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Afibrinogenemia Prophylactic Treatment with Fibrinogen Concentrate Haemocomplettan

Afibrinogenemia Prophylactic Treatment with Fibrinogen Concentrate Haemocomplettan

Blood 100(11): Abstract No 1924

Objectives: Afibrinogenemia is a rare coagulation disorder with an estimated incidence of one to two per million. It is inherited as an autosomal recessive trait and occurs mostly in families with consanguity. Congenital afibrinogenemia refers to the lack of potency to produce fibrinogen and renders blood incoagulable. Clinical manifestations are ranging from minimal bleeding to fatal hemorrhage and were usually treated in case of bleeding (on-demand). We started a long-term prophylactic therapy of congenital afibrinogenemia using Haemocomplettan(R) (Aventis Behring, Germany) as a virus-inactivated fibrinogen concentrate. The results of the first year of prophylactic treatment with Haemocomplettan(R) are presented. Methods: Currently, eight patients suffering from afibrinogenemia are included in a registry and were treated with Haemocomplettan(R), a virus-inactivated lyophilised fibrinogen concentrate produced from human blood plasma. Four of them are treated in the Hemophilia-Center in Muenster, the other four patients are treated in four different centers in Germany. Three patients receive Haemocomplettan(R) prophylactically with a dosage of approximately 0.1 g/kg BW every four weeks, one patient is medicated prophylactically every two weeks. The other four patients are treated only in case of bleeding. Bleeding episodes, seroconversion (Hep A, Hep B, Hep C, HIV) and adverse events are documented. Results: Patients are observed since March 2001 until now (18 months) and frequently tested for hepatitis A, B and C as well as for HIV at least two times a year. All patients show no F I activity, a Quick value of 0 % and a PTT of > 180 sec. Under the scheme of prophylactic treatment with Haemocomplettan(R) (60 - 130 mg / kg BW) one bleeding episode (intracranial hemorrhage) was observed. No seroconversion for hepatitis A, B, C or HIV was detected, no adverse events or allergic reactions were reported. Conclusion: Our results suggest that prophylactic treatment of afibrinogenemia with Haemocomplettan(R) is a promising alternative and supplement to the on-demand treatment in case of bleedings. The results confirm virus-safety of Haemocomplettan(R) under routine therapeutic conditions. Due to the short period of observation and the small number of patients as yet, the investigation is ongoing. A registry of all afibrinogenemic patients in Germany is intended. The protocol of the registry will be presented.

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