A 15-year-old female presented to the emergency depament with history of recurrent epistaxis, hematuria and hematochezia. There was a history of profuse bleeding from the umbilical stump at bih. Previous investigations revealed normal prothrombin time, activated paial thromboplastin time, thrombin time and fibrinogen levels. Her platelet counts as well as platelet function tests were normal but urea clot lysis test was positive. Which one of the following clotting factor is most likely to be deficient?
Factor XIII deficiency causes formation of blood clots that are unstable and susceptible to fibrinolytic degradation by plasmin. As a result, affected individuals have an increased tendency to bleed. Bleeding from the umbilical stump during the first few days of life is common, and intracranial hemorrhage is observed more frequently than in other inherited bleeding disorders. Ecchymoses, muscle hematomas, prolonged bleeding following trauma and hemahroses also are characteristic. PT and aPTT are normal in factor XIII deficiency. Diagnosis of factor XIII deficiency is established by demonstrating increased clot solubility in 5 M urea, dilute monochloroacetic acid, or acetic acid. Factor XIII activity also can be determined quantitatively by a chromogenic assay or by an incorporation assay. Ref: Seligsohn U., Zivelin A., Salomon O. (2010). Chapter 125. Inherited Deficiencies of Coagulation Factors II, V, VII, X, XI, and XIII and Combined Deficiencies of Factors V and VIII and of the Vitamin K-Dependent Factors. In J.T. Prchal, K. Kaushansky, M.A. Lichtman, T.J. Kipps, U. Seligsohn (Eds), Williams Hematology, 8e.
Factor X
Factor XI
Factor XI1
Factor Xlll