thrombotic microangiopathy
Also written as: TMA — Thrombotic Microangiopathy
Group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction including acute kidney injury.
Full Definition
Thrombotic microangiopathy (TMA) encompasses several conditions including hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and atypical HUS, all characterized by endothelial damage leading to microvascular thrombosis. In pediatric patients, TMA most commonly presents as post-diarrheal HUS following Shiga toxin-producing E. coli infection. The classic triad includes microangiopathic hemolytic anemia (with schistocytes on blood smear), thrombocytopenia, and acute kidney injury. Atypical forms may be complement-mediated or associated with medications, malignancy, or genetic mutations. Treatment varies by etiology and may include supportive care, plasma exchange, complement inhibitors, or specific targeted therapies.
Usage
Usage note: Commonly abbreviated as TMA; encompasses multiple related but distinct conditions.
In Context
- "The child presented with classic signs of thrombotic microangiopathy following a diarrheal illness." — Emergency department consultation note
- "Genetic testing for atypical thrombotic microangiopathy revealed a complement factor H mutation." — Pediatric hematology-oncology report