A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)

Discussion In this section, we discuss the mechanisms responsible for lymphomagenesis in the various inborn errors of immunity and provide an overview of the treatment. Defects in Immune Responses That Predispose to Lymphomagenesis in PIDDs The complex immune mechanisms and their interplay that predisposes to neoplastic transformation of B or T cells and development of lymphomas in PIDD patients has not been fully elucidated. However, it is expected that the etiology in most cases is multifactorial and related to a dynamic regulation of immune response and environmental triggers (Figure 3). An underlying intrinsic susceptibility to DNA damage in some of these PIDDs, may provide a substrate for uncontrolled cell growth, impaired apoptosis of damaged cells, premature cellular senescence, all of which may be compounded by increased antigenic stimulation of adaptive lymphocytes by viruses, such as EBV (112). This uncontrolled stimulation in the setting of altered immune checkpoints and immune dysregulation characterized by T cell exhaustion, defective anti-tumor immune surveillance and overproduction of inflammatory cytokines provides a fertile setting for neoplastic transformation and lymphoproliferation (Figure 3). FIGURE 3 Figure 3. Illustration of potential interplay of mechanisms implicated in pathogenesis of malignancies in PIDs. Defective DNA Repair DNA integrity is constantly challenged at various levels as part of physiological processes. In lymphocyte...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research

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Source: Expert Review of Anti-Infective Therapy - Category: Infectious Diseases Tags: Expert Rev Anti Infect Ther Source Type: research
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Source: Expert Review of Hematology - Category: Hematology Tags: Expert Rev Hematol Source Type: research
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Source: New Zealand Medical Journal - Category: General Medicine Tags: N Z Med J Source Type: research
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Source: New Zealand Medical Journal - Category: General Medicine Tags: N Z Med J Source Type: research
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