Timmer Verhaegh, Anais Panossian, Fady Michael, Tanvir Rahman, Aram A Namavar, Zubair Ilyas
Thrombotic microangiopathy (TMA) is a rare, life-threatening hematologic syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ dysfunction. Among TMAs, thrombotic thrombocytopenic purpura (TTP), an emergent subtype caused by severe deficiency of a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13), requires urgent recognition and prompt treatment. We present a 61-year-old woman who initially presented with abdominal pain and hematuria and was treated for a presumed urinary tract infection. She later developed chest pain, unilateral weakness, melena, and petechiae. Laboratory evaluation revealed severe anemia, thrombocytopenia, acute kidney injury, elevated lactate dehydrogenase, and schistocytes on peripheral smear, consistent with microangiopathic hemolysis. Urgent hematologic evaluation raised concern for TTP versus hemolytic uremic syndrome. Her PLASMIC score was high risk, prompting empiric initiation of plasma exchange and corticosteroids prior to confirmatory testing. Brain magnetic resonance imaging (MRI) demonstrated punctate acute infarcts in the right parietal lobe. ADAMTS13 activity returned <10%, confirming acquired TTP. The patient improved with plasma exchange, corticosteroids, and rituximab, and was discharged in stable condition with outpatient follow-up. This case highlights the importance of early recognition, use of clinical prediction tools, and prompt treatment of TTP in patients with multisystem involvement and neurologic deficits to reduce morbidity and mortality.