Reduced difference of α₂-plasmin inhibitor levels between plasma and serum in patients with severe factor XIII deficiency, including autoimmune hemorrhaphilia due to anti-factor XIII antibodies.
Coagulation factor XIII/13 (FXIII/13) stabilizes fibrin molecules by creating crosslinks with other fibrin molecules as well as with α₂-plasmin inhibitor (α₂-PI). "Hemorrhagic acquired FXIII/13 deficiency" was formerly considered rare, but has been increasing recently in Japan. During the 10 months of our nationwide campaign, we diagnosed five new patients with "acquired hemorrhaphilia due to anti-FXIII/13 autoantibodies," after examining 20 newly suspected cases of "hemorrhagic acquired FXIII/13 deficiency." When FXIII/13 activity was reduced to less than 50% of normal, it was proportional to the difference in α₂-PI levels between plasma and serum (plasma-serum α₂-PI), likely due to its cross-linking to fibrin by activated FXIII/13. Accordingly, decreased amounts of the plasma-serum α₂-PI ex vivo may reflect reduced FXIII/13 activity in vivo. The plasma-serum α₂-PI may thus also be a useful diagnostic marker for severe FXIII/13 deficiency.
Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, 2-2-2 Iida-Nishi, Yamagata 990-9585, Japan. aichinos@med.id.yamagata-u.ac.jp
Akitada
A
Ichinose
Masayoshi
M
Souri
D001323:Autoantibodies / Q000032:analysis; D015551:Autoimmunity; D015415:Biomarkers / Q000097:blood; D005176:Factor XIII / Q000037:antagonists & inhibitors*; D005177:Factor XIII Deficiency / Q000097:blood* / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology; D006801:Humans; D007564:Japan; D010949:Plasma / Q000378:metabolism; D044967:Serum / Q000378:metabolism; D012720:Severity of Illness Index; D000979:alpha-2-Antiplasmin / Q000032:analysis*
D001323:Autoantibodies; D015415:Biomarkers; C534949:SERPINF2 protein, human; D000979:alpha-2-Antiplasmin; D005176:Factor XIII