Refractory acquired thrombotic thrombocytopenic purpura in a patient with sickle cell trait successfully treated with caplacizumab.

ABSTRACT

Methods: We report a case of a 20-year-old Nigerian male who presented with acquired thrombotic thrombocytopenic purpura (aTTP) and sickle cell trait. The coexistence of published cases of TTP and sickle cell hemoglobinopathies is rare.Results: Despite the initial treatment with plasma exchange and glucocorticoids, our patient relapsed and also required caplacizumab which resulted in successful remission.Discussion: We conclude by reviewing the cases of TTP in patients with sickle cell hemoglobinopathies and review how vaso-occlusive crises with multiorgan injury can mimic TTP.Conclusion: Ours is the first published case of aTTP with confirmed ADAMTS13 autoantibodies in a patient with a sickle cell hemoglobinopathy and contributes to the literature on the successful use of caplacizumab in clinical practice.