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BMJ Case Reports 2013; doi:10.1136/bcr-2012-007303
  • CASE REPORT

Factor XIII deficiency presenting with intracerebral bleed

  1. Anil Kumar Tripathi2
  1. 1Department of Medicine, C.S.M. Medical University (Erstwhile K.G.M.U.), Lucknow, Uttar Pradesh, India
  2. 2Department of Clinical Hematology, C.S.M. Medical University, Lucknow, Uttar Pradesh, India
  1. Correspondence to Dr Kamal Kumar Sawlani, kksawlani{at}gmail.com
  • Received 30 August 2012
  • Revised 29 November 2012
  • Accepted 7 December 2012
  • Published 10 January 2013

Summary

It has been recognised for some time that many disorders such as vascular malformations, hypertension, collagen vascular diseases, tumours, eclampsia, central nervous system infection, bacterial endocarditis and blood dyscrasias can cause non-traumatic intracerebral haemorrhage in young adults. Factor XIII deficiency is a rare cause of intracranial bleed. The impressive clinical signs with bleeding starting in the neonatal period (prolonged bleeding from the umbilical cord), followed by severe, life-threatening episodes of intracranial haemorrhage should raise the clinical suspicion of factor XIII deficiency. Tests for factor XIII deficiency in high index clinical suspicious cases despite negative coagulative screening tests are essential for diagnosis. The diagnosis of factor XIII deficiency is difficult but has important therapeutic consequences. We are reporting here the clinical outcome of a young woman with intracranial bleed due to factor XIII deficiency. The patient was managed successfully with fresh frozen plasma transfusion and supportive treatments. The prophylactic substitution therapy of factor XIII (recombinant factor XIII, cryoprecipitate and fresh frozen plasma) enables patients to live normal lives, free from catastrophic bleeding episodes.

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