Nocturnal hemoglobin desaturation is associated with reticulocytosis in adults with sickle cell disease and is independent of obstructive sleep apnea

Rotz, Seth J.; O’Riordan, Mary Ann; Kim, Ceonne; Langer, Nathan; Cruz, Carissa M.; Schilz, Robert; Lance, Colleen; Little, Jane A.

doi:10.1002/ajh.24432

Cited by 14 publications

(8 citation statements)

References 6 publications

(2 reference statements)

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“…Although this model is debated (Hebbel, 2011) and sometimes difficult to apply in African countries (Dubert et al, 2017), consistent data have been reported in European and US countries that partly support this pathophysiological scheme (Ballas, 1991;Nolan et al, 2005Nolan et al, , 2006Gladwin and Vichinsky, 2008;Gurkan et al, 2010;Maier-Redelsperger et al, 2010;Connes et al, 2013). Our results confirm the link already reported between oxygen hemoglobin saturation, hemolysis, and anemia (Homi et al, 1997;Quinn and Ahmad, 2005;Rotz et al, 2016) and suggest that nocturnal hypoxemia could play a key role in the development of complications from the hemolytic phenotype.…”

Section: Discussionsupporting

confidence: 90%

“…In contrast, a recent study performed in SCD children failed to find an association between OSA or low nocturnal oxyhemoglobin saturation (SpO2) and the frequency of vaso-occlusive like events (Willen et al, 2018). Low nocturnal oxyhemoglobin saturation has been associated with biomarkers of hemolysis and endothelial activation in SCD (Setty et al, 2003;Rotz et al, 2016), and enhanced hemolysis plays a central role in the pathophysiology of SCD (Nader et al, 2021). However, the associations between complications related to chronic hemolysis and low oxyhemoglobin saturation have not been investigated.…”

Section: Introductionmentioning

confidence: 95%

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Nocturnal Hypoxemia Rather Than Obstructive Sleep Apnea Is Associated With Decreased Red Blood Cell Deformability and Enhanced Hemolysis in Patients With Sickle Cell Disease

et al. 2021

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Background: Although obstructive sleep apnea (OSA) could act as a modulator of clinical severity in sickle cell disease (SCD), few studies focused on the associations between the two diseases.Research Question: The aims of this study were: (1) to explore the associations between OSA, nocturnal oxyhemoglobin saturation (SpO2) and the history of several acute/chronic complications, (2) to investigate the impact of OSA and nocturnal SpO2 on several biomarkers (hematological, blood rheological, and coagulation) in patients with SCD.Study Design and Methods: Forty-three homozygous SCD patients underwent a complete polysomnography recording followed by blood sampling.Results: The proportion of patients suffering from nocturnal hypoxemia did not differ between those with and those without OSA. No association between OSA and clinical severity was found. Nocturnal hypoxemia was associated with a higher proportion of patients with hemolytic complications (glomerulopathy, leg ulcer, priapism, or pulmonary hypertension). In addition, nocturnal hypoxemia was accompanied by a decrease in RBC deformability, enhanced hemolysis and more severe anemia.Interpretation: Nocturnal hypoxemia in SCD patients could be responsible for changes in RBC deformability resulting in enhanced hemolysis leading to the development of complications such as leg ulcers, priapism, pulmonary hypertension or glomerulopathy.Clinical Trial Registration:www.ClinicalTrials.gov, identifier: NCT03753854.

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Section: Discussionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 95%

Nocturnal Hypoxemia Rather Than Obstructive Sleep Apnea Is Associated With Decreased Red Blood Cell Deformability and Enhanced Hemolysis in Patients With Sickle Cell Disease

et al. 2021

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“…Tissue hypoxia promotes endothelial injury and hypoxic pulmonary vasoconstriction. Nocturnal oxygen desaturation has been associated with biomarkers of hemolysis ( 133 ) and endothelial activation and adhesion ( 134 ). Associations between poor sleep quality, SDB, and desaturation with depression and pain are likely multidirectional ( 125 , 126 , 135 – 137 ) and need further investigation.…”

Section: Sdb and Hypoxemiamentioning

confidence: 99%

Identifying Clinical and Research Priorities in Sickle Cell Lung Disease. An Official American Thoracic Society Workshop Report

Ruhl¹,

Sadreameli²,

Allen³

et al. 2019

Annals ATS

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Background: Pulmonary complications of sickle cell disease (SCD) are diverse and encompass acute and chronic disease. The understanding of the natural history of pulmonary complications of SCD is limited, no specific therapies exist, and these complications are a primary cause of morbidity and mortality. Methods: We gathered a multidisciplinary group of pediatric and adult hematologists, pulmonologists, and emergency medicine physicians with expertise in SCD-related lung disease along with an SCD patient advocate for an American Thoracic Society–sponsored workshop to review the literature and identify key unanswered clinical and research questions. Participants were divided into four subcommittees on the basis of expertise: 1 ) acute chest syndrome, 2 ) lower airways disease and pulmonary function, 3 ) sleep-disordered breathing and hypoxia, and 4 ) pulmonary vascular complications of SCD. Before the workshop, a comprehensive literature review of each subtopic was conducted. Clinically important questions were developed after literature review and were finalized by group discussion and consensus. Results: Current knowledge is based on small, predominantly observational studies, few multicenter longitudinal studies, and even fewer high-quality interventional trials specifically targeting the pulmonary complications of SCD. Each subcommittee identified the three or four most important unanswered questions in their topic area for researchers to direct the next steps of clinical investigation. Conclusions: Important and clinically relevant questions regarding sickle cell lung disease remain unanswered. High-quality, multicenter, longitudinal studies and randomized clinical trials designed and implemented by teams of multidisciplinary clinician-investigators are needed to improve the care of individuals with SCD.

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“…In small cohorts of adults followed prospectively, 44% to 50% of subjects were found to have SDB with mild to moderate OSA based on apneahypopnea index (AHI). [79][80][81] The classification of OSA does differ between children and adults. In children, an AHI of 1 to 5 events per hour is classified as mild, 5 to 10 events per hour is moderate, and .10 events per hour constitutes severe OSA.…”

Section: Sdb and Nighttime Hypoxemia In Scdmentioning

confidence: 99%

How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders

Machogu

Machado

2018

Blood

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Hemoglobinopathies are caused by genetic mutations that result in abnormal hemoglobin molecules, resulting in hemolytic anemia. Chronic complications involving the lung parenchyma, vasculature, and cardiac function in hemoglobinopathies result in impaired gas exchange, resulting in tissue hypoxia. Hypoxia is defined as the deficiency in the amount of oxygen reaching the tissues of the body and is prevalent in patients with hemoglobinopathies, and its cause is often multifactorial. Chronic hypoxia in hemoglobinopathies is often a sign of disease severity and is associated with increased morbidity and mortality. Therefore, a thorough understanding of the pathophysiology of hypoxia in these disease processes is important in order to appropriately treat the underlying cause and prevent complications. In this article, we discuss management of hypoxia based on three different cases: sickle cell disease, β-thalassemia, and hereditary spherocytosis. These cases are used to review the current understanding of the disease pathophysiology, demonstrate the importance of a thorough clinical history and physical examination, explore diagnostic pathways, and review the current management.

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Nocturnal hemoglobin desaturation is associated with reticulocytosis in adults with sickle cell disease and is independent of obstructive sleep apnea

Cited by 14 publications

References 6 publications

Nocturnal Hypoxemia Rather Than Obstructive Sleep Apnea Is Associated With Decreased Red Blood Cell Deformability and Enhanced Hemolysis in Patients With Sickle Cell Disease

Nocturnal Hypoxemia Rather Than Obstructive Sleep Apnea Is Associated With Decreased Red Blood Cell Deformability and Enhanced Hemolysis in Patients With Sickle Cell Disease

Identifying Clinical and Research Priorities in Sickle Cell Lung Disease. An Official American Thoracic Society Workshop Report

How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders

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