Diagnosis of factor VIII deficiency.
The correct diagnosis of factor VIII deficiency and the assessment of severity of the disease are essential for a patient-tailored treatment strategy. An optimal diagnostic procedure comprises sensitive and specific screening methods and factor VIII activity assays. Different screening reagents show variable characteristics and receiver operator characteristic curves are presented showing the relation between sensitivity and specificity of eleven activated partial thromboplastin time reagents. The details of the three methods for factor VIII activity assay, one-stage and two-stage assay and chromogenic assays, are discussed. The chromogenic assay seems to be more sensitive than the one-stage assay with regard to the detection of severe haemophilia. Discrepant results obtained with one-stage and two-stage assays are reviewed and discussed.
Radboud University Nijmegen Medical Centre, Central Laboratory for Haematology, Nijmegen, The Netherlands. H.verbnuggen@chl.uncm.nl
B
B
Verbruggen
P
P
Meijer
I
I
Novákova
W
W
Van Heerde
D001777:Blood Coagulation / Q000502:physiology*; D002863:Chromogenic Compounds / Q000302:isolation & purification; D019411:Clinical Laboratory Techniques / Q000295:instrumentation; D006467:Hemophilia A / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D010641:Phenotype; D011933:Reagent Kits, Diagnostic; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D013925:Thromboplastin / Q000302:isolation & purification
D002863:Chromogenic Compounds; D011933:Reagent Kits, Diagnostic; D013925:Thromboplastin