Bleeding disorders

Definition | Aetiology | Pathophysiology | Risk Factors | Signs and Symptoms | Investigations | Management

Definition

Bleeding disorders refer to conditions that impair the blood clotting process, leading to excessive or prolonged bleeding following injury, surgery, or spontaneously.

Aetiology

  • Platelet disorders: thrombocytopenia, platelet dysfunction.
  • Coagulation factor deficiencies: haemophilia A (factor VIII deficiency), haemophilia B (factor IX deficiency), von Willebrand disease.
  • Acquired disorders: liver disease, disseminated intravascular coagulation (DIC), vitamin K deficiency.
  • Medication-induced: anticoagulants (warfarin, heparin), antiplatelets (aspirin, clopidogrel).

Pathophysiology

  • Defects in primary haemostasis (platelet dysfunction) lead to mucosal and skin bleeding (petechiae, purpura).
  • Defects in secondary haemostasis (coagulation factor deficiencies) lead to deep tissue and joint bleeding.
  • Acquired conditions (e.g., DIC) disrupt normal clotting pathways, leading to both thrombosis and bleeding.

Risk Factors

  • Family history of bleeding disorders.
  • Use of anticoagulant or antiplatelet medication.
  • Chronic liver disease.
  • Recent infection or malignancy (e.g., leukaemia-associated thrombocytopenia).

Signs and Symptoms

  • Easy bruising.
  • Frequent nosebleeds (epistaxis).
  • Prolonged bleeding from minor cuts.
  • Menorrhagia (heavy menstrual bleeding).
  • Haemarthrosis (joint bleeding in haemophilia).
  • Gastrointestinal or intracranial haemorrhage in severe cases.

Investigations

  • Full blood count (FBC): assesses platelet count.
  • Prothrombin time (PT) and activated partial thromboplastin time (APTT): screens for coagulation disorders.
  • Bleeding time: evaluates platelet function.
  • Factor assays: measure specific coagulation factor deficiencies.
  • Von Willebrand factor antigen and activity: assesses von Willebrand disease.
  • Bone marrow biopsy: if haematological malignancy is suspected.

Management

1. General Management:

  • Avoidance of NSAIDs and aspirin.
  • Optimise coagulation status before surgery or procedures.

2. Specific Treatments:

  • Haemophilia: recombinant factor VIII or IX replacement.
  • Von Willebrand disease: desmopressin (DDAVP) or factor replacement.
  • Platelet disorders: platelet transfusions if severe.
  • DIC: treat underlying cause, supportive care, and blood product replacement if needed.
  • Vitamin K deficiency: vitamin K supplementation.

3. Referral:

  • Haematology: all suspected inherited bleeding disorders or unexplained prolonged bleeding.
  • Gastroenterology: if GI bleeding is present.
  • Obstetrics/gynaecology: for heavy menstrual bleeding related to a bleeding disorder.
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