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Autoimmune complications of chronic lymphocytic leukemia
1Department of Immunohaematology, University of Southampton, Southampton General Hospital, Royal Bournemouth Hospital, Kings College Hospital, London, UK. terjoha@aol.com
Insights
Autoimmune complications, like autoimmune hemolytic anemia, frequently occur in chronic lymphocytic leukemia (CLL). Treatment strategies are being explored, with promising results from cyclosporine and rituximab.
Area of Science:
- Hematology
- Immunology
Background:
- Autoimmune complications affect up to 25% of patients with chronic lymphocytic leukemia (CLL).
- Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are the most common manifestations.
- Nonhematologic autoimmune conditions are rare in CLL.
Purpose of the Study:
- To explore the pathogenesis and management of autoimmune complications in CLL.
- To investigate potential triggers and novel treatment options for CLL-associated autoimmunity.
Main Methods:
- Review of existing literature on CLL-associated autoimmunity.
- Analysis of proposed pathogenetic mechanisms involving CLL cells as antigen-presenting cells (APCs) and regulatory T-cell dysfunction.
- Evaluation of treatment outcomes for conventional protocols, cyclosporine, and rituximab.
Main Results:
- Autoimmunity in CLL is common, primarily affecting blood cells.
- Pathogenesis may involve CLL cells presenting antigens and impaired regulatory T-cell function.
- Autoimmune episodes can be severe, potentially triggered by treatments like purine analogs.
- Conventional treatments may have limited efficacy, with cyclosporine and rituximab showing promise.
Conclusions:
- Autoimmunity is a significant clinical issue in CLL, with complex pathogenesis.
- Effective management requires understanding potential triggers and exploring advanced therapies.
- Cyclosporine and rituximab represent promising therapeutic options for refractory cases.
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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