Incidence of malignancy in patients with common variable immunodeficiency according to therapeutic delay: an Italian retrospective, monocentric cohort study

Veronica Pedini; Jacopo Umberto Verga; Irene Terrenato; Denise Menghini; Cristina Mezzanotte; Maria Giovanna Danieli

doi:10.1186/s13223-020-00451-z

Incidence of malignancy in patients with common variable immunodeficiency according to therapeutic delay: an Italian retrospective, monocentric cohort study

Allergy Asthma Clin Immunol. 2020 Jun 26:16:54. doi: 10.1186/s13223-020-00451-z. eCollection 2020.

Authors

Veronica Pedini^{1

2}, Jacopo Umberto Verga³, Irene Terrenato⁴, Denise Menghini¹, Cristina Mezzanotte¹, Maria Giovanna Danieli¹

Affiliations

¹ Medical Clinic, United Hospitals and DISCLIMO, Polytechnic University of Marche, Ancona, Italy.
² Medicine Departement, Destra Secchia Hospital, Pieve di Coriano, ASST Mantova, Mantua, Italy.
³ Molecular and Applied Biology, Polytechnic University of Marche, Ancona, Italy.
⁴ Biostatistic and Bioinformatic Unit, Scientific Direction, IRCCS Regina Elena National Cancer Institute, Rome, Italy.

Abstract

Background: Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency and has a broad spectrum of clinical manifestations. Among non-infectious complications, an increased incidence of malignancies may have a special relevance for survival, but little is known about treatment efficacy on malignant complications.

Methods: This was a monocenter retrospective study on CVID patients, designed to provide preliminary data for the investigation of the possible link between therapeutic delay and tumor incidence.

Results: A total of 67 CVID subjects were included. The median diagnostic delay was 7.5 years (range: 0-63 years), and the median therapeutic delay was 8.5 years (range: 0-67 years). Malignancies were diagnosed in 18 (27%) patients. Eight out of 18 (44%) patients with a malignancy had lymphoma. Patients who developed a malignancy showed a longer therapeutic delay in comparison to patients with no malignancy, although no statistical significance was achieved (11 years vs 8 years, respectively, p = 0.424). We observed a lower frequency of malignancy in CVID patients with reduced therapeutic delay compared with patients with therapeutic delay ≥ 10 years. With a therapeutic delay of > 1 year, 74% had no tumor, and 25% had a tumor; with a therapeutic delay of > 10 years, 65% had no tumor and 35% had a malignancy. Among patients who had no malignancy, 64% had a therapeutic delay of < 10 years, and 36% had a therapeutic delay of ≥ 10 years. Among patients with malignancy, 47% of subjects had a therapeutic delay < 10 years, and 53% a therapeutic delay ≥ 10 years.

Conclusions: The observation of clinical characteristics of our patients with CVID may suggest that an early institution of IgG replacement therapy could be of benefit for the prevention of malignant complications.Name of the registry: Comitato Etico Regionale delle Marche. Trial registration number: 1505. Date of registration: 27/10/2016, Retrospectively registered URL of trial registry record: http://www.ospedaliriuniti.marche.it/portale/archivio13_cerm-ancona_0_446_1.html. The trial was not registered before the first participant was enrolled.

Keywords: Common variable immunodeficiency; Diagnostic delay; Ig replacement therapy; Therapeutic delay.