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Classic findings in TTP and other primary thrombotic microangiopathies
- Fever
- Anemia
- Thrombocytopenia
- Renal failure
- Altered sensorium
On blood smear, see both schistocytes (3-5 per field) and polychromasia due to reticulocytosis.
Posted 12/06/16 11:36:05 AM by Anna Krigel
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Primary TMA Syndromes
- TTP - hereditary or acquired, TMA resulting from ADAMTS13 deficiency (defined as activity <10%)
- Upshaw-Shulman syndrome = hereditary deficiency of ADAMTS13
- Acquired TTP results from inhibition of ADAMTS13 activity by an autoantibody
- Shiga toxin-mediated HUS
- Drug-induced TMA syndromes
- Complement-mediated TMA - hereditary or acquired
Posted 12/06/16 04:52:04 PM by Anna Krigel
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- Plasma exchange - started urgently in all patients with suspected TTP and no obvious underlying cause for another TMA syndrome; it is not recommended to wait for ADAMTS13 activity based on high mortality of TTP without plasma exchange; unclear if plasma exchange helps in typical HUS
- Glucocorticoids - also given in acquired TTP for immunosuppression
- Eculizumab - monoclonal Ab to complement factor C5, used in patients with complement-mediated HUS, has been shown in case series to be beneficial in patients with typical HUS and neurologic symptoms
Posted 12/06/16 06:44:18 PM by Anna Krigel
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For further reading...
Posted 12/06/16 06:49:27 PM by Anna Krigel
Created by Christopher Kelly
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It is not meant to replace the advice of a health professional.