Coagulopathies and Sickle Cell Disease

Document Type

Book Chapter

Publication Date

2026

Identifier

DOI: 10.1016/B978-0-443-12431-0.00004-8

Abstract

Hemostasis is the reaction of the body to endothelial damage of blood vessels to prevent bleeding. Primary hemostasis involves platelets, von Willebrand factor, collagen, and fibrinogen to form a platelet plug. Secondary hemostasis occurs as thrombin is generated to form a fibrin clot. The process is complex and multistep and must be tightly regulated to control bleeding. Pediatric patients should be appropriately screened for hemostatic disorders before surgery, and those with hemostatic disorders should be managed carefully in conjunction with a hematologist throughout the entire operative process. Sickle cell disease (SCD) is a multisystem disorder affecting more than 100,000 individuals in the United States. It results from variants in the β-globin chain of hemoglobin. Primary pathophysiology is due to anemia from polymerization that reduces red blood cell survival. Pediatric patients with SCD face unique and potentially life-threatening complications with surgical procedures and deep sedation and require appropriate preoperative, intraoperative, and postoperative management by a multidisciplinary team.

Journal Title

Holcomb and Ashcraft's Pediatric Surgery (Eighth Edition)

First Page

53

Last Page

65

Keywords

Acute chest syndrome; Avascular necrosis; Coagulation defects; Hemostasis; Hypoxia; Infiltration; Platelet disorder; Retinopathy; Sequestration; Sickle cell disease; Sickle cell pain; Splenic sequestration; Transfusion; von Willebrand disease

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