Spectrum of lymphoproliferation in common variable immunodeficiency disorders - review of the literature

Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency disorders, characterized by a deficit of antibody production although other aspects of the immune system are frequently altered too. These disorders usually present with recurrent and/or severe infections, affecting mainly the respiratory and gastrointestinal tracts. A considerable proportion of these patients also present non infective complications, including autoimmunity and different manifestations of chronic lymphoid proliferation. Such lymphoid proliferation may affect different organs simultaneously, including spleen, lymph nodes, lung and gut. CVIDs are associated with a higher risk of lymphoma and the pathophysiology is poorly understood. The current therapeutic strategies and management of CVID patients result in longer survival, mainly due to the decrease of infectious complications and has increased awareness of non -infective complications. In this paper, the authors review the literature, in regard to the different presentations of lymphoproliferation, from benign to malignant, and discuss the possible mechanisms underlying this form of disease expression found in subgroups of patients with CVIDs