Primary immunodeficiency diseases (PID) are caused by inborn errors of the immune system. PID patients with inadequate tumor immunity are at an elevated risk of developing malignancies, such as lymphoma, leukemia, and gastrointestinal cancer.
Retrospective study of cases of primary immune deficiency (PID), who developed cancer over a period of 37 years (1987-2024) in the Pediatric Immuno-Haematology Department, CNGMO, Tunis.
We collected 11 cases of cancer among 710 patients with PID (1.5%). Patients were distributed as follows: 2/112 cases of ataxia telangiectasia syndrome, 5/49 cases of variable common immune deficiency, one case of LOCID, 1/40 cases of Burton’s agammaglobulinemia, 1/30 cases of hyper IgM syndrome, 1/7 cases of severe CD4 lymphopenia, and 1/6 cases of Wiskott-Aldrich syndrome. The mean age at diagnosis was 5.5 years (2-16 years). The cancers found were as follows: 5 cases of lymphoma, including 2 cases of Hodgkin’s lymphoma and 4 cases of malignant non-Hodgkin’s lymphoma, 1 case of acute lymphoblastic leukemia, 1 case of Kaposi’s sarcoma, 1 case of genital neoplasia, 1 case of bronchoalveolar carcinoma, 1 case of gastric adenocarcinoma, and 1 case of cancer in the head of the pancreas. Death occurred in 8 cases.
After infection, malignancy is the most prevalent cause of death in both children and adults with PIDs.
