Thrombotic microangiopathy (TMA) is a heterogenous group of rare disorders characterized by thrombocytopenia and microangiopathic hemolytic anemia and end organ damage that can occur due to ischemic injury of organs, such as renal injury or stroke. Because of the variety of TMA disorders and different treatment options for each, prompt and accurate diagnosis can significantly impact a patient’s outcome.
Thrombotic microangiopathy Test menu
Optimizing treatment for challenging conditions
Plasma exchange — the treatment of choice for iTTP patients — has been shown to reduce morbidity from around 90% to less than 20%. TTP may be confirmed with ADAMTS13 activity and inhibition studies.
Patients diagnosed with STEC-HUS benefit from early supportive interventions. Intravenous volume expansion can potentially decrease renal damage. Antibiotics, which can cause the release of toxins, are to be avoided.
Management of TMA associated with other secondary causes involves identifying and treating the underlying cause. Medication-induced TMA is initially managed by withdrawing the causative medication. The diagnosis of aHUS, which is caused by a dysregulation of the complement alternative pathway, is one of exclusion. Complement serologic and genetic testing are useful to support a clinical diagnosis of aHUS, and management of the condition includes complement inhibition with medications such as eculizumab and ravulizumab.
Thrombotic microangiopathies include the following diagnoses:
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