Table 3. Bleeding Disorders and Associated Conditions Presenting With Normal Initial Hematologic Laboratory Study Results
ConditionRecommended Screening Tests
VWD23*VWF antigen, VWF ristocetin cofactor activity, and FVIII activity assays
FXIII deficiency59Quantitative functional FXIII assay (ammonia-release or amine-incorporation assay)
Fibrinolytic disorders (α2-antiplasmin and PAI-1 deficiencies)13,56,57,60Specific functional (activity) and antigen assays
Collagen disorders (eg, EDS, BJHS)58Hypermobility assessment tools (eg, Beighton score, Brighton criteria)58

  • ↵* Initial hematologic laboratory studies will be normal in only some cases of von Willebrand disease (VWD). Nevertheless, additional screening tests should be performed before excluding VWD based on a normal activated partial thromboplastin time or Platelet Function Analyzer, when clinically indicated.

  • BJHS, benign joint hypermobility syndrome; EDS, Ehlers-Danlos syndrome; FXIII, factor XIII; FVIII, factor VIII; PAI, plasminogen activator inhibitor; VWF, von Willebrand factor.