A RARE CASE OF BLEEDING DIATHESIS

Fibrinogen deficiency is a very rare inherited bleeding disorder. There are three kinds of fibrinogen deficiency afibrinogenemia, hypofibrinogenemia, and dysfibrinogenemia. Afibrinogenemia is a rare bleeding disorder with an estimated prevalence of 11,000,000. It is an autosomal recessive disease resulting from mutations in any of the 3 genes that encode the 3 polypeptide chains of fibrinogen and are located on the long arm of chromosome 4. Spontaneou bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures are the principal manifestations. Replacement therapy is the mainstay of treatment of bleeding episodes in these patients and plasma-derived fibrinogen concentrate is the agent of choice. Cryoprecipitate and fresh frozen plasma are alternative treatments that should be used only when fibrinogen concentrate is not available.