title: Down's syndrome, interferon sensitivity and the development of leukaemia.
abstract: I suggest that the occurrence of transient leukemoid reactions, myelofibrosis and leukaemia in Down's syndrome may all result from the greatly increased sensitivity of Down's syndrome cells to interferon with consequent aberrant antigen expression leading to autoimmune disease. In the bone marrow this may manifest itself as premature egress of blast cells (TLR), inflammation and imperfect repair (myelofibrosis), and the binding of autoantibodies to cell nuclei resulting in malignant transformation leading to leukaemia. Therapy consistent with this hypothesis would be directed at blocking the effects of gamma interferon, and such therapy might also be useful in certain forms of leukaemia associated with viral infection.
authors.affiliation: Wellcome Institute for the History of Medicine, London, U.K.
authors.forename: L
authors.initials: L
authors.lastname: Zihni
mesh_terms: D001323:Autoantibodies / Q000378:metabolism; D015551:Autoimmunity; D004314:Down Syndrome / Q000150:complications* / Q000235:genetics / Q000276:immunology; D006680:HLA Antigens / Q000378:metabolism; D006801:Humans; D007371:Interferon-gamma / Q000502:physiology*; D007938:Leukemia / Q000209:etiology*; D007955:Leukemoid Reaction / Q000209:etiology; D055728:Primary Myelofibrosis / Q000209:etiology
chemical_list: D001323:Autoantibodies; D006680:HLA Antigens; D007371:Interferon-gamma