Vaskulárna medicína 1/2023

Acquired thrombotic thrombocytopenic purpura

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a very rare life-threatening disease characterized by a pentad of clinical manifestations

• fever, microangiopathic hemolytic anemia, severe thrombocytopenia, renal insufficiency, and ischemic involvement of the central nervous system (CNS). The cause is a congenital or acquired deficiency of ADAMTS13 activity. Examining the activity of ADAMTS13 helps us to make a quick diagnosis and timely targeted treatment. The current standard in the therapy of acquired TTP is a triple combination: therapeutic exchange plasmapheresis, caplacizumab, and immunosuppressive treatment, thanks to which the morbidity and mortality of patients are decreased significantly. Case description: Our case report describes the successful use of the agent caplacizumab as the 1st line treatment and the complexity of the management in a patient with an acute form of newly diagnosed acquired TTP. Conclusion: TTP is a life-threatening disease, and therefore early diagnosis and initiation of therapy are of utmost importance. Close cooperation with centers specialized in the diagnostics and treatment of hemostasis disorders, equipped with TPE and the possibility of examining ADAMTS13 activity, is necessary.

Keywords: thrombotic thrombocytopenic purpura, caplacizumab, therapeutic plasma exchange, immunosuppressive therapy