Lymphomas of mucosal‐associated lymphoid tissue in common variable immunodeficiency

Abstract

Common variable immunode®ciency (CVID) is a primary immunode®ciency disease characterized by low serum immunoglobulins IgG, IgA, and usually IgM. The central immune de®ciency is impaired secretion of immunoglobulins and lack of antibody production; however, T cell dysfunction and a variety of in¯ammatory complications suggest global immune dysregulation. A number of reports have documented the association of primary immunode®ciency diseases with the development of non-Hodgkin's lymphoma (NHL). In CVID, the risk has been estimated to lie between 1.4% and 7%. As for NHL arising in other immunode®ciency states, the lymphomas in CVID are extranodal and are usually B cell in type. Of 22 B cell lymphomas that have appeared over a period of 25 years in a cohort of subjects with CVID, ®ve lymphomas, appearing in more recently studied subjects, that arose in mucosal sites would be classi®ed as mucosaassociated lymphoid tissue (MALT) lymphomas. MALT lymphomas are low-grade B cell lymphomas that result from a proliferation of neoplastic marginal-zone related cells of lymphoid tissue and tend to occur in organs that have acquired lymphoid tissue due to long-term infectious or autoimmune stimulation. Lymphomas of this kind have not been described in patients with congenital immunode®ciency, although chronic mucosal antigen stimulation is an integral part of these immune de®ciency states. Am.

Are antibody deficiency disorders associated with a narrower range of cancers than other forms of immunodeficiency?

“…25, 26 We found an 8-fold increased risk for NHL for all forms of PID combined; 95% CI indicates 95% confidence interval; E, expected number of cases; NHL, non-Hodgkin lymphoma; O, observed number of cases; PID, primary immune deficiency; and SIR, standardized incidence ratio. *C00-C96, excluding C44.…”

Decrease in phenotypic regulatory T cells in subsets of patients with common variable immunodeficiency

“…It may manifest at any age; however, two-thirds of patients are adults at the time of diagnosis [1,2]. In addition, CVID patients may develop splenomegaly (~30%), a variety of autoimmune diseases (~20%), chronic inflammatory disorders such as granulomatous disease (~10%) and less frequently malignancies (non-Hodgkin's lymphoma or gastric cancer) [1,[3][4][5].…”

Serum bactericidal activity against Helicobacter pylori in patients with hypogammaglobulinaemia

“…In particular, there is an elevated risk in CVID patients for gastric cancer and lymphomas, such as extra-nodal marginal zone lymphomas [described formerly as mucosa-associated lymphoid tissue (MALT) lymphomas] [3,[12][13][14][15]. In XLA the risk for colorectal cancer is elevated [16].…”