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The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia

Autoimmune cytopenias (AICs) have been reported as a common complication in chronic lymphocytic leukemia (CLL) with autoimmune hemolytic anemia (AIHA), accounting for most cases. According to iwCLL guidelines, AICs poorly responsive to corticosteroids are considered indication for CLL-directed treat...

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Autores principales: Noto, Alessandro, Cassin, Ramona, Mattiello, Veronica, Reda, Gianluigi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8151128/
https://www.ncbi.nlm.nih.gov/pubmed/34065833
http://dx.doi.org/10.3390/jcm10102064
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author Noto, Alessandro
Cassin, Ramona
Mattiello, Veronica
Reda, Gianluigi
author_facet Noto, Alessandro
Cassin, Ramona
Mattiello, Veronica
Reda, Gianluigi
author_sort Noto, Alessandro
collection PubMed
description Autoimmune cytopenias (AICs) have been reported as a common complication in chronic lymphocytic leukemia (CLL) with autoimmune hemolytic anemia (AIHA), accounting for most cases. According to iwCLL guidelines, AICs poorly responsive to corticosteroids are considered indication for CLL-directed treatment. Chemo-immunotherapy has classically been employed, with variable results, and little data are available on novel agents, the current backbone of CLL therapy. The use of idelalisib in the setting of AICs is controversial and recent recommendations suggest avoiding idelalisib in this setting. Ibrutinib, through ITK-driven Th1 polarization of cell-mediated immune response, is known to produce an immunological rebalancing in CLL, which stands as a fascinating rationale for its use to treat autoimmunity. Although treatment-emergent AIHA has rarely been reported, ibrutinib has shown rapid and durable responses when used to treat AIHA arising in CLL. There is poor evidence regarding the role of BCL-2 inhibitors in CLL-associated AICs and the use of venetoclax in such cases is debated. Furthermore, their frequent use in combination with anti-CD20 agents might represent a confounding factor in evaluating their efficacy. In conclusions, because of their ability to mitigate an immunological dysregulation that is (at least partly) responsible for autoimmunity in CLL, to date BTK-inhibitors stand out as the most suitable choice when treatment of autoimmune cytopenias is required.
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spelling pubmed-81511282021-05-27 The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia Noto, Alessandro Cassin, Ramona Mattiello, Veronica Reda, Gianluigi J Clin Med Review Autoimmune cytopenias (AICs) have been reported as a common complication in chronic lymphocytic leukemia (CLL) with autoimmune hemolytic anemia (AIHA), accounting for most cases. According to iwCLL guidelines, AICs poorly responsive to corticosteroids are considered indication for CLL-directed treatment. Chemo-immunotherapy has classically been employed, with variable results, and little data are available on novel agents, the current backbone of CLL therapy. The use of idelalisib in the setting of AICs is controversial and recent recommendations suggest avoiding idelalisib in this setting. Ibrutinib, through ITK-driven Th1 polarization of cell-mediated immune response, is known to produce an immunological rebalancing in CLL, which stands as a fascinating rationale for its use to treat autoimmunity. Although treatment-emergent AIHA has rarely been reported, ibrutinib has shown rapid and durable responses when used to treat AIHA arising in CLL. There is poor evidence regarding the role of BCL-2 inhibitors in CLL-associated AICs and the use of venetoclax in such cases is debated. Furthermore, their frequent use in combination with anti-CD20 agents might represent a confounding factor in evaluating their efficacy. In conclusions, because of their ability to mitigate an immunological dysregulation that is (at least partly) responsible for autoimmunity in CLL, to date BTK-inhibitors stand out as the most suitable choice when treatment of autoimmune cytopenias is required. MDPI 2021-05-12 /pmc/articles/PMC8151128/ /pubmed/34065833 http://dx.doi.org/10.3390/jcm10102064 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Noto, Alessandro
Cassin, Ramona
Mattiello, Veronica
Reda, Gianluigi
The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia
title The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia
title_full The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia
title_fullStr The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia
title_full_unstemmed The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia
title_short The Role of Novel Agents in Treating CLL-Associated Autoimmune Hemolytic Anemia
title_sort role of novel agents in treating cll-associated autoimmune hemolytic anemia
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8151128/
https://www.ncbi.nlm.nih.gov/pubmed/34065833
http://dx.doi.org/10.3390/jcm10102064
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