[Life-long hemorrhagic diathesis in a young man with unclottable global coagulation tests--congenital afibrinogenemia].
Congenital afibrinogenemia is a rare autosomal recessive hemostatic disorder leading to unclottable global coagulation tests. Furthermore, it is associated with abnormal platelet aggregation and with severe bleeding episodes if untreated. Surprisingly, thrombotic complications may be observed quite frequently in afibrinogenemic patients following replacement of fibrinogen. A case of congenital afibrinogenemia is described in a patient who suffered from severe bleeding episodes in the absence of replacement therapy but developed a deep vein thrombosis with multiple pulmonary emboli after fibrinogen replacement and surgical treatment of a hip fracture, despite conventional heparin prophylaxis.
Peter
K
K
Hämatologisches Zentrallabor der Universität, Inselspital, Bern.
Furlan
M
M
Lämmle
B
B
D000328:Adult; D000347:Afibrinogenemia / Q000097:blood / Q000188:drug therapy / Q000235:genetics*; D001780:Blood Coagulation Tests; D005340:Fibrinogen / Q000008:administration & dosage / Q000009:adverse effects / Q000378:metabolism; D006474:Hemorrhagic Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D006801:Humans; D008297:Male; D011655:Pulmonary Embolism / Q000097:blood / Q000139:chemically induced; D013924:Thrombophlebitis / Q000097:blood / Q000139:chemically induced
D005340:Fibrinogen