Congenital factor XIII deficiency associated with von Willebrand disease.
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.
Grand
B
B
Department of hemostasis and Thrombosis, National Academy of Medicine, Buenos Aires, Argentina.
Blanco
A
A
Riveros
D
D
Ventura
A
A
Maugeri
N
N
Woods
A
A
Lazzari
M
M
D005177:Factor XIII Deficiency / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D014842:von Willebrand Diseases / Q000097:blood / Q000150:complications* / Q000175:diagnosis