Trauma in Hemophilia B

20 Sep

RAGING HYPOTHETICAL:


Your next patient is a 3 yo male with Hemophilia B whose leg was hit by a car at low speed while he was playing in the street. He had soft tissue swelling/abrasion and a negative plain film of the leg.  What do you do now?
 
 

HEMOPHILIA B (QUICK HITS):


Hemophilia B is deficiency of Factor IX. Also known as Christmas Disease. It is x-linked recessive.
 
Severity is based on factor IX activity level at baseline: <1% is severe, 1-5% is moderate, and >5% is mild
 
Trauma deserves special attention because of hemorrhage out of proportion to injury, delayed bleeding, and potential for joint destruction from hemarthrosis
 
Factor replacement should be given asap
 
Factor IX can be given as either recombinant or purified
 
Give enough to increase factor level by 40-50% in cases of single joint hemarthrosis, and >50% in severe hemorrhage or any significant head injury
 
Dose in IU = wgt (kg) x desired percent increase x (1.3unit/kg for benefix or 1unit/kg for alphanine or mononine)
 
A continuous infusion can also be given after the initial bolus, to help maintain factor levels, prevent rebleeding, and allow for less frequent monitoring of levels
 
Consult with hem/onc for specific dosing and monitoring recs
 
Submitted by M. Smith.
References: Hoots WK, Shapiro AD. Treatment of hemophilia. www.uptodate.com. 2012.; picture
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