title: Comparing bleed frequency and factor concentrate use between haemophilia A and B patients.
abstract: Haemorrhagic manifestations in patients with haemophilia A and B are considered quite similar for comparable level of factor deficiency. We investigated the bleeding frequency and factor usage between HA and HB patients with comparable disease severities. We collected data on frequency of bleeds and factor concentrate utilization over 3 years, from January 2001 to December 2003. Information was gathered from home infusion logs recorded by patients or their parents, and treatment records from the Hemophilia Clinic or the Hospital Emergency Department. Data were available on 58 patients with severe HA (FVIII < 0.01 U mL(-1)), 10 with moderate HA (FVIII < 0.05 U mL(-1)), 15 with severe HB, and five with moderate HB who required treatment for episodic bleeds, postoperative haemostasis and for primary or secondary prophylaxis. The HA patients bled more frequently than HB patients (14.4 vs. 8.63 bleeds/patient/year), but used similar amounts of concentrate per year. HA patients underwent surgical procedures 3.2 times more frequently than HB patients to correct musculoskeletal complications. A total of 21,363,409 IU of recombinant FVIII was used by patients with HA (104,722 IU/patient/year) and 6,430, 960 IU of recombinant factor IX, by patients with HB (107,182 IU/patient/year). The difference in factor concentrate usage is not statistically significant (P > 0.05). The decrease in bleed frequency in haemophilia B indicates that the conclusions from randomized trials of prophylaxis in HA may not be accurately applied to HB.
authors.affiliation: Hamilton Health Sciences, McMaster University, Hamilton, ON L8N3Z5, Canada.
authors.forename: K
authors.initials: K
authors.lastname: Nagel
authors.forename: I
authors.initials: I
authors.lastname: Walker
authors.forename: K
authors.initials: K
authors.lastname: Decker
authors.forename: A K C
authors.initials: AK
authors.lastname: Chan
authors.forename: M K
authors.initials: MK
authors.lastname: Pai
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003029:Coagulants / Q000008:administration & dosage*; D005164:Factor IX / Q000008:administration & dosage*; D005169:Factor VIII / Q000008:administration & dosage*; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy*; D002836:Hemophilia B / Q000150:complications* / Q000188:drug therapy*; D006470:Hemorrhage / Q000453:epidemiology*; D006801:Humans; D007223:Infant; D008875:Middle Aged; D011994:Recombinant Proteins / Q000008:administration & dosage; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D003029:Coagulants; D011994:Recombinant Proteins; D005169:Factor VIII; D005164:Factor IX