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Published on: September 5, 2016
Thrombotic microangiopathy and indications for therapeutic plasma exchange
1Department of Laboratory Medicine and Pathology, Mayo Clinic Arizona, Phoenix, AZ.
Abstract:
Thrombotic microangiopathy (TMA) is a clinicopathological condition associated with a wide variety of medical conditions. TMA is classically characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombi that cause end-organ damage. The most prominent diagnoses associated with TMA are thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Although TTP and HUS can have similar clinical and laboratory features and are often lumped together as a combined entity referred to as "TTP/HUS," the pathologic processes causing TMA and optimal therapies for these conditions are different. Empiric use of therapeutic plasma exchange (TPE) in the setting of TMA is common. The high risk of morbidity and mortality associated with some causes of TMA justify rapid institution of this relatively low-risk procedure. However, many causes of TMA do not respond to TPE and prolonged courses of exchange in the absence of an underlying diagnosis may cause a detrimental delay in appropriate medical therapy. The American Society of Apheresis has published guidelines for the use of TPE for several distinct conditions associated with TMA. This list is not comprehensive and the use of TPE for other causes of TMA may be considered if the mechanism of the underlying disease process provides a clear rationale for this intervention.
Insights
Thrombotic microangiopathy (TMA) involves anemia, low platelets, and clots causing organ damage. While therapeutic plasma exchange (TPE) is common, it
Area of Science:
- Hematology
- Pathology
- Internal Medicine
Background:
- Thrombotic microangiopathy (TMA) is a serious condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombi.
- Key diagnoses include thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), which share clinical features but differ in underlying pathology and treatment.
- Therapeutic plasma exchange (TPE) is frequently used empirically for TMA, despite varying efficacy depending on the specific cause.
Purpose of the Study:
- To review the clinicopathological spectrum of TMA.
- To discuss the role and limitations of therapeutic plasma exchange (TPE) in managing TMA.
- To highlight the importance of accurate diagnosis for guiding appropriate therapy.
Main Methods:
- Review of existing literature and clinical guidelines.
- Analysis of the pathophysiology of different TMA subtypes.
- Evaluation of the efficacy and indications for therapeutic plasma exchange (TPE).
Main Results:
- TMA presents with anemia, thrombocytopenia, and end-organ damage due to microvascular thrombi.
- TTP and HUS are distinct entities within the TMA spectrum, requiring tailored treatments.
- TPE is beneficial for some TMA causes but may delay definitive therapy in others.
Conclusions:
- Accurate diagnosis is crucial for effective TMA management.
- The use of TPE should be guided by the specific underlying cause of TMA.
- Further research is needed to optimize treatment strategies for diverse TMA etiologies.
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