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Published on: November 26, 2018
A Concise Review of Autoimmune Cytopenias in Chronic Lymphocytic Leukemia
Mazie Tsang1, Sameer A Parikh2
1Division of Hematology, Department of Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Insights
Chronic lymphocytic leukemia (CLL) often causes autoimmune complications. Accurate diagnosis of these cytopenias is crucial for effective treatment and improved patient outcomes.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Chronic lymphocytic leukemia (CLL) frequently presents with autoimmune complications, including autoimmune hemolytic anemia and immune thrombocytopenia.
- Accurate differentiation between autoimmune cytopenias and those caused by bone marrow infiltration is critical for prognosis and therapeutic decisions in CLL patients.
Purpose of the Study:
- To review the pathogenesis and management of autoimmune complications in chronic lymphocytic leukemia.
- To highlight the importance of accurate diagnosis for guiding therapy in CLL-associated cytopenias.
Main Methods:
- Literature review of pathogenesis and treatment strategies for autoimmune cytopenias in CLL.
- Analysis of traditional and emerging therapeutic approaches, including corticosteroids, anti-CD20 antibodies, and novel oral agents.
Main Results:
- The pathogenesis involves CLL cells presenting antigens to B cells, leading to autoantibody production and altered T cell responses.
- Conventional treatments include corticosteroids and anti-CD20 antibodies; treating the underlying CLL is effective for suboptimal responses.
- Novel oral therapies show promise but require further prospective data for routine use in autoimmune cytopenias of CLL.
Conclusions:
- Autoimmune cytopenias are significant complications of CLL requiring careful diagnosis and management.
- While established therapies exist, the role of novel oral agents in managing these complications needs further investigation with long-term data.
Abstract:
Chronic lymphocytic leukemia (CLL) is frequently associated with autoimmune complications such as autoimmune hemolytic anemia, immune thrombocytopenia, pure red cell aplasia, and autoimmune granulocytopenia. It is critical to diagnose cytopenias from these secondary complications of CLL accurately, since prognosis and therapy are substantially different from patients who have cytopenias due to extensive bone marrow infiltration by CLL. The pathogenesis of autoimmune cytopenias in CLL is complex; and it involves antigen presentation by CLL cells to polyclonal B cells resulting in production of autoantibody, and alteration of the T cell milieu tilting the balance in favor of an autoimmune response. Traditional therapy of autoimmune complications in CLL consists of immunosuppression with corticosteroids and/or anti-CD20 monoclonal antibodies. In patients who have a suboptimal response, treating the underlying CLL is generally effective in ameliorating secondary cytopenias. Although novel oral therapies such as ibrutinib, idelalisib, and venetoclax have been shown to be extremely effective in the management of CLL, prospective data from larger numbers of patients with longer follow-up are needed prior to recommending their routine use in the management of autoimmune cytopenias in CLL.
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