Thrombotic Microangiopathy

Aka: Thrombotic Microangiopathy

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II. Definitions

  1. Thrombotic Microangiopathy
    1. Life-threatening conditions causing Microangiopathic Hemolytic Anemia, Thrombocytopenia and thrombosis

III. Causes

  1. Heparin Induced Thrombocytopenia
  2. Disseminated Intravascular Coagulation
  3. Drug-Induced Thrombotic Microangiopathic Anemia
    1. See Drug-Induced Thrombotic Microangiopathic Anemia for a list of medication causes
    2. Presents with fever, chills, gastrointestinal symptoms, renal dysfunction and microvascular thrombosis
    3. May be indistinguishable on presentation with TTP
  4. Antiphospholipid Syndrome
    1. History of Recurrent Pregnancy Loss, VTE, CVA
    2. Longterm Aspirin for primary prevention (Anticoagulation for secondary prevention)
  5. Thrombotic Thrombocytopenic Purpura (TTP)
    1. Presents with fever, Renal Failure, microvascular thrombosis (esp. CNS with Headaches)
  6. Hemolytic Uremic Syndrome
    1. Presents with Abdominal Pain, bloody Diarrhea and Renal Failure (esp. children)

IV. Labs

  1. Complete Blood Count
    1. Thrombocytopenia
    2. Microangiopathic Anemia (Anemia with Hemolysis on Peripheral Smear)
  2. Serum Creatinine Increased (Acute Kidney Injury)
    1. Drug-Induced Thrombotic Microangiopathic Anemia
    2. Thrombotic Thrombocytopenic Purpura (in severe cases)
    3. Hemolytic Uremic Syndrome
  3. Lactate Dehydrogenase Increased
    1. Drug-Induced Thrombotic Microangiopathic Anemia
  4. D-Dimer elevated and Fibriniogen Decreased
    1. Disseminated Intravascular Coagulation
  5. ADAMTS13 Acitivity decreased
    1. Thrombotic Thrombocytopenic Purpura
    2. Drug-Induced Thrombotic Microangiopathic Anemia (mildly decreased or normal)
  6. Antiphospholipid Antibody (Anticardiolipin Antibody, Lupus Anticoagulant, anti-beta-Glycoprotein)
    1. Antiphospholipid Syndrome

V. Management

  1. See Thrombocytopenia
  2. Emergent Hematology Consultation
  3. Hospitalization and Supportive Care
    1. High mortality without prompt evaluation and management
  4. Withdraw all suspected causative medications
    1. Drug-Induced Thrombotic Microangiopathic Anemia
    2. Heparin Induced Thrombocytopenia
  5. Plasmapheresis or Plasma Exchange
    1. Complement-mediated Thrombotic Thrombocytopenic Purpura
    2. Drug-Induced Thrombotic Microangiopathic Anemia in which TTP cannot be excluded

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