Relapsing thrombotic thrombocytopenic purpura with low ADAMTS13 antigen levels: An indication for splenectomy?

  • Anna Maria Lombardi | annamaria.lombardi@unipd.it Department of Medicine, University of Padua, Italy.
  • Irene Di Pasquale Department of Medicine, University of Padua, Italy.
  • Maria Antonietta Businaro Department of Medicine, University of Padua, Italy.
  • Irene Cortella Department of Medicine, University of Padua, Italy.
  • Silvia Ferrari Department of Medicine, University of Padua, Italy.
  • Fabrizio Fabris Department of Medicine, University of Padua, Italy.
  • Fabrizio Vianello Department of Medicine, University of Padua, Italy.

Abstract

With more recent modalities of immunosuppression, splenectomy is now rarely considered in refractory/relapsed thrombotic thrombocytopenic purpura (TTP). However, the surgical approach had shown convincing evidences of high efficacy in the pre-rituximab era and therefore may still represent a lifesaving option in selected challenging cases. To define the characteristics of subjects who may benefit from splenectomy may ease clinical decision making. In this paper we describe the clinical and laboratory data of 2 multiple relapsing TTP cases who successfully underwent splenectomy in the pre-rituximab era. Whereas high anti-ADAMTS13 antibody titre and low ADAMTS13 activity never correlated with remission and relapse, a drop in the ADAMTS13 antigen level was always associated with the acute phase, whereas levels consistently returned to normal following splenectomy, heralding long term remission. Splenectomy may therefore be considered in refractory TTP cases associated with increased ADAMTS13 antigen clearance, irrespective of persistence of inhibitory antibodies.

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Published
2019-03-19
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Issue
Section
Case Reports
Keywords:
thrombotic thrombocytopenic purpura, splenectomy
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How to Cite
Lombardi, A. M., Di Pasquale, I., Businaro, M. A., Cortella, I., Ferrari, S., Fabris, F., & Vianello, F. (2019). Relapsing thrombotic thrombocytopenic purpura with low ADAMTS13 antigen levels: An indication for splenectomy?. Hematology Reports, 11(1). https://doi.org/10.4081/hr.2019.7904