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Autoimmune complications of chronic lymphocytic leukemia

Autoimmune complications of chronic lymphocytic leukemia
Autoimmune complications of chronic lymphocytic leukemia
Autoimmune complications are common in chronic lymphocytic leukemia (CLL), occurring in up to a quarter of all patients during the course of the illness. By far the most common manifestation is autoimmune hemolytic anemia (AIHA), followed by immune thrombocytopenia (ITP). It is not true to say that autoimmunity is confined to the formed elements of the blood since conditions such as paraneoplastic pemphigus and acquired angioedema do occur in CLL, but nonhematologic autoimmunity is very rare indeed. The pathogenesis of autoimmunity in CLL is unknown. It may be related to the ability of the CLL cells to act as antigen-presenting cells (APCs), and to process antigen (particularly the Rh protein) so as to reveal cryptic peptides that are seen as foreign by helper T cells. It is likely that a failure of regulatory T-cell function is also involved. Autoimmune episodes may be triggered by treatment, particularly with purine analogues. Such episodes are often severe and may be fatal. Treatment of CLL-associated autoimmunity follows conventional protocols, but non-response to primary treatments is not uncommon. Promising results have been obtained with cyclosporine and rituximab.
paraneoplastic pemphigus, idiopathic thrombocytopenic purpura, red-cell aplasia, vh4-21 gene segment, leukemia, monoclonal-antibody, natural autoantibodies, time, b-cells, t-cells
0093-7754
230-239
Hamblin, Terry J.
57389613-7900-48fd-b3e6-8ca8fbdceccb
Hamblin, Terry J.
57389613-7900-48fd-b3e6-8ca8fbdceccb

Hamblin, Terry J. (2006) Autoimmune complications of chronic lymphocytic leukemia. Seminars in Oncology, 33 (2), 230-239. (doi:10.1053/j.seminoncol.2006.01.011).

Record type: Article

Abstract

Autoimmune complications are common in chronic lymphocytic leukemia (CLL), occurring in up to a quarter of all patients during the course of the illness. By far the most common manifestation is autoimmune hemolytic anemia (AIHA), followed by immune thrombocytopenia (ITP). It is not true to say that autoimmunity is confined to the formed elements of the blood since conditions such as paraneoplastic pemphigus and acquired angioedema do occur in CLL, but nonhematologic autoimmunity is very rare indeed. The pathogenesis of autoimmunity in CLL is unknown. It may be related to the ability of the CLL cells to act as antigen-presenting cells (APCs), and to process antigen (particularly the Rh protein) so as to reveal cryptic peptides that are seen as foreign by helper T cells. It is likely that a failure of regulatory T-cell function is also involved. Autoimmune episodes may be triggered by treatment, particularly with purine analogues. Such episodes are often severe and may be fatal. Treatment of CLL-associated autoimmunity follows conventional protocols, but non-response to primary treatments is not uncommon. Promising results have been obtained with cyclosporine and rituximab.

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More information

Published date: April 2006
Keywords: paraneoplastic pemphigus, idiopathic thrombocytopenic purpura, red-cell aplasia, vh4-21 gene segment, leukemia, monoclonal-antibody, natural autoantibodies, time, b-cells, t-cells

Identifiers

Local EPrints ID: 62771
URI: http://eprints.soton.ac.uk/id/eprint/62771
ISSN: 0093-7754
PURE UUID: f6d273b9-89b0-43dc-9698-f0d53c7d7f98

Catalogue record

Date deposited: 11 Sep 2008
Last modified: 13 Mar 2019 20:27

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