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.

Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology

“…Notably, there may be therapeutics used to treat TMAs that could have therapeutic benefits for patients with SCD, particularly options that may prove to be viable intervention options for acute crisis. For example, Caplacizumab, a VWF platelet-binding antagonist, used in TTP, has not been tested in the context of SCD management [ 38 ▪ ]. ULVWF multimers are described in the literature as hyperreactive with the enhanced ability to bind platelets.…”

A Rare Association of Thrombotic Thrombocytopenic Purpura with Systemic Lupus Erythematosus in a Sudanese Woman: Case Report

“…weekly led to dramatic improvement in the patient's condition and normalization of the platelet count. 22 Critical care specialists need to develop a multidisciplinary approach urgently, emphasizing early recognition and initiation of treatment to ensure better outcomes in this scenario.…”