Literature DB >> 28643468

Eltrombopag use in a patient with Wiskott-Aldrich syndrome.

Maria Gabelli1, Antonio Marzollo1, Lucia Dora Notarangelo2, Giuseppe Basso1, Maria Caterina Putti1.   

Abstract

Wiskott-Aldrich syndrome (WAS) is an inherited X-linked disorder characterized by microthrombocytopenia, immunodeficiency, and eczema. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice. Eltrombopag, a thrombopoietin receptor agonist, may be useful to prevent bleeding while awaiting HSCT. We present a case of a male with WAS, profound thrombocytopenia, and bleeding diathesis successfully managed with eltrombopag before HSCT. Eltrombopag was given for 32 weeks obtaining a stable platelet count without any platelet transfusion. The patient did not experience any bleeding symptom. Eltrombopag may be a suitable therapeutic option for patients with WAS and severe thrombocytopenia as "bridge" to definitive cure.
© 2017 Wiley Periodicals, Inc.

Entities:  

Keywords:  Wiskott-Aldrich syndromezzm321990; eltrombopag; hematopoietic stem cell transplantation; thrombocytopenia

Mesh:

Substances:

Year:  2017        PMID: 28643468     DOI: 10.1002/pbc.26692

Source DB:  PubMed          Journal:  Pediatr Blood Cancer        ISSN: 1545-5009            Impact factor:   3.167


  7 in total

Review 1.  Treatment of inherited thrombocytopenias.

Authors:  Carlo L Balduini
Journal:  Haematologica       Date:  2022-06-01       Impact factor: 11.047

Review 2.  Clinical Manifestations and Pathophysiological Mechanisms of the Wiskott-Aldrich Syndrome.

Authors:  Fabio Candotti
Journal:  J Clin Immunol       Date:  2017-10-30       Impact factor: 8.542

Review 3.  Inherited thrombocytopenias: history, advances and perspectives.

Authors:  Alan T Nurden; Paquita Nurden
Journal:  Haematologica       Date:  2020-06-11       Impact factor: 9.941

4.  Lentiviral gene therapy corrects platelet phenotype and function in patients with Wiskott-Aldrich syndrome.

Authors:  Lucia Sereni; Maria Carmina Castiello; Dario Di Silvestre; Patrizia Della Valle; Chiara Brombin; Francesca Ferrua; Maria Pia Cicalese; Loris Pozzi; Maddalena Migliavacca; Maria Ester Bernardo; Claudio Pignata; Roula Farah; Lucia Dora Notarangelo; Nufar Marcus; Lorella Cattaneo; Marco Spinelli; Stefania Giannelli; Marita Bosticardo; Koen van Rossem; Armando D'Angelo; Alessandro Aiuti; Pierluigi Mauri; Anna Villa
Journal:  J Allergy Clin Immunol       Date:  2019-03-27       Impact factor: 10.793

5.  Eltrombopag for the treatment of inherited thrombocytopenias: a phase II clinical trial.

Authors:  Carlo Zaninetti; Paolo Gresele; Antonella Bertomoro; Catherine Klersy; Erica De Candia; Dino Veneri; Serena Barozzi; Tiziana Fierro; Maria Adele Alberelli; Valeria Musella; Patrizia Noris; Fabrizio Fabris; Carlo L Balduini; Alessandro Pecci
Journal:  Haematologica       Date:  2019-07-04       Impact factor: 9.941

Review 6.  Role of Thrombopoietin Receptor Agonists in Inherited Thrombocytopenia.

Authors:  José María Bastida; José Ramón Gonzalez-Porras; José Rivera; María Luisa Lozano
Journal:  Int J Mol Sci       Date:  2021-04-21       Impact factor: 5.923

Review 7.  Clinical Features, Cancer Biology, Transplant Approach and Other Integrated Management Strategies for Wiskott-Aldrich Syndrome.

Authors:  Smitha Hosahalli Vasanna; Maria A Pereda; Jignesh Dalal
Journal:  J Multidiscip Healthc       Date:  2021-12-23
  7 in total

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