Thrombotic thrombocytopenic purpura differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

The main differential diagnosis of TTP is hemolytic-uremic syndrome. TTP should be diffrential from the other disaeses such as TMA syndromes, disseminated intravascular coagulation ,hypertension, immune thrombocytopenic purpura (ITP)

• Malignant hypertension
• Hematological abnormalities
• Ischemic manifestations linked to autoimmune diseases

Differential Diagnosis

The main differential diagnosis of TTP is hemolytic-uremic syndrome (HUS, which has neurosymptoms, renal failure, hypertension and fever). Note that ADAMTS13 activity is normal in HUS.

TTP must be differentiated from

• TMA syndromes
• Disseminated intravascular coagulation
• Hypertension
• Immune hrombocytopenic purpura (ITP)
• Malignant hypertension
• Hematological abnormalities
• Ischemic manifestations linked to autoimmune diseases

Differentiating TTP from other diseases on the basis of Symptoms:

• On the basis thrombocytopenia, MAHA, fluctuating neurological signs, renal faluer and fever, TTP must be differentiated from
  • Autoimmune haemolysis
  • Evans syndrome disseminated intravascular coagulation pregnancy-associated such as:
    • HELLP (haemolysis
    • Elevated liver enzymes and low platelets)
    • eclampsia
  • Haemolytic uraemic syndrome
  • Drugs:
    • Quinine
    • simvastatin
    • interferon
    • Calcineurin inhibitors
  • Malignant hypertension
  • Infections (typically viral (cytomegalovirus, adenovirus, herpes simplex virus)
  • Severe bacterial (meningococcus, pneumococcus)
  • Fungal autoimmune disease (lupus nephritis, acute scleroderma)
  • Vasculitis haemolytic uraemic syndrome (diarrhoea positive/negative)
  • Malignancy catastrophic antiphospholipid syndrome