Hematology: General Bleeding Disorders

Congenital Bleeding Disorders

Von Willebrand’s Disease

Hemophilia

Factor XI Deficiency (Hemophilia C/Rosenthal Syndrome)

  • Deficiency: Factor XI
  • Presentation:
    • Most Do Not Have Spontaneous Bleeding, Even if Disease Severe
    • Increased Bleeding After Trauma or Surgery
  • Severity:
    • Partial: Factor Activity 20-60% of Normal
    • Severe: Factor Activity < 20% of Normal
    • *Bleeding Severity Poorly Correlates to Factor Levels
  • Treatment:
    • Major/Life-Threatening Bleeding: FFP
      • May Consider Factor XI Concentrate (Limited Availability)
    • Goals Prior to Major Surgery: Generally Only Treated Prophylactically for a Significant Bleeding History or if Level is < 20%
      • Otherwise Reserved for Severe/Uncontrolled Bleeding

Other Factor Deficiencies

  • Factor I (Fibrinogen) Deficiency
  • Factor II (Prothrombin) Deficiency
  • Factor V Deficiency
  • Factor VII Deficiency
  • Factor X Deficiency

Platelet Disorders

  • Bernard Soulier
    • Deficiency: GpIb Receptor
    • Defect in Plt-vWF/Collagen Adhesion
  • Glanzmann Thrombasthenia
    • Deficiency: GpIIb/IIIa Receptor
    • Defect in Plt-Plt Adhesion
  • Wiskott-Aldrich Syndrome
    • Deficiency: WAS Protein
    • Presentation:
      • Immunodeficiency
      • Thrombocytopenia (Small Platelets)/Bleeding
      • Eczema
  • General Treatment if Bleeding: Platelet Transfusion

Acquired Bleeding Disorders

General

  • Most Common Cause of Surgical Bleeding: Incomplete Hemostasis
  • Most Common Cause of ICU Coagulopathy: Vitamin K Deficiency

Heparin Induced Thrombocytopenia (HIT)

Disseminated Intravascular Coagulation (DIC)

Hyperfibrinolysis

  • Definitions:
    • Primary Fibrinolysis: Breakdown of Clots Due to Excessive Production of Plasminogen Activator or Decreased Production of Fibrinolysis Inhibitors
    • Secondary Fibrinolysis: Clot Breakdown Due to Secondary Causes
  • Secondary Causes:
    • Fibrinolytic Medications (tPA) – May Be Therapeutic
    • Trauma
    • Cancer
    • Sepsis
    • Can Be a Component of DIC
    • Cirrhosis – Decreased tPA Clearance
    • Transurethral Resection of the Prostate (TURP) – Endogenous Urokinase Release
    • Cardiopulmonary Bypass
  • Labs:
    • PT/PTT: Normal or Prolonged
    • Platelet Count: Normal (Different than DIC)
    • Fibrinogen: Low
    • Fibrin Split Products (D-Dimer): Elevated
    • TEG: Increased LY30
  • Treatment: Treat Underlying Cause
    • Also Consider Tranexamic Acid (TXA) or ε-Aminocaproic Acid

Uremia Induced Platelet Dysfunction

  • Uremia Due to ESRD Causes Platelet Dysfunction with Increased Bleeding Risk
    • Functional Defect in Platelet Adhesion – GP IIb/IIIa Dysfunction
  • Presentation:
    • Prolonged Bleeding from Trauma or Surgery
    • Easy Bruising
    • Mucosal Bleeding
    • GI Bleeding
  • Bleeding Treatment: DDAVP (Desmopressin)
    • Dialysis May Also Help