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Thrombotic thrombocytopenic purpura
Johanna A Kremer Hovinga1,2, Paul Coppo3, Bernhard Lämmle1,4
1Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, Freiburgstrasse, Bern, Switzerland.
Nature Reviews. Disease Primers
|April 7, 2017
Summary
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening condition caused by a deficiency in ADAMTS13 activity. Early diagnosis and plasma therapy significantly improve survival rates for TTP patients.
Area of Science:
- Hematology
- Internal Medicine
- Pathophysiology
Background:
- Thrombotic thrombocytopenic purpura (TTP), also known as Moschcowitz disease, presents with thrombocytopenia, microangiopathic hemolytic anemia, and organ damage.
- Historically, TTP had a high mortality rate, but plasma therapy has improved survival to 80-90%.
- Despite improved survival, TTP patients face risks of relapse and long-term complications, necessitating timely and accurate diagnosis.
Purpose of the Study:
- To elucidate the pathophysiology of TTP, focusing on ADAMTS13 deficiency.
- To differentiate TTP from other thrombotic microangiopathies with similar clinical presentations.
- To outline current therapeutic strategies for congenital and acquired TTP.
Main Methods:
- Review of the underlying pathophysiology of TTP, emphasizing the role of ADAMTS13.
- Comparison of TTP clinical presentation with other conditions like hemolytic uremic syndrome.
- Discussion of therapeutic interventions including plasma exchange and immunosuppressive agents.
Main Results:
- TTP pathophysiology involves deficiency of ADAMTS13, a protease cleaving von Willebrand factor (vWF) strings.
- Ultra-large vWF strings lead to microthrombi formation and organ damage.
- Congenital TTP results from ADAMTS13 mutations, while acquired TTP is autoimmune.
Conclusions:
- Accurate diagnosis of TTP is crucial but challenging due to overlapping symptoms with other microangiopathies.
- Plasma exchange is a cornerstone therapy for TTP.
- Immunosuppressive therapy, including corticosteroids and rituximab, is vital for acquired TTP management.