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Bleeding Coagulopathies

Hayley Hawkins

  • Bleeding tendency or dysfunction in clot formation can occur from either a quantitative or qualitative platelet defect, deficiency or inhibitor of coagulation factors, or compromise of vascular integrity
  • Platelet disorders will present with mucocutaneous bleeding, petechiae, mild bleeding immediately following surgery, impaired wound healing
  • Coagulation defects will present with deep tissue bleeding in joints/muscles resulting in hemarthroses, hematomas, sometimes delayed but severe bleeding after surgery, and intracranial hemorrhage

Qualitative or Quantitative Platelet Defects

  • Thrombocytopenia: see "thrombocytopenia" section

von Willebrand Disease (vWD)

  • Most common inherited bleeding disorder, but can be secondary or acquired by other disease states
  • Three types
    • Type 1 (most common): quantitative defect with normal vWF function
    • Type 2: qualitative defect with abnormal vWF function despite normal quantity
    • Type 3 (rare): complete absence of vWF, phenotypically similar to hemophilia A (vWF forms complex with factor VIII)
  • Causes of acquired vWD
    • Lymphoproliferative disorders: CLL, NHL, plasma cell dyscrasias
    • Myeloproliferative disorders: PV, ET, or myelofibrosis
    • Autoimmune diseases: SLE
    • Sheer stress: aortic stenosis – Heidi syndrome, LVAD, ECMO
    • Hypothyroidism
  • Diagnosis:
    • “vW Profile” = vWF Ag, Factor VIII Activity, Ristocetin Cofactor Activity
  • Management:
    • Major bleeding or major surgery: give VFW concentrate (some formulations have FVIII)
    • Minor bleeding or surgery or prophylaxis: DDAVP, TXA or aminocaproic acid in some circumstances

Other causes of qualitative platelet defects

  • Uremic platelet dysfunction
  • Medications: NSAIDs, anti-platelets (ASA, Plavix), SSRIs (serotonin required for platelet activation), and melatonin (suggested by pre-clinical studies)

Deficiency or Inhibitor of Coagulation Factors

Hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency)

  • X-linked recessive inheritance
  • Frequently presents with hemarthroses and hematomas
  • Diagnosis: Isolated prolonged PTT with normalization upon mixing study
  • Management: Purified/recombinant Factor VIII or IX, mild disease can be managed with desmopressin, consult Benign Hematology on admission

Acquired FVIII Inhibitor

  • Onset in older age (60s) and frequently presents with significant intramuscular hematomas
  • Disease associations in 50% of cases (other 50% idiopathic): autoimmune (e.g. SLE), malignancy (paraneoplastic), Pemphigus, drug-induced (e.g. interferon, penicillins), or chronic GvHD
  • Diagnosis: Elevated PTT that does not normalize with mixing study
  • Management:
    • Always consult Hematology (rare disorder with major bleeding complications)
    • Bleeding: Typically need a factor VIII bypassing agent (FEIBA)
    • Immunosuppression: prednisone 1mg/kg, rituximab often also used front line

Other Causes

  • Factor VIII deficiency: see vWD above
  • Vitamin K deficiency
    • Caused by malnutrition, liver disease (biliary obstruction), or iatrogenic with warfarin
    • Diagnosis: elevated PT, prolonged PTT if severe, hepatic function panel
    • Management: IV vitamin K 10mg x 3 days
  • DIC and liver dysfunction/cirrhosis
    • A FVIII level can help distinguish between the two (elevated or normal in liver dysfunction and decreased in DIC since it is not hepatically derived)
  • Amyloidosis: can cause factor X deficiency
  • Medications: warfarin, DOACs

Compromise of Vascular Integrity

  • Amyloidosis: can present as GI bleeding, purpura or ecchymoses (e.g. Raccoon eyes)
  • Vasculitis: purpura, alveolar hemorrhage (DAH), intracranial hemorrhage, GI bleeding
  • Vitamin C deficiency: can present with bleeding gums, GI bleeding, ecchymoses, hematomas, anemia
  • AVMs (fragile vessels that easily bleed)
  • Hereditary hemorrhagic Telangiectasia
    • 2nd most common inherited bleeding disorder
    • Often presents with epistaxis, GI bleeding
    • AVMs found in GI tract, lung (may cause shunting), liver (can contribute to cirrhosis), brain
  • vWD
    • von Willebrand multimers play direct role in modulating angiogenesis
  • Heidi Syndrome: acquired vWD due to aortic stenosis, presents with GI bleeding from AVMs