A RARE CASE OF BLEEDING DIATHESIS

Abstract

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.