Thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura
Updated: 07/25/2023
© Jun Wang, MD, PhD

General features
Clinical presentations
  • Pentad
    • Microangiopathic hemolytic anemia
    • Thrombocytopenic purpura
    • Neurologic abnormalities: Alteration of mental status, seizures, hemiplegia, paresthesias, visual disturbance, and aphasia
    • Fever
    • Renal disease: Abnormal renal functions
  • May have history of bloody diarrhea, need to differentiate from HUS
Key pathogenesis
  • ADAMTS13 (Adam is 13) deficiency associated ultra-large von Willebrand factors
  • Deficiency can be either congenital or due to autoantibodies
  • Endotoxic (Escherichia coli O157:H7 and Shigella dysenteriae serotype I) induced endothelial cell secretion of ultra-large von Willebrand factors
  • Ultra-large von Willebrand factors tends to promote platelet adhesion and aggregate, resulting in thrombosis
  • Medications: Mitomycin, cyclosprorin A, cisplatin, etc
  • Pregnancy, probably anti-endothelium autoantibodies
Key Laboratory findings
  • Thrombocytopenia
  • Hemolytic anemia
  • Hemoglobinuria
  • Other lab findings of hemolysis
  • Fragmented red cells (Schistocytosis)
  • Usually normal PT, aPTT, D-dimers (different from coagulative disorders, DIC)
  • Fibrinogen may be elevated
  • Low ADAMTS13 quantity/activity
Key morphological features

Differential diagnosis


Management
  • Medical emergency
  • Plasma exchange
  • Cryopoor plasma or corticosteroid
  • NO platelet transfusion

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