Thrombin generation in platelet-poor plasma is normal in patients with hereditary mucocutaneous haemorrhages.
Mild hereditary bleeding disorders presenting with mucocutaneous haemorrhages are usually difficult to diagnose. We measured thrombin generation in platelet-poor plasma (TG-PPP) in 206 patients with a clinically unequivocal bleeding tendency: 45 with von Willebrand disease (vWD), 49 with platelet aggregation/secretion defects (PASD), 10 with a combination of both and 102 who did not fit the diagnostic criteria for any known haemostatic disorder. TG-PPP was not significantly different from controls in all patient groups, indicating that an abnormality in the plasmatic clotting system is unlikely to contribute to the bleeding in patients with type 1 vWD and PASD. In patients with undiagnosed mild hereditary bleeding disorders, there must be other mechanisms which explain the abnormal haemorrhagic tendency, most likely as yet unrecognized defects in platelet-vessel wall interaction. As a next step we plan to investigate thrombin generation in PRP.
Quiroga
Teresa
T
Department of Clinical Laboratory, School of Medicine, Catholic University of Chile, Santiago, Chile. tquiroga@med.puc.cl
Goycoolea
Manuela
M
Giesen
Peter L A
PL
Morales
María
M
Muñoz
Blanca
B
Aranda
Eduardo
E
Rodríguez
Soledad
S
Panes
Olga
O
Martínez
Carlos
C
Pereira
Jaime
J
Mezzano
Diego
D
D000293:Adolescent; D000328:Adult; D001780:Blood Coagulation Tests; D001791:Blood Platelet Disorders / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003374:Coumarins / Q000032:analysis; D004730:Endothelium, Vascular / Q000473:pathology; D005260:Female; D005456:Fluorescent Dyes / Q000032:analysis; D005470:Fluorometry; D006474:Hemorrhagic Disorders / Q000097:blood* / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009092:Mucous Membrane / Q000098:blood supply; D009842:Oligopeptides / Q000032:analysis; D010949:Plasma; D010976:Platelet Count; D011446:Prospective Studies; D012867:Skin / Q000098:blood supply; D013917:Thrombin / Q000032:analysis / Q000096:biosynthesis*; D014842:von Willebrand Diseases / Q000097:blood
D003374:Coumarins; D005456:Fluorescent Dyes; D009842:Oligopeptides; C411346:benzyloxycarbonyl-glycyl--glycyl-arginine-4-methylcoumaryl-7-amide; D013917:Thrombin