[A rare coagulation disorder. Diagnostics and management in cases of hereditary dysfibrinogenemia].
Before elective surgery, it is mandatory that a precise history be taken to detect increased hemorrhagic diathesis and that thrombocytes, Quick/INR, and aPTT be determined. If pathological levels are found, further laboratory tests are necessary after frequent causes (e.g., liver cirrhosis) have been excluded. Single-factor analysis for the von Willebrand's factor antigen and if necessary further tests to check for von Willebrand's syndrome (multimeric analysis) as well as platelet function tests should be performed.Dysfibrinogenemia is a rare coagulation disorder, which causes elevated INR. It shows a wide spectrum of clinical manifestations including thrombophilia, excessive bleeding, and even asymptomatic cases. We present a 72-year-old patient with asymptomatic dysfibrinogenemia who needed hip replacement due to arthrosis. Lowered fibrinogen levels were substituted prior to operation and the clinical course afterwards was uneventful under additional prophylactic anticoagulation in order to prevent thrombosis. The case report illustrates the interdisciplinary teamwork which is very important in the management of patients with coagulation disorders.
Radke
J
J
Medizinische Klinik und Poliklinik I, Universitätsklinikum Carl Gustav Carus, Fetscherstrasse 74, 01307, Dresden, Deutschland. Joergen.Radke@uniklinikum-dresden.de
Teich
M
M
Meyer
M
M
Kirschner
S
S
Neidel
J
J
Ehninger
G
G
Siegert
G
G
Platzbecker
U
U
D000347:Afibrinogenemia / Q000150:complications* / Q000175:diagnosis / Q000628:therapy*; D000368:Aged; D000925:Anticoagulants / Q000008:administration & dosage*; D019644:Arthroplasty, Replacement, Hip / Q000009:adverse effects*; D005260:Female; D006801:Humans; D019990:Perioperative Care / Q000379:methods; D011292:Premedication / Q000379:methods*; D035583:Rare Diseases / Q000517:prevention & control; D013927:Thrombosis / Q000209:etiology* / Q000517:prevention & control*; D016896:Treatment Outcome
D000925:Anticoagulants