Long term follow up study on a large group of patients with congenital factor XIII deficiency treated prophylactically with fibrogammin P®

Factor XIII deficiency (FXIIID) is an extremely rare hemorrhagic disorder with a
prevalence of 1/3-5 million. Management of disease is performed by fresh frozen plasma
(FFP), Cryoprecipitate (CP) or FXIII concentrate (Fibrogammin P®). Our objective was to
assess safety and effectiveness of Fibrogammin P® in patients with FXIIID. For this purpose
we designed this long-term follow up study on a large group of patients with FXIIID.
This prospective study was conducted on 213 patients with FXIIID since 2009 to 2013.
Administrated dose for Fibrogammin P® according to clinical situations of patients ranged
from 10 to 26 IU/kg every 4-6 weeks. All patients in 6-month intervals were checked for human
immunodeficiency virus (HIV), hepatitis A, B and C viruses (HAV, HBV, HCV).
Twelve percent of participants had at least one ICH episode until 2008 but after administration
of Fibrogammin P® did not have any major bleeding or episode of ICH, except in one patient.
We also had 7 females with recurrent miscarriage that were managed successfully with a dose
of 10 to 26 IU/kg every 4-6 weeks. This dose also was quite successful in management of
major and minor surgery. None of the participants showed allergic reaction during treatment. A
total of 7155450 IU of Fibrogammin P® were infused but nobody was positive for HIV, HAV,
HBV, and HCV. We found that Fibrogammin P® is a safe and effective therapeutic choice in
management of FXIIID