Humanized anti CD-20 as an alternative in chronic management of relapsing thrombotic thrombocytopenic microangiopathy re
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CASE REPORT
Humanized anti CD‑20 as an alternative in chronic management of relapsing thrombotic thrombocytopenic microangiopathy resistant to rituximab due to anti chimeric antibody Pedram Ahmadpoor1 · Cedric Aglae1 · Florian Garo1 · Sylvain Cariou1 · Sophie Renaud1 · Pascal Reboul1 · Olivier Moranne1,2 Received: 26 May 2020 / Revised: 7 September 2020 / Accepted: 1 October 2020 © Japanese Society of Hematology 2020
Abstract Acquired Immune thrombotic thrombocytopenic purpura (iTTP) is considered among clinical situations that needs not only urgent treatment in acute setting but also long term management to prevent relapses. Important progresses have been made in management of these patients that are definitely associated with reduced mortality and relapse rate. However, there are still noticeable percentage of patients that may relapse despite application of modern treatment strategies including preemptive rituximab infusions. Hereby, we share our experience concerning a frequently relapsing iTTP due to development of antirituximab antibody. In our case administration of obinutuzumab, a humanized type II anti CD-20 antibody was associated with complete peripheral blood B cell depletion and increasing plasma ADAMTS-13 activity. Keywords TTPi · ADAMS-13 · DFPP · FFP
Introduction First cases of Thrombotic thrombocytopenic purpura (TTP) explained in the literature about 70 years ago as a fatal disease [1, 2]. Nowadays, the standard treatment of acquired immune TTP consists of rapid elimination of autoantibodies against ADAMTS -13 by plasma exchange, urgent replacement of ADAMTS-13 by infusion of high volume of fresh frozen plasma (FFP) and suppression of autoantibody production by immunosuppression. Plasma exchange significantly reduced the mortality of TTP from more than 80–90% to twenty-eight percent [3]. Moreover, adding rituximab to steroids from the beginning is associated with shorter time to achieve the remission and reduced risk of recurrence [4–7]. More recent innovation in the treatment of acute phase of acquired TTP is adding caplacizumab that * Olivier Moranne olivier.moranne@chu‑nimes.fr 1
Department of Nephrology, Dialysis and Apheresis, University Hospital Caremeau, Nimes, France
Service de Néphrologie, Dialyse, Apherese, Hopital Universitaire Caremeau, 4 place Pr Robert‑Debré, Nimes 3029, France
2
blocks Von Willebrand factor (vwf) attachment to platelets leading to reduce time to recovery [8, 9]. Acquired immune TTP relapses in up to 50% of cases that mandates long term follow up of patients [6, 7, 10]. Hence, considering treatment strategies to reduce the long-term clinical relapse is crucially important. Plasma ADAMTS -13 activity recommended to be monitored regularly (every 3 months for 3 years then less frequently) [11]. Persistently undetectable ADAMTS-13 activity (less than 10%) despite clinical remission is associated with higher risk of relapse [10]. It is shown preemptive rituximab administration in patients on clinical remission but undetectable ADAMTS-13 will reduce signific
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