Immune Thrombotic Thrombocytopenic Purpura: Diagnosis and Management

Thrombotic thrombocytopenic purpura (TTP), the most common type of which is immune-mediated (iTTP), is a rare, life-threatening hematologic disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and end-organ damage. Prompt recognition and early treatment are essential for survival. Early diagnosis and initiation of treatment are critical in iTTP. Nurses play a key role in recognizing symptoms, supporting patients through treatment, and monitoring for relapse. This review outlines the pathophysiology and clinical presentation of iTTP, along with criteria for diagnosis, with an emphasis on the role of the von Willebrand factor-cleaving protease ADAMTS-13. Current evidence-based treatment strategies are discussed, including therapeutic plasma exchange and immunosuppression with corticosteroids and monoclonal antibody-based therapies. A case study of a middle-aged patient who experienced a relapse of iTTP is presented, illustrating key clinical decision points, challenges in long-term management, and the need for interdisciplinary collaboration.