Citation

Bodrozic J, Lekovic D, Koncar I, Sulović Dzelatović N, Miljic P. Blood Res. 2022; ePub(ePub): ePub.

Copyright

(Copyright © 2022, Korean Society of Hematology)

DOI

10.5045/br.2022.2022112

PMID

36348636

Abstract

TO THE EDITOR: Hemophilia is the most common inherited bleeding disorder caused by factor VIII (hemophilia A) or factor IX (FIX) (hemophilia B) deficiency. The incidence of hemophilia A is 1 in 5,000, and that of hemophilia B is 1 in 30,000 live male births. Increased bleeding tendency is the main clinical manifestation of the disease; however, the bleeding pattern may be highly variable depending on the residual activity of the missing factor. In patients with a severe form of the disease (residual activity <1 IU/dL), spontaneous joint and muscle bleeding are common. In patients with residual factor activity >1 IU/dL, prolonged bleeding after trauma or surgery may predominate in clinical presentation [1].
Firearm injuries in patients with hemophilia, even if treated immediately, undoubtedly have uncertain outcomes. Only two cases of firearm injury in patients with hemophilia have been reported, with one being fatal [2, 3]. Therefore, the clinical course and treatment of these injuries in patients with hemophilia are unknown.

Herein we present a patient with moderate hemophilia B who sustained a gunshot wound to the right lower leg that was complicated by acute limb compartment syndrome (ACS) development.

The patient, born in 1983, was diagnosed with moderate hemophilia B at the age of 10 years after prolonged bleeding following tooth extraction. In the initial finding, the FIX level was 4 IU/dL. The patient never experienced significant spontaneous hemorrhage and received FIX replacement therapy on one occasion in 2015 for pilonidal sinus surgery.

The patient was brought to the emergency department of our institution on January 6, 2019, with a penetrating gunshot wound in the right lower leg, which was sustained after a bullet from a 7.65 mm caliber pistol backfired.

Immediately after the injury, the bleeding was intense …

Language: en