Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease

Abstract Sickle cell disease (SCD) is a monogenic disease, resulting from a single‐point mutation, that presents a complex pathophysiology and high clinical heterogeneity. Inflammation stands as a prominent characteristic of SCD. Over the past few decades, the role of different cells and molecules i...

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Main Authors: Karina Tozatto‐Maio, Felipe A. Rós, Ricardo Weinlich, Vanderson Rocha
Format: Article
Language:English
Published: Wiley 2024-12-01
Series:HemaSphere
Online Access:https://doi.org/10.1002/hem3.70032
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author Karina Tozatto‐Maio
Felipe A. Rós
Ricardo Weinlich
Vanderson Rocha
author_facet Karina Tozatto‐Maio
Felipe A. Rós
Ricardo Weinlich
Vanderson Rocha
author_sort Karina Tozatto‐Maio
collection DOAJ
description Abstract Sickle cell disease (SCD) is a monogenic disease, resulting from a single‐point mutation, that presents a complex pathophysiology and high clinical heterogeneity. Inflammation stands as a prominent characteristic of SCD. Over the past few decades, the role of different cells and molecules in the regulation of the inflammatory process has been elucidated. In conjunction with the polymerization of hemoglobin S (HbS), intravascular hemolysis, which releases free heme, HbS, and hemoglobin‐related damage‐associated molecular patterns, initiates multiple inflammatory pathways that are not yet fully comprehended. These complex phenomena lead to a vicious cycle that perpetuates vaso‐occlusion, hemolysis, and inflammation. To date, few inflammatory biomarkers can predict disease complications; conversely, there is a plethora of therapies that reduce inflammation in SCD, although clinical outcomes vary widely. Importantly, whether the clinical heterogeneity and complications are related to the degree of inflammation is not known. This review aims to further our understanding of the roles of main immune cells, and other inflammatory factors, as potential prognostic biomarkers for predicting clinical outcomes or identifying novel treatments for SCD.
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issn 2572-9241
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spelling doaj-art-47a7f2a2046a4f838f90b7119aa747fb2025-01-07T12:35:28ZengWileyHemaSphere2572-92412024-12-01812n/an/a10.1002/hem3.70032Inflammatory pathways and anti‐inflammatory therapies in sickle cell diseaseKarina Tozatto‐Maio0Felipe A. Rós1Ricardo Weinlich2Vanderson Rocha3Centro de Ensino e Pesquisa Hospital Israelita Albert Einstein São Paulo BrazilDivisão de Hematologia, Hemoterapia e Terapia Celular Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo São Paulo BrazilCentro de Ensino e Pesquisa Hospital Israelita Albert Einstein São Paulo BrazilDivisão de Hematologia, Hemoterapia e Terapia Celular Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo São Paulo BrazilAbstract Sickle cell disease (SCD) is a monogenic disease, resulting from a single‐point mutation, that presents a complex pathophysiology and high clinical heterogeneity. Inflammation stands as a prominent characteristic of SCD. Over the past few decades, the role of different cells and molecules in the regulation of the inflammatory process has been elucidated. In conjunction with the polymerization of hemoglobin S (HbS), intravascular hemolysis, which releases free heme, HbS, and hemoglobin‐related damage‐associated molecular patterns, initiates multiple inflammatory pathways that are not yet fully comprehended. These complex phenomena lead to a vicious cycle that perpetuates vaso‐occlusion, hemolysis, and inflammation. To date, few inflammatory biomarkers can predict disease complications; conversely, there is a plethora of therapies that reduce inflammation in SCD, although clinical outcomes vary widely. Importantly, whether the clinical heterogeneity and complications are related to the degree of inflammation is not known. This review aims to further our understanding of the roles of main immune cells, and other inflammatory factors, as potential prognostic biomarkers for predicting clinical outcomes or identifying novel treatments for SCD.https://doi.org/10.1002/hem3.70032
spellingShingle Karina Tozatto‐Maio
Felipe A. Rós
Ricardo Weinlich
Vanderson Rocha
Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease
HemaSphere
title Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease
title_full Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease
title_fullStr Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease
title_full_unstemmed Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease
title_short Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease
title_sort inflammatory pathways and anti inflammatory therapies in sickle cell disease
url https://doi.org/10.1002/hem3.70032
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