Congenital Factor XIII deficiency associated with von Willebrand disease

B. Grand, A. Blanco, D. Riveros, A. Ventura, N. Maugeri, A. Woods, M. Lazzari

Research output: Contribution to journalArticlepeer-review


A boy with umbilical bleeding and severe hemorrhages after minor trauma, without family bleeding history, was studied. Coagulation tests showed abnormalities in FXIII subunits and FVIII/vWF complex. Both parents presented results compatible with a heterozygote state for FXIII deficiency and the father had abnormalities of FVIII/vWF. The propositus was diagnosed as congenital FXIII deficiency associated with vWD. No severe hemorrhagic complication was observed after a prophylactic regimen with cryoprecipitates.

Original languageEnglish
Pages (from-to)208-209
Number of pages2
JournalAmerican Journal of Hematology
Issue number3
Publication statusPublished - 1990


  • blood coagulation disorders
  • factor VIII/von Willebrand factor
  • factor XIIIA
  • factor XIIIB

ASJC Scopus subject areas

  • Hematology


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