Pathophysiology: TTP is caused by a defect in ADAMTS13, a metalloproteinase responsible for the cleavage of vWF. This results in a hyper-coagulable state.
Clinical Presentation – Classic Pentad:
- Thrombocytopenia
- Renal Failure
- MAHA
- Fever
- Change in MS
Etiology:
Primary TTP: Idiopathic, autoimmune Abs against ADAMTS13
Secondary TTP: Mechanism unknown. Triggered by cancer, pregnancy etc.
HUS:
Seen more commonly in children. Triad of symptoms (MAHA, renal failure, and thrombocytopenia). Triggered by E. coli 0157:H7 infection.
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