Search

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The authors declare that this study received funding from PharmingGroup NV. The funder had the following involvement in the study: financial support for the research and publication of this article. Medical writing and editorial assistance was provided by Julie Howard Ph.D. of Inspire Medical Affairs Ltd and was funded by Pharming Group NV, in accordance with Good Publications Practice guidelines. Experts were contracted by Pharming to provide their expert perspectives., (Copyright © 2025 Conti, Moratti, Sabattini and Zinzani.)