Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypofibrinogenemia

A Case Report of Congenital Afibrinogenemia

“…Secondary prophylaxis may be commenced to prevent re-bleeding, particularly following lifethreatening hemorrhages, which commonly consist of weekly infusions. Infusions once 29 two weeks, or a month may be applied as well [17,18]. Our patient was on primary prophylaxis with fibrinogen concentrates once two weeks, prolonged to once a month upon lack of re-bleeding episodes.…”

Management of Congenital Afibrinogenemia: Report of Two Cases

“…A weekly infusion is a widely accepted regimen for treating hematologist, but monthly and every 2 weeks regimens have also been used [10]. Surprisingly, the frequency of bleeding among the group who enrolled in the preventive program is the same as the group who received the replacement therapy on-demand [12]. The desired therapeutic target is to maintain the level of fibrinogen above 0.5 g/L [13].…”

“…Several preventive measures have been proposed to prevent the occurrence of catastrophic bleeding in patients with congenital afibrinogenemia [12]. A weekly infusion is a widely accepted regimen for treating hematologist, but monthly and every 2 weeks regimens have also been used [10].…”

Management of Inherited Bleeding Disorders in Pregnancy

“…If fibrinogen concentrate is not available, cryoprecipitate can be used. 190 Antenatal prophylaxis with fibrinogen replacement therapy is recommended for severe fibrinogen deficiency (for reducing the risk of bleeding and pregnancy loss) and there is evidence of better outcomes if started preconception. 164 Higher doses of fibrinogen concentrate are likely to be required to maintain fibrinogen activity as pregnancy progresses.…”

“…122,131,189,190 Partial quantitative (hypofibrinogenaemia), with or without qualitative defects (dysfibrinogenaemia), can be autosomal dominant in inheritance and may be asymptomatic or associated with a variable risk of bleeding and/or venous and arterial thrombosis. [191][192][193][194] Poor wound healing and splenic rupture have also been reported with severe and partial fibrinogen deficiency.…”