title: Impaired phagocyte antibacterial effector functions in beta-thalassemia: a likely factor in the increased susceptibility to bacterial infections.
abstract: Bacterial diseases are serious complications of beta-thalassemia syndromes but the mechanisms underlying the increased susceptibility to these infections are not fully understood. Factors which are likely to be involved are anemia, splenectomy, iron-overload and alterations in innate/adaptive immune responses. There is substantial evidence that a defect in innate effector functions of phagocytes (neutrophils, monocytes/macrophages) plays an important role in the weakened resistance to pathogenic bacteria and is at least in part due to iron overload. There is substantial evidence of an iron-related defect in bacterial phagocytosis by neutrophils. Moreover, reduced chemotaxis by these phagocytes has been repeatedly demonstrated. Similarly, an impairment of monocyte bacterial phagocytosis and generation of anti-bacterial compounds have recently been delineated but any relation to iron overload needs to be established. Additional mechanisms of defective innate immune responses such as altered expression of pathogen recognising receptors and function seem possible and have to be explored. Further insight into innate phagocyte effector functions in beta-thalassemia is essential for understanding the increased susceptibility to bacterial infections and their management.
authors.affiliation: Departmentof Haematology, Imperial College Faculty of Medicine, Hammersmith Hospital, London, UK. e.weiner@ic.ac.uk
authors.forename: Edith
authors.initials: E
authors.lastname: Wiener
mesh_terms: D000740:Anemia / Q000209:etiology; D000942:Antigens, Bacterial / Q000276:immunology; D001424:Bacterial Infections / Q000453:epidemiology / Q000209:etiology*; D002634:Chemotaxis, Leukocyte; D004198:Disease Susceptibility; D006801:Humans; D007113:Immunity, Innate; D007153:Immunologic Deficiency Syndromes / Q000209:etiology* / Q000276:immunology; D019190:Iron Overload / Q000209:etiology / Q000276:immunology*; D008264:Macrophages / Q000473:pathology; D009000:Monocytes / Q000473:pathology; D009504:Neutrophils / Q000473:pathology; D010586:Phagocytes / Q000473:pathology*; D010587:Phagocytosis; D016897:Respiratory Burst; D013156:Splenectomy / Q000009:adverse effects; D017086:beta-Thalassemia / Q000150:complications / Q000276:immunology*
chemical_list: D000942:Antigens, Bacterial