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An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura

Bérangère S Joly1, Paul Coppo2, Agnès Veyradier1

  • 1a Service d'hématologie biologique and EA3518 Centre Hayem, Institut Universitaire d'Hématologie, Groupe Hospitalier Saint-Louis - Lariboisière, Assistance Publique - Hôpitaux de Paris, Université Paris Dider , Paris , France.

Expert Review of Hematology
|May 21, 2019
PubMed

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumption thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. Areas covered: VWF/ADAMTS13 interaction is specific, and ADAMTS13 conformation has been elucidated recently. ADAMTS13 deficiency is congenital or acquired. Several targets are used in TTP treatment. Expert opinion: Differential diagnosis of TTP may be challenging and ADAMTS13 investigations are needed. ADAMTS13 conformation could be a promising parameter for TTP diagnosis and prognosis in the coming years. Plasma therapy remains the first-line treatment of the acute phase of TTP. Additional curative and preemptive rituximab are used in acquired TTP. Clinical trials dedicated to innovative drugs are promising.

Keywords:
ADAMTS13Thrombotic microangiopathyautoimmunityrare diseasethrombotic thrombocytopenic purpuravon Willebrand factor

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