From inborn errors of immunity to lymphoma: A hematologist’s point of view

After infections, malignancies, lymphomas especially, are the second most frequent cause of death in patients with inborn errors immunity. Factors predetermining appearance and aggressiveness include gene defects, defects immune surveillance regulation as well infections oncogenic viruses. Aggressive non-Hodgkin lymphomas, mostly diffuse large B-cell Bukit subtypes predominant deoxyribonucleic acid repair while Hodgkin lymphoma becomes equally present regulation. Marginal zone mucosa-associated lymphoid tissue appear to be antibody production, especially common variable deficiency. The prevalence Epstein-Barr virus may vary within entities, but there is no entity without at least a few cases co-infection. Standard treatment associated severe combined deficiencies, stem cell transplantation. Lymphomas immunity less clinical presentation, should treated immunochemotherapy monoclonal antibodies (Brentuximab, Rituximab) wherever feasible. There data about usefulness checkpoint inhibitors, bi-specific T-cells chimeric antigen receptor. Allogeneic transplantation represents major indication for relapse/refractory any error Potential benefit therapy Chimeric receptor Natural-killer cells immunity, remains seen future studies.