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Secondary Rituximab‐associated vs. primary immunodeficiencies: the enigmatic border

Ottaviano, Giorgio; Sgrulletti, Mayla; Moschese, Viviana; (2022) Secondary Rituximab‐associated vs. primary immunodeficiencies: the enigmatic border. European Journal of Immunology , 52 (10) pp. 1572-1580. 10.1002/eji.202149667. Green open access

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Abstract

Rituximab (RTX), a chimeric monoclonal antibody targeting CD20-positive cells, is a valuable treatment option for malignant and benign immune-related disorders. The rationale of targeting the CD20 antigen relies on depletion of both healthy and autoreactive/malignant CD20-espressing cells, but normal B cell reconstitution is expected within months after treatment. Nevertheless, a number of recent studies have documented prolonged B cell deficiency associated with new-onset hypogammaglobulinemia in patients receiving RTX. Awareness of post-RTX hypogammaglobulinemia has become wider among clinicians, with a growing number of reports about the increased incidence, especially in children. Although these patients were previously regarded as affected by secondary/iatrogenic immunodeficiency, atypical clinical and immunological manifestations (e.g. severe or opportunistic infections; prolonged B cell aplasia) raise concerns of delayed manifestations of genetic immunological disorders that have been unveiled by B cell perturbation. As more patients with undiagnosed primary immune deficiency (PID) receiving RTX have been identified, it remains the challenge in discerning those that might display a higher risk of persistent RTX-associated hypogammaglobulinemia and need a tailored immunology follow-up. In this review we summarize the principal evidence regarding post-RTX hypogammaglobulinemia and provide a guideline for identifying patients at higher risk of RTX-associated hypogammaglobulinemia that could harbor an inborn error of immunity.

Type: Article
Title: Secondary Rituximab‐associated vs. primary immunodeficiencies: the enigmatic border
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/eji.202149667
Publisher version: https://doi.org/10.1002/eji.202149667
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Rituximab, hypogammaglobulinemia, B cell depletion, inborn errors of immunity secondary, immunodeficiency
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
URI: https://discovery.ucl.ac.uk/id/eprint/10153162
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