PATHOLOGIES
Thrombotic Microangiopathy (TMA)
Thrombotic Microangiopathy (TMA) is a group of rare but serious disorders characterized by the formation of small blood clots (thrombi) in the tiny blood vessels (microvasculature) throughout the body. These clots can block blood flow, causing organ damage, especially in the kidneys, brain, and heart. TMA is associated with two primary features: microangiopathic hemolytic anemia (MAHA), where red blood cells are destroyed as they pass through the narrowed vessels, and thrombocytopenia, a low platelet count due to excessive clot formation.
Thrombotic Microangiopathy (TMA)
TMA can occur as part of several conditions, including thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and as a complication of certain diseases like lupus, infections, or cancer. It may also be triggered by medications, pregnancy, or bone marrow transplantation.
Symptoms of TMA depend on the organs affected and may include fatigue, jaundice (due to anemia), easy bruising or bleeding (due to low platelets), confusion, seizures, high blood pressure, kidney dysfunction, and chest pain. In severe cases, TMA can lead to multi-organ failure.
Diagnosis involves blood tests showing signs of MAHA (schistocytes on a blood smear), thrombocytopenia, and evidence of organ dysfunction, along with specialized tests to identify specific causes, such as ADAMTS13 levels in TTP or shiga toxin in HUS.
Treatment depends on the underlying cause. TTP often requires urgent plasma exchange therapy and steroids, while HUS may require supportive care, dialysis, or targeted therapies like eculizumab. Managing the trigger, such as infections or autoimmune disease, is also critical.
TMA is a life-threatening condition requiring prompt diagnosis and treatment. Advances in targeted therapies have improved outcomes for many patients, but early recognition remains essential.
