Incidence Of Malignancy In Patients With Common Variable Immune Deficiency And Their Family Members

It is well known that malignancies especially of lymphoreticular and gastrointestinal origin are more common in patients with common variable immune deficiency (CVID). Incidence of malignancy among relatives of patiens with CVID was reported in a limited number of studies. In this study incidence of malignancy among patients with CVID, IgA deficiency and their relatives were investigated. We evaluated 25 CVID and 68 IgA deficiency patients, and their 727 and 1802 respective relatives for the presence of malignancy. In addition, all the first degree relatives of patients with CVID were screened for the presence of IgA deficiency and autoantibodies in terms of positive anti nuclear antibody (ANA) and anti double stranded DNA antibody (dsDNA). Incidence of malignancy in patients with CVID and their relatives were 12% and 1.9%, respectively. A history of malignancy was positive in none of the patients with IgA deficiency but in 2.3% of their relatives. None of the patients with CVID had positive ANA or anti dsDNA. Our results confirm the notion that incidence of malignancy is increased in patients with CVID. However, relatives of neither CVID nor IgA deficiency patients , displayed any increase in the incidence of malignancy. Screening of patients with CVID for malignancy and their relatives for IgA deficiency might provide early diagnosis of these conditions.