Caplacizumab in the Treatment of Patients with Recurrent Thrombotic Thrombocytopenic Purpura (TTP)

Abstract

We report two cases of recurrent thrombotic thrombocytopenic purpura, both patients without significant personal pathological antecedents, in which the evolution was favorable following the association of Caplacizumab with the classic treatment with plasmapheresis and cortisone therapy. We studied two cases of thrombotic thrombocytopenic purpura in two young women, without comorbidities or background treatment, who relapsed after the initial treatment with corticotherapy and plasma exchange, after a period of 4 and 6 years with a different clinical picture, compared to the initial one. The difference is given by the presence/absence of neurological manifestations, both at the first presentation and at the relapse of the disease. In both exposed cases, following the association of Caplacizumab with the classic PTT treatment represented by plasmapheresis and cortisone therapy, the patients had a favorable response to therapy and a maintenance of remission at 1,3 and 2+ years after the relapse. In conclusion, the treatment of relapsed thrombotic thrombocytopenic purpura with a humanized monoclonal antibody targeting von Willebrand factor, combined with plasma exchange and corticosteroids, enhances the treatment of this pathology and the remission.

Keywords

• thrombotic thrombocytopenic purpura
• caplacizumab
• platelets
• adamts 13