Developments in Thrombotic Thrombocytopenic Purpura

Dear Colleagues,

Thrombotic thrombocytopenic purpura is a severe disease that can lead to significant morbidity and potentially mortality due to the diffuse formation of microthrombi affecting all organ systems. At the center of its pathology is a deficiency in the enzyme ADAMTS13 which, under normal physiological conditions, cleaves von Willebrand factor (vWF) multimers. The majority of the TTP cases are due to the formation of autoantibodies (immune-mediated or iTTP) that bind the enzyme and prevent it from exerting its physiological role. The resulting consumptive thrombocytopenia is profound and, if left untreated, can prove fatal. For over three decades, therapeutic plasma exchange (TPE), an apheresis procedure that exchanges the patient’s plasma with donated plasma, has been the first-line therapy for this disease. The removal of the patient’s plasma containing the offending autoantibody and its replacement with donated plasma containing the deficient ADAMTS13 enzyme lead to the restoration of platelet counts that may often require many TPE procedures. The therapeutic benefit is improved when immunosuppression in the form of steroids is used in conjunction with TPE. Reports outlining the benefits of using the monoclonal rituximab in the setting of TTP continue to grow in the literature. Furthermore, the recent approval and use of the monoclonal antibody caplacizumab, the first TTP-specific therapy that prevents the binding of vWF to platelets, have improved the chances of earlier platelet recovery and result in shorter hospitalizations.

Thus, the goal of this Special Issue is to invite submissions that outline advances in our understanding of TTP. We welcome novel research from basic science to translational and clinical work in TTP as well as comprehensive reviews of available data of the latest discoveries. Submissions of case reports, unless they are in the context of a comprehensive review of the relevant literature, are not encouraged.

Dr. Robert Maitta
Guest Editor

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• thrombotic thrombocytopenic purpura
• thrombocytopenia
• ADAMTS13
• rituximab
• immunosuppression
• therapeutic plasma exchange
• von Willebrand factor