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PERSPECTIVE article

Front. Immunol.
Sec. Primary Immunodeficiencies
Volume 15 - 2024 | doi: 10.3389/fimmu.2024.1517543

Expert Insights on Hodgkin's Lymphoma Development in an Activated PI3K Delta Syndrome Patient Undergoing Leniolisib Treatment

Provisionally accepted
Francesca Conti Francesca Conti 1,2Mattia Moratti Mattia Moratti 3*Elena Sabattini Elena Sabattini 4Pier Luigi Zinzani Pier Luigi Zinzani 2,5
  • 1 Pediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Emilia-Romagna, Italy
  • 2 Department of Medical and Surgical Sciences, University of Bologna, Bologna, Emilia-Romagna, Italy
  • 3 Specialty School of Paediatrics, University of Bologna, Bologna, Emilia-Romagna, Italy
  • 4 Haematopathology Unit, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Bologna, Italy
  • 5 IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli", Bologna, Italy

The final, formatted version of the article will be published soon.

    Activated PI3K Delta Syndrome (APDS) is a primary immunodeficiency that is caused by mutations in the PI3K signalling pathway resulting in either gain of function or loss of function phenotypes of APDS 1 and 2. Malignancy is one of the most serious complications associated with APDS patients, with the most commonly occurring of these being lymphoma, and is the most common cause of death in APDS patients.Management of APDS is complex and variable due to the heterogeneous nature of the disease and ranges from antimicrobial and immunosuppressant agents to Haematopoetic Stem Cell Transplantation. More recently, an increasing level of interest has been shown in the use of more targeted agents such as PI3Kδ specific inhibitors.Here we provide expert perspective on the suspected causality of a case of lymphoma observed in a 20-year-old female patient who was included in a clinical trial of leniolisib, a PI3K inhibitor.

    Keywords: Activated PI3K Delta Syndrome (APDS), Leniolisib, Hodgkin (cHL), PI3K inhibitor, Hodgkin lymphoma (HL)

    Received: 26 Oct 2024; Accepted: 11 Dec 2024.

    Copyright: © 2024 Conti, Moratti, Sabattini and Zinzani. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Mattia Moratti, Specialty School of Paediatrics, University of Bologna, Bologna, 40126, Emilia-Romagna, Italy

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.