department of Pediatrics, Division of Pediatric Hematology and OncologyCardinal Glennon Children’s Medical Center, Saint Louis UniversitySt. LouisUSA