Factor XIII Deficiency Presenting with Intra-cerebral Hemorrhage

[摘要] Background: Factor XIII deficiency is a rare disorder which generally presents with life threatening bleeds. Case Report: A 44 year old male presented with spontaneous left sided weakness and frontal headache for 3 days. The patient had recurrent episodes of umbilical stump bleeding and recurrent muco-cutaneous bleeds. The patient was transfused with blood components in the past. The coagulation profile showed normal prothrombin time, activated partial thromboplastin time and platelet count. Patient showed decreased clot stability with 5M urea solubility test. CT brain showed right temporal large hemorrhagic contusion. A parieto-occipital craniotomy and evacuation was done after transfusion of cryoprecipitate. Post-operative period was uneventful. Conclusion: The investigation by clot lysis test in the presence of a normal coagulation profile on a case with high index of suspicion is recommended. The patient was successfully managed with cryoprecipitate transfusion and supportive care.

[学科分类] 生理学

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