Clinical disorders of haemostasis

Bleeding disorders

Clinical manifestations

• Platelet / VWF deficiency - skin/mucosa, nose, vaginal, gut bleeding
• Coagulation defects - cavities, intramuscular, bladder, joint space bleeding

Congenital disorders

• VWF deficiency
• Factor VIII deficiency (Haemophilia A)
• Factor IX (Haemophilia B)
• GIIb/IIIA deficiency (Glanzmann thrombasthenia)
  + other rare isolated factor deficiencies

Acquired disorders

• Liver disease
• Acquired thrombocytopenia e.g. idiopathic thrombocytopenia purpura
• DIC (Disseminated intravascular coagulation) > depleted factors
• Anticoagulant or antiplatelet therapy

Prothrombotic disorders

Complications

• Venous thrombus > Pulmonary embolism
• Arterial thrombus > Arterial embolus > Ischaemia/Infarct e.g. stroke, acute limb ischaemia, bowel infarct

Congenital disorders

• Protein C and S deficiency
• Antithrombin III deficiency
• Factor V Leiden (mutation resulting in resistance to protein C inactivation)

Tests of clotting disorders

• Activated partial thromboplastin time - intrinsic pathway e.g. VWF, Factor VIII, IX deficiency + Factor XI, XXII deficiency, Heparin
• Prothrombin time (PT) / International normalised ratio (INR) - extrinsic pathway e.g. Factor V deficiency (rare), warfarin, liver disease