Lymphoma complicating common variable immunodeficiency with granulomatous disease: report of two cases

Abstract

Common variable immunodeficiency (CVID) is a primary defect that is characterized by impaired antibody production. CVID patients may develop recurrent infections, autoimmune disorders and/or systemic granulomatosis. It is well documented that CVID patients are at risk to develop malignant lymphomas. However, to the best of our knowledge, lymphoma complicating the course of CVID associated with systemic granulomatosis has never been reported. We describe two CVID patients with systemic granulomatosis who developed B-cell lymphomas, one related to Epstein Barr virus infection, 5 and 12 yr after CVID had been diagnosed.

Granulomatous disease in common variable immunodeficiency

“…Mycofenolate mofetil (CellCept) may be an alternative option especially in patients diagnosed with granulomatous and lymphocytic infiltrations as it may exert a preferentially suppressive effect on activated T cells, inhibits expression of adhesion molecules and inhibit nitric oxide (NO) synthesis [65]. However, immunosuppressive therapy may expose patients to a risk of opportunistic infections and possibly malignancy, a risk in CVID [1; 36; 66]. In pulmonary granulomatous lymphocytic lung disease, lung transplantation has been performed (as for case #6 here) however granulomas can recur in the transplanted lung [9].…”

Prospective open‐label study of pharmacokinetics, efficacy and safety of a new 10% liquid intravenous immunoglobulin in patients with hypo‐ or agammaglobulinemia

“…Furthermore, CVID is widely recognized to be associated with a predisposition to lymphoma [39]. Specifically, the development of non‐Hodgkin's lymphomas such as B‐cell lymphomas is known to occur with an increased frequency in CVID patients [40–43].…”

Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions

“…These lymphomas almost always extra‐nodal, and have a tendency to locate in mucosal regions (Altschul & Cunningham‐Rundles, 2002). The development of lymphoma has been variously associated with the presence of lymphoid hyperplasia, granulomatous disease (Le Guern et al , 2003) and the retention of serum IgM (Chapel et al , 2008). As BCL6 mutations have commonly been found in CVID lymphomas, a germinal centre origin has been suggested (Ariatti et al , 2000).…”