We identified a heterozygous pathogenic variant of PI3Ks gene in a male patient with recurrent infections, COPD, lymphomas and GLILD (c.1573G > A; p.Glu525Lys) and a heterozygous pathogenic variant of the TNFRSF13B gene in one patient with non-malignant lymph node enlargement and recurrent infections. The gene discussed is TNFRSF13B; the disease is lymphoma.