High risk and low prevalence diseases: Hemophilia emergencies

Leen Alblaihed, Sarah B. Dubbs, Alex Koyfman, Brit Long

Research output: Contribution to journalReview articlepeer-review


Introduction: Hemophilia is a bleeding disorder due to coagulation pathway factor deficiency that is associated with significant morbidity and mortality. Objective: This review highlights the pearls and pitfalls of the emergency department (ED) evaluation of hemophilia, including diagnostic procedures, imaging, and management based on current evidence. Discussion: Hemophilia is marked by deficiency in factor VIII (in hemophilia A) or IX (in hemophilia B), which may result in severe bleeding. The severity of the disease depends upon factor levels. Patients with severe deficiency most commonly present in the first two years of life. Severe bleeding may include intracranial hemorrhage, retroperitoneal bleeding, large hematomas, bleeding within the chest or abdomen/pelvis, and subacute or delayed postpartum bleeding. ED management is the immediate replacement of clotting factors based on the suspicion of bleed rather than the confirmation of one. The doses for factor concentrate replacement to achieve factor levels of 100% are 50 U/kg for FVIII and 100 U/kg for FIX. The development of inhibitors can complicate the clinical picture and treatment possibilities. Consultation with the hematology specialist is recommended to assist with evaluation and management. Conclusion: Emergency physician knowledge of hemophilia, including the presentation, evaluation, and management, can improve the care of these patients.

Original languageEnglish (US)
Pages (from-to)21-27
Number of pages7
JournalAmerican Journal of Emergency Medicine
StatePublished - Jun 2022


  • Bleeding
  • Factor replacement
  • Hematology
  • Hemophilia

ASJC Scopus subject areas

  • Emergency Medicine


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