Abstract
Thrombotic microangiopathy (TMA) is a group of diseases that are life-threatening and can lead to end organ damage (EOD) due to ischemia caused by microthrombi in capillaries and arterioles. TMAs can affect any organ system but usually affect the kidney, intestines, and nervous system. The triad of TMA is Coombs-negative hemolytic anemia with schistocytes seen on peripheral smear, thrombocytopenia (platelets under 150,000 or a decrease of 25% or more from baseline), and evidence of ischemic EOD. Primary TMAs include Thrombotic Thrombocytopenic Purpura (TTP), Hemolytic Uremic Syndrome (HUS) and atypical HUS (aHUS). Pathophysiologically, all of these diseases are caused by aggregation of von Willebrand Factor (vWF) multimers, via different mechanisms, which eventually leads to thrombus formation. TTP and aHUS benefit from plasma exchange (PEX), whereas HUS is treated symptomatically. Urgent recognition with timely treatment is crucial to managing these potentially life-threatening conditions.
Highlights
Thrombotic microangiopathy (TMA) is a group of diseases that are life-threatening and can lead to end organ damage due to ischemia caused by microthrombi in capillaries and arterioles [1]
The triad of TMA is Microangiopathic Hemolytic Anemia (MAHA), defined as Coombs-negative hemolytic anemia with schistocytes seen on peripheral smear, thrombocytopenia, and evidence of ischemic end organ damage (EOD) [2]
It is important for the clinician to confirm MAHA on a peripheral smear and thrombocytopenia on CBC
Summary
Thrombotic microangiopathy (TMA) is a group of diseases that are life-threatening and can lead to end organ damage due to ischemia caused by microthrombi in capillaries and arterioles [1]. A useful way for the clinician to categorize TMA is primary versus secondary, with primary syndromes being symptoms caused by the main disease process and secondary being those resulting from a systemic disease. As a general primary TMAs usually involved kidney injury and have acute onset of several days duration Exceptions to this rule are that 1) TTP commonly does. Miscellaneous Causes of TMAs Drug-induced (Quinine, VEGF inhibitors, clopidogrel, ticlopidine) Cobalamin deficiency Infection/Sepsis related (especially HIV) Autoimmune (Systemic lupus erythematosus, scleroderma renal crisis, antiphospholipid syndrome) Malignancy related Pregnancy related (HELLP, pregnancy-related TTP, pre-eclampsia, eclampsia) Disseminated intravascular coagulation Hematopoietic stem-cell transplant-related Severe hypertension-related not present with kidney injury [1], and 2) that drug mediated TMAs tend to take place immediately over a couple hours duration.
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