Title: Management of hypodysfibrinogenemia presenting multiple episodes of internal and external bleeding: a case report
Abstract: BACKGROUND Fibrinogen, major factor of clotting, can impact the bleeding issues clinically precipitating uncontrolled bleeding which is to be managed with plasma transfusion. If its levels or functioning is altered it results in fibrinogen disorders of which Hypo-, Dys-, Hypodys-fibrinogenemia are the three major types. MATERIAL AND METHODOLOGY A single patient of hypodysfibrinogenemia, rarest of its type, presented with multiple episodes of internal & external bleeding and diagnosed with SDH secondary to hypodys-. Due to uncontrolled bleeding, the patient is operative for 2 times and is managed with plasma transfusion. In post-operative period the patient is transfused with Fresh Frozen Plasma and Cryoprecipitate to manage bleeding episodes. RESULTS & CONCLUSION Upon transfusing FFP once every 3 or 4 days, the bleeding is seen within 24 hrs and recurrence of SDH is seen thrice within a time period of 28 days. On transfusion of CRYO the bleeding is not seen for 61 days and even the CT-scan shown reversal of SDH. CRYO can manage bleeding episodes very efficiently in hypodysfibrinogenemia than FFP. However, the evidence provided here is of single patient and is not enough to justify the above proof, but may help in deciding the therapeutic regimen in treating hypodysfibrinogenemia.