Hemophilia in the Emergency Department – Part 1

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June 20, 2017 by dailybolusoflr

By: Casey Carr


Pathophysiology:

Hemophilia is an X-linked recessive disorder, and is divided into hemophilia A and hemophilia B. Hemophilia A causes a deficiency in Factor VIII, and hemophilia causes a deficiency in Factor IX.

Both diseases are categorized by severity, which is represented by the level of clotting factor activity.

Severe – <1% normal clotting factor activity (35% of hemophilia A patients have severe disease, 60% of hemophilia B have severe disease)
Moderate – 1%-5% normal clotting factor activity (15% of hemophilia A have moderate disease)
Mild – 5-40% normal clotting factor activity (50% of hemophilia A have mild disease)
Patients with >40% of normal clotting factor activity are unlikely to be symptomatic


Typical acute bleeding presentations:

Hemarthrosis
Hematoma and soft tissue bleeding
Musosal bleeding
Intracranial hemorrhage
Post-surgical/procedural bleeding
Deep lacerations
Hematuria
Gastrointestinal bleeding


Treatment:

Many patients with known hemophilia are trained to self administer treatment when a bleeding episode begins.

Goal is to rapidly correct deficit clotting factor.

Factor level assays will NOT return while patient is in acute setting in the emergency department – in limb or life threatening presentations, assume the factor level is 0%; therefore factor dosing should be done empirically based on suspected or known injury


References:

National Hemophilia Foundation Guidelines for emergency department management of individuals with hemophilia. National Hemophilia Foundation, New York (2006) [MASAC Document #175

Singleton T, Kruse-Jarres R, Leissinger C. Emergency department care for patients with hemophilia and von Willebrand disease. The Journal of Emergency Eedicine. 39(2):158-65. 2010

Franchini M, Mannucci PM. Hemophilia A in the third millennium. Blood reviews. 27(4):179-84. 2013.

Mathew P, Dinter H, Church N, Humphries TJ, Kulkarni R. Inhibitors in haemophilia A: a perspective on clotting factor products as a potential contributing factor. Haemophilia : the official journal of the World Federation of Hemophilia. 22(3):334-41. 2016

Schwartz KR, Rubinstein M. Hemophilia And Von Willebrand Disease In Children: Emergency Department Evaluation And Management. Pediatric emergency medicine practice. 12(9):1-20; quiz 20-1. 2015

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