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Hematology Reports
Volume 11
Issue 1
10.4081/hr.2019.7904
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Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

Relapsing Thrombotic Thrombocytopenic Purpura with Low ADAMTS13 Antigen Levels: An Indication for Splenectomy?

by
Anna Maria Lombardi
*,
Irene Di Pasquale
,
Maria Antonietta Businaro
,
Irene Cortella
,
Silvia Ferrari
,
Fabrizio Fabris
and
Fabrizio Vianello
Department of Medicine, University of Padua, Via Ospedale 105, 35128 Padua, Italy
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2019, 11(1), 7904; https://doi.org/10.4081/hr.2019.7904
Submission received: 9 October 2018 / Revised: 22 February 2019 / Accepted: 3 March 2019 / Published: 19 March 2019

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.
Keywords: thrombotic thrombocytopenic purpura; splenectomy thrombotic thrombocytopenic purpura; splenectomy

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MDPI and ACS Style

Lombardi, A.M.; Di Pasquale, I.; Businaro, M.A.; Cortella, I.; Ferrari, S.; Fabris, F.; Vianello, F. Relapsing Thrombotic Thrombocytopenic Purpura with Low ADAMTS13 Antigen Levels: An Indication for Splenectomy? Hematol. Rep. 2019, 11, 7904. https://doi.org/10.4081/hr.2019.7904

AMA Style

Lombardi AM, Di Pasquale I, Businaro MA, Cortella I, Ferrari S, Fabris F, Vianello F. Relapsing Thrombotic Thrombocytopenic Purpura with Low ADAMTS13 Antigen Levels: An Indication for Splenectomy? Hematology Reports. 2019; 11(1):7904. https://doi.org/10.4081/hr.2019.7904

Chicago/Turabian Style

Lombardi, Anna Maria, Irene Di Pasquale, Maria Antonietta Businaro, Irene Cortella, Silvia Ferrari, Fabrizio Fabris, and Fabrizio Vianello. 2019. "Relapsing Thrombotic Thrombocytopenic Purpura with Low ADAMTS13 Antigen Levels: An Indication for Splenectomy?" Hematology Reports 11, no. 1: 7904. https://doi.org/10.4081/hr.2019.7904

APA Style

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), 7904. https://doi.org/10.4081/hr.2019.7904

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