Sickle Cell Disease and Its Respiratory Complications

Abstract

Sickle cell disease (SCD) is a hematological disorder that is inherited in an autosomal recessive (AR) fashion. It is caused by mutations in the genes encoding for the globin apoprotein of hemoglobin (Hb), leading to diminished oxygen-carrying ability. Its pathophysiologic mechanism affects multiple organ systems, making it crucial to understand the complications of SCD and find the best ways to prevent and treat them. Some important ways that SCD manifests in the respiratory system are acute chest syndrome (ACS), pulmonary hypertension (PH), asthma, and venous thromboembolism (VTE). This article summarizes their salient features, including pathogenesis related to the adverse outcomes, screening practices, and management guidelines, with the intent to provide greater insight into forming better practices that increase the quality of life in SCD patients.

Exploring the Interplay between Asthma and Hemoglobinopathies: A Comprehensive Review

“…It was highlighted that the symptoms of asthma in SCD are caused by bronchial hyperresponsiveness, episodic bronchoconstriction, and acute-on-chronic inflammation. These exacerbations lead to mucous plugging, ventilation–perfusion mismatch, and hypoxemia, which can trigger the sickling of red blood cells and predispose individuals to ACS [ 34 , 44 , 45 ]. The interplay of inflammatory pathways in conditions such as SCD and thalassemia underscores the complexity of respiratory health in affected individuals.…”

New Clues to Cardiovascular Disease: Erythrocyte Lifespan

“…Red blood cells play a role in the formation of thrombi, which can lead to heart attacks. Patients with Sickle Cell Anemia (SCA) are at a significantly higher risk of pulmonary embolism than healthy individuals [54][55][56][57][58]. These emboli form within the lungs rather than from lower limb deep veins [55,56].…”

“…Patients with Sickle Cell Anemia (SCA) are at a significantly higher risk of pulmonary embolism than healthy individuals [54][55][56][57][58]. These emboli form within the lungs rather than from lower limb deep veins [55,56]. Patients with hereditary spherocytosis (HS) also have an increased risk of arterial thrombosis after splenectomy [59].…”

Incidência e manejo das urgências clínicas de pacientes portadores de anemia falciforme

“…A Doença Falciforme (DF) é o termo que define um grupo de doenças hereditárias, incluindo Anemia Falciforme (AF), hemoglobinopatia SC (HbSC) e HbS β-talassemia, as quais ocorrem devido a uma mutação pontual na cadeia β-globina da hemoglobina (Hb) (KATO et al, 2018). Nesse cenário, na AF ocorre a substituição do ácido glutâmico pela valina na posição 6 da cadeia de β-hemoglobina, criando uma molécula de Hb com funcionalidade anômala, chamada hemoglobina S (KHAN et al, 2022). Essa variação afeta a estrutura das hemácias, fazendo-as tomar a forma de "foice" ou "meia-lua", além de limitar seu tempo de vida (SILVA et al, 2021).…”