title: Coagulation and fibrinolysis abnormalities in familial amyloid polyneuropathy.
abstract: OBJECTIVE
  Familial amyloid polyneuropathy (FAP) is an autosomal dominant form of hereditary amyloidosis. Several studies reported coagulation factor X deficiency and excessive fibrinolysis in immunoglobulin light chain amyloidosis. However, few have investigated coagulation and fibrinolysis in FAP. The objective of this study was to determine abnormalities in plasma biomarkers of coagulation and fibrinolysis in FAP.
  
  
  METHODS
  We prospectively recruited eight FAP patients with transthyretin mutations and ten age-matched control patients with other neuropathies in our university. We examined plasma biomarkers of coagulation and fibrinolysis including prothrombin time, activated partial thromboplastin time, fibrinogen, fibrin/fibrinogen degradation products, D-dimer, α2-antiplasmin, antithrombin, plasminogen, thrombin-antithrombin complex, plasmin-α2-antiplasmin complex, prothrombin fragment 1+2, and coagulation factor X. The Mann-Whitney U test was performed for statistical comparisons between FAP and control groups.
  
  
  RESULTS
  FAP patients exhibited significantly decreased levels of coagulation factor X, plasminogen and α2-antiplasmin, and significantly increased levels of prothrombin fragment 1+2 compared to control patients.
  
  
  CONCLUSIONS
  Our results indicate abnormalities of coagulation and fibrinolysis in FAP patients.
authors.affiliation: Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Takara-machi, Kanazawa, Japan.
authors.forename: Ryoichi
authors.initials: R
authors.lastname: Takahashi
authors.forename: Kenjiro
authors.initials: K
authors.lastname: Ono
authors.forename: Tokuhei
authors.initials: T
authors.lastname: Ikeda
authors.forename: Akio
authors.initials: A
authors.lastname: Akagi
authors.forename: Daisuke
authors.initials: D
authors.lastname: Noto
authors.forename: Ichiro
authors.initials: I
authors.lastname: Nozaki
authors.forename: Kenji
authors.initials: K
authors.lastname: Sakai
authors.forename: Hidesaku
authors.initials: H
authors.lastname: Asakura
authors.forename: Kazuo
authors.initials: K
authors.lastname: Iwasa
authors.forename: Masahito
authors.initials: M
authors.lastname: Yamada
mesh_terms: D000328:Adult; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000097:blood*; D001778:Blood Coagulation Disorders / Q000097:blood*; D016022:Case-Control Studies; D005171:Factor X Deficiency; D005260:Female; D005342:Fibrinolysis; D006474:Hemorrhagic Disorders; D006801:Humans; D008297:Male; D008875:Middle Aged; D010446:Peptide Fragments / Q000097:blood; D010958:Plasminogen / Q000172:deficiency; D011446:Prospective Studies; D011498:Protein Precursors / Q000097:blood; D011516:Prothrombin; D018709:Statistics, Nonparametric; D000979:alpha-2-Antiplasmin / Q000172:deficiency
chemical_list: D010446:Peptide Fragments; D011498:Protein Precursors; D000979:alpha-2-Antiplasmin; C029733:prothrombin fragment 1; C044183:prothrombin fragment 2; D011516:Prothrombin; D010958:Plasminogen