Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
3199025 | Journal of Allergy and Clinical Immunology | 2011 | 15 Pages |
Abstract
Primary immunodeficiencies (PIDs) are commonly characterized by an increased susceptibility to specific infections and, in certain instances, a higher than usual incidence of malignancies. Although improved diagnosis and early treatment of PIDs have reduced early morbidity and mortality from infection, the development of cancer remains a significant cause of premature death. The emergence of cancer in patients with PIDs often results from impairments in the immune response that lead to weakened surveillance against oncogenic viruses, premalignant or malignant cells, or both. Here we review the clinical and biologic features of several PIDs associated with enhanced susceptibility to viral infections and cancer, including X-linked lymphoproliferative disease; IL-2-inducible T-cell kinase deficiency; epidermodysplasia verruciformis; warts, hypogammaglobulinemia, infections, and myelokathexis syndrome; autosomal recessive hyper-IgE syndrome; X-linked agammaglobulinemia; and common variable immunodeficiency. It is of importance that we gain in-depth insights into the fundamental molecular nature of these unique PIDs to better understand the pathogenesis of virus-associated malignancies and to develop innovative therapeutic strategies.
Keywords
stromal cell–derived factor 1Tyk2ITKiNKTSTAT3XLACVIDDOCK8Warts, hypogammaglobulinemia, infections, and myelokathexisWHIMHSCTHIESPLC-γIVIgXLPGPCRSLAM-associated proteinHLHSDF-1SH2D1APIDDDRX-linked lymphoproliferative syndromeAR-HIESnatural killerFIMG protein–coupled receptorSLAMepidermodysplasia verruciformisX-linked agammaglobulinemiaIntravenous immunoglobulinMalignanciesX-linked lymphoproliferative diseasetyrosine kinase 2hyper-IgE syndromeSAPViral infectioninvariant natural killer Tphospholipase C-γHodgkin lymphomaHemophagocytic lymphohistiocytosissignal transducer and activator of transcription 3Dedicator of cytokinesis 8signaling lymphocytic activation moleculeCVID, common variable immunodeficiencyDNA damage responseHuman papilloma virusHPVHematopoietic stem cell transplantationImmunodeficiencyprimary immunodeficiency
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Authors
Nima MD, PhD, Mona MD, Asghar MD, PhD, Kim E. MD,