Indolfi Cristiana, Dinardo Giulio, Grella Carolina, Klain Angela, Perrotta Alessandra, Mondillo Gianluca, Marrapodi Maria Maddalena, Decimo Fabio, Miraglia Del Giudice Michele
Department of Woman, Child and of General and Specialized Surgery, University of Campania Luigi Vanvitelli, 80138 Naples, Italy.
J Clin Med. 2024 May 31;13(11):3263. doi: 10.3390/jcm13113263.
Asthma, a prevalent chronic respiratory condition characterized by inflammation of the airways and bronchoconstriction, has demonstrated a potential association with hemoglobinopathies such as thalassemia and sickle cell disease (SCD). Numerous studies have highlighted a higher prevalence of asthma among thalassemia patients compared to the general population, with rates ranging around 30%. Similarly, asthma frequently coexists with SCD, affecting approximately 20-48% of patients. Children with SCD often experience heightened lower airway obstruction and airway hyper-reactivity. Notably, the presence of asthma in SCD exacerbates respiratory symptoms and increases the risk of severe complications like acute chest syndrome, stroke, vaso-occlusive episodes, and early mortality. Several studies have noted a decrease in various cytokines such as IFN-γ and IL-10, along with higher levels of both IL-6 and IL-8, suggesting an overactivation of pro-inflammatory mechanisms in patients with hemoglobinopathies, which could trigger inflammatory conditions such as asthma. The exact mechanisms driving this association are better elucidated but may involve factors such as chronic inflammation, oxidative stress, and immune dysregulation associated with thalassemia-related complications like chronic hemolytic anemia and iron overload. This review aims to comprehensively analyze the relationship between asthma and hemoglobinopathies, with a focus on thalassemia and SCD. It emphasizes the importance of interdisciplinary collaboration among pulmonologists, hematologists, and other healthcare professionals to effectively manage this complex interplay. Understanding this link is crucial for improving care and outcomes in affected individuals.
哮喘是一种常见的慢性呼吸道疾病,其特征为气道炎症和支气管收缩,已显示出与地中海贫血和镰状细胞病(SCD)等血红蛋白病存在潜在关联。大量研究表明,与普通人群相比,地中海贫血患者中哮喘的患病率更高,约为30%。同样,哮喘常与SCD并存,影响约20 - 48%的患者。患有SCD的儿童常出现更严重的下气道阻塞和气道高反应性。值得注意的是,SCD患者中哮喘的存在会加重呼吸道症状,并增加急性胸部综合征、中风、血管闭塞性发作和早期死亡等严重并发症的风险。多项研究指出各种细胞因子如IFN - γ和IL - 10减少,同时IL - 6和IL - 8水平升高,这表明血红蛋白病患者的促炎机制过度激活,可能引发如哮喘等炎症性疾病。导致这种关联的确切机制尚待进一步阐明,但可能涉及慢性炎症、氧化应激以及与地中海贫血相关并发症如慢性溶血性贫血和铁过载相关的免疫失调等因素。本综述旨在全面分析哮喘与血红蛋白病之间的关系,重点关注地中海贫血和SCD。它强调了肺科医生、血液科医生和其他医疗专业人员之间跨学科合作对于有效管理这种复杂相互作用的重要性。了解这种联系对于改善受影响个体的护理和预后至关重要。