title: Severe inhibitor-negative acquired factor XIII/13 deficiency with aggressive subdural haemorrhage.
abstract: Acquired factor XIII (FXIII) deficiency is a common disease and seldom causes bleeding. However, severe FXIII deficiency may result in life-threatening bleeding. Although the inhibitor against FXIII has recently been focused as the cause of haemorrhagic acquired FXIII deficiency, the pathophysiology of inhibitor-negative cases could also be involved. We report a case of an 85-year-old Japanese man with serious subdural haemorrhage showing a remarkable decreased level of FXIII activity. He also manifested complications of compensated disseminated intravascular coagulation (DIC) with chronic renal failure, abdominal aortic aneurysm (AAA) and right renal carcinoma. Despite the successful evacuation of the haemorrhage, acute subdural haemorrhage subsequently developed that necessitated further craniotomies. Plasma cross-mixing studies and dot blot assay revealed no inhibitors against FXIII. We speculated that the decreased FXIII activity could be mainly due to hyperconsumption by DIC and surgery. Because plasma-derived FXIII concentrates are available to stop bleeding, clinicians should be aware of severe acquired inhibitor-negative FXIII deficiency in cases of unexplained excessive bleeding.
authors.affiliation: Department of Medicine (Hematology), Kobe University Graduate School of Medicine, Kobe, Japan. hkawano@med.kobe-u.ac.jp
authors.forename: Hiroki
authors.initials: H
authors.lastname: Kawano
authors.forename: Daisuke
authors.initials: D
authors.lastname: Yamamoto
authors.forename: Yoshito
authors.initials: Y
authors.lastname: Uchihashi
authors.forename: Kanako
authors.initials: K
authors.lastname: Wakahashi
authors.forename: Yuko
authors.initials: Y
authors.lastname: Kawano
authors.forename: Akiko
authors.initials: A
authors.lastname: Sada
authors.forename: Kentaro
authors.initials: K
authors.lastname: Minagawa
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Katayama
authors.forename: Eiji
authors.initials: E
authors.lastname: Kohmura
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Souri
authors.forename: Akitada
authors.initials: A
authors.lastname: Ichinose
mesh_terms: D000369:Aged, 80 and over; D005177:Factor XIII Deficiency / Q000097:blood* / Q000150:complications*; D006408:Hematoma, Subdural / Q000097:blood* / Q000209:etiology*; D006801:Humans; D008297:Male