Hemophilia and von Willebrand Disease: A Review of Emergency Department Management

J Emerg Med. 2020 May;58(5):756-766. doi: 10.1016/j.jemermed.2020.02.019. Epub 2020 Apr 2.

Abstract

Background: Hemophilia and von Willebrand disease (VWD) are the most common congenital coagulation factor deficiencies. Patients with these disorders who experience bleeding complications are often initially managed in the emergency department (ED).

Objective of the review: This review will focus on the emergency department management of patients with these disorders and provide an update on current treatment options.

Discussion: The mainstay of management is initial stabilization, control of bleeding when possible, and administration of specific factors. Early coordination of care with hematology is critical.

Conclusions: Emergency medicine providers must have an understanding of the pathophysiology, clinical presentation, and management strategies in order to optimally care for these complex patients.

Keywords: emergency department management; hemophilia; von Willebrand disease.

Publication types

  • Review

MeSH terms

  • Emergency Service, Hospital*
  • Factor VIII / therapeutic use
  • Hemophilia A* / complications
  • Hemophilia A* / therapy
  • Hemorrhage / etiology
  • Hemorrhage / therapy
  • Humans
  • von Willebrand Diseases* / complications
  • von Willebrand Diseases* / therapy

Substances

  • Factor VIII