Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P®

Document Type: Research article

Authors

1 Genetic Research Center in Non-Communicable Disease, Zahedan University of Medical sciences

2 Hematology Research Center, Shiraz University of MedicalSciences, Shiraz, Iran

3 Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran

4 Affiliation

Abstract

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.

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