Overview
Definition
Life-threatening microangiopathic hemolytic anemia.
Epidemiology
Medical emergency. Higher incidence in women.
Etiology & Risk Factors
- Deficiency or autoantibody against ADAMTS13 (metalloprotease that cleaves vWF)
Clinical Symptoms
- Pentad: Thrombocytopenia, Microangiopathic Hemolytic Anemia (MAHA), Neurologic symptoms, Renal failure, Fever
Clinical Approach
Diagnosis
- Smear (Schistocytes)
- High LDH
- Low platelets
- Normal PT/aPTT
Management
- Immediate Plasmapheresis (Plasma exchange)
- Corticosteroids
- DO NOT give platelets
Complications
- Stroke
- Renal failure
- Death (90% mortality if untreated)