Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review

Chronic lymphocytic leukemia (CLL) patients have a greater predisposition to develop autoimmune complications. The most common of them is autoimmune hemolytic anemia (AIHA) with a frequency of 7–10% of cases. Pathogenesis is multifactorial involving humoral, cellular, and innate immunity. CLL B-cell...

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Autores principales:	Francesco Autore, Raffaella Pasquale, Idanna Innocenti, Alberto Fresa, Federica Sora’, Luca Laurenti
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	CLL AIHA steroids rituximab targeted drugs Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/ed72441656cd464e93b1703b104c4cc7
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spelling	oai:doaj.org-article:ed72441656cd464e93b1703b104c4cc72021-11-25T17:04:14ZAutoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review10.3390/cancers132258042072-6694https://doaj.org/article/ed72441656cd464e93b1703b104c4cc72021-11-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/22/5804https://doaj.org/toc/2072-6694Chronic lymphocytic leukemia (CLL) patients have a greater predisposition to develop autoimmune complications. The most common of them is autoimmune hemolytic anemia (AIHA) with a frequency of 7–10% of cases. Pathogenesis is multifactorial involving humoral, cellular, and innate immunity. CLL B-cells have damaged apoptosis, produce less immunoglobulins, and could be responsible for antigen presentation and releasing inflammatory cytokines. CLL B-cells can act similar to antigen-presenting cells activating self-reactive T helper cells and may induce T-cell subsets imbalance, favoring autoreactive B-cells which produce anti-red blood cells autoantibodies. Treatment is individualized and it depends on the presence and severity of clinical symptoms, disease status, and comorbidities. Corticosteroids are the standardized first-line treatment; second-line treatment comprises rituximab. Patients not responding to corticosteroids and rituximab should be treated with CLL-specific drugs as per current guidelines according to age and comorbidities. New targeted drugs (BTK inhibitors and anti BCL2) are recently used after or together with steroids to manage AIHA. In the case of cold agglutinin disease, rituximab is preferred, because steroids are ineffective. Management must combine supportive therapies, including vitamins; antibiotics and heparin prophylaxis are indicated in order to minimize infectious and thrombotic risk.Francesco AutoreRaffaella PasqualeIdanna InnocentiAlberto FresaFederica Sora’Luca LaurentiMDPI AGarticleCLLAIHAsteroidsrituximabtargeted drugsNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5804, p 5804 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	CLL AIHA steroids rituximab targeted drugs Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	CLL AIHA steroids rituximab targeted drugs Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Francesco Autore Raffaella Pasquale Idanna Innocenti Alberto Fresa Federica Sora’ Luca Laurenti Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review
description	Chronic lymphocytic leukemia (CLL) patients have a greater predisposition to develop autoimmune complications. The most common of them is autoimmune hemolytic anemia (AIHA) with a frequency of 7–10% of cases. Pathogenesis is multifactorial involving humoral, cellular, and innate immunity. CLL B-cells have damaged apoptosis, produce less immunoglobulins, and could be responsible for antigen presentation and releasing inflammatory cytokines. CLL B-cells can act similar to antigen-presenting cells activating self-reactive T helper cells and may induce T-cell subsets imbalance, favoring autoreactive B-cells which produce anti-red blood cells autoantibodies. Treatment is individualized and it depends on the presence and severity of clinical symptoms, disease status, and comorbidities. Corticosteroids are the standardized first-line treatment; second-line treatment comprises rituximab. Patients not responding to corticosteroids and rituximab should be treated with CLL-specific drugs as per current guidelines according to age and comorbidities. New targeted drugs (BTK inhibitors and anti BCL2) are recently used after or together with steroids to manage AIHA. In the case of cold agglutinin disease, rituximab is preferred, because steroids are ineffective. Management must combine supportive therapies, including vitamins; antibiotics and heparin prophylaxis are indicated in order to minimize infectious and thrombotic risk.
format	article
author	Francesco Autore Raffaella Pasquale Idanna Innocenti Alberto Fresa Federica Sora’ Luca Laurenti
author_facet	Francesco Autore Raffaella Pasquale Idanna Innocenti Alberto Fresa Federica Sora’ Luca Laurenti
author_sort	Francesco Autore
title	Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review
title_short	Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review
title_full	Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review
title_fullStr	Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review
title_full_unstemmed	Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review
title_sort	autoimmune hemolytic anemia in chronic lymphocytic leukemia: a comprehensive review
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/ed72441656cd464e93b1703b104c4cc7
work_keys_str_mv	AT francescoautore autoimmunehemolyticanemiainchroniclymphocyticleukemiaacomprehensivereview AT raffaellapasquale autoimmunehemolyticanemiainchroniclymphocyticleukemiaacomprehensivereview AT idannainnocenti autoimmunehemolyticanemiainchroniclymphocyticleukemiaacomprehensivereview AT albertofresa autoimmunehemolyticanemiainchroniclymphocyticleukemiaacomprehensivereview AT federicasora autoimmunehemolyticanemiainchroniclymphocyticleukemiaacomprehensivereview AT lucalaurenti autoimmunehemolyticanemiainchroniclymphocyticleukemiaacomprehensivereview
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Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review

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