Acute chest syndrome in sickle cell anemia: Diagnostic challenges and therapeutic strategies
Keywords:
Sickle cell anemia, Acute Thoracic Syndrome, Hemolysis, Blood disease.Abstract
Sickle cell disease is a genetic blood disorder caused by a mutation in the amino acid glutamic acid, which is replaced by valine in the beta chain of the hemoglobin molecule. In this context, acute chest syndrome is a serious disease with a high morbidity and mortality rate, accounting for approximately 25% of deaths in patients with sickle cell anemia. The aim of this study is to describe the main aspects of Acute Thoracic Syndrome (ATS) in patients with sickle cell anemia. This is a review study of a scoping review in which data collection was carried out in the research portal of the Virtual Health Library (VHL), the databases used were: Online System for Search and Analysis of Medical Literature (MEDLINE) and Latin American and Caribbean Literature in Health Sciences (LILACS) where the following descriptors were used for the search: "Sickle Cell Anemia" AND "Acute Thoracic Syndrome". Using the descriptors, 451 articles were found. The inclusion criteria were: articles published between 2019 and 2024, available in full and free of charge; and the exclusion criteria were repeated articles, paid articles and methods with an emphasis on literature reviews. The inclusion criteria were: articles published between 2019 and 2024, available in full and free of charge; and the exclusion criteria were repeated, paid-for articles and methods with an emphasis on literature reviews. The criteria established 20 studies for the final sample. Acute chest syndrome is characterized by fever and/or respiratory symptoms with pulmonary infiltrates, which can lead to sepsis and cause stroke in patients with sickle cell anemia. In addition, increased hemolysis during sickle cell crises leads to a faster depletion of nitric oxide, which is a potent vasodilator of metabolism and a factor in cardiopulmonary hemodynamics. In this context, ultrasound is recognized as the gold standard for diagnosing the syndrome, due to the absence of radiation, with high accuracy, sensitivity and specificity. Changes in lung function in acute chest syndrome cause variations in the rates of inflammatory markers that can help to recognize the condition and treat it more effectively, such as phosphatidylserine, a specific type of phospholipid that is essential in cell membranes, serum ferritin, which increases due to an attempt to compensate for the increase in hemolysis, and IL-6, which reflects the recruitment of monocytes and other innate immune cells in the lungs. In view of the intense hemolysis, red blood cell transfusions can be seen as a protective factor for acute chest syndrome and can even be considered the definitive therapy for the syndrome, as they improve oxygen supply to the tissues, increase the overall hemoglobin level and decrease the fraction of sickle-shaped red blood cells. This allows the patient to get relief from respiratory symptoms more quickly. Therefore, acute chest syndrome in patients with sickle cell anemia requires appropriate interventions and should be treated like rare diseases in health facilities, whether the primary health care unit or the emergency room, in order to guarantee appropriate treatment without delay. Thus, the management that should be carried out is analgesia, hydration, antibiotic therapy, bronchodilators, ventilation, which can be invasive or non-invasive, oxygen and blood transfusion.
