Pathophysiologic considerations for the interactions between obstructive sleep apnea and sickle hemoglobinopathies
Section snippets
Background
Sickle cell trait is a benign genetic carrier state without hematological manifestations. In the United States, sickle cell trait occurs in 7–10% of the black population [1]. Obstructive sleep apnea is an increasingly common disorder affecting 4% of middle-aged adults [2] and African-Americans may have a higher prevalence than Caucasian-Americans [3]. Therefore, co-existent sickle cell trait and OSA would not be expected to be rare.
Normal hemoglobin (HbA) consists of two alpha and two beta
Hypothesis
Vaso-occlusion in sickle hemoglobinopathies involves multiple pathways that include polymerization, sickle cell–endothelial cell interactions, vascular reactivity, leucocyte activation and hypercoagulability. Since OSA adversely affects these processes it can convert sickle cell trait to a condition with overt vaso-occlusive episodes.
Polymerization
The most important determinant in the occurrence of polymerization is deoxygenation. The degree of hypoxia required to initiate polymerization varies with the quantity of HbS in the erythrocyte. In HbAS erythrocytes, polymerization begins at oxygen saturation of 60% [14]. This degree of hypoxia is unlikely to be simply secondary to OSA but other conditions that promote hemoglobin deoxygenation exist in OSA.
Respiratory acidosis is common in obstructive sleep apnea. Hydrogen ions shift the
Conclusion
There seems to be enough preliminary evidence to justify investigating a possible interaction between OSA and sickling hemoglobinopathies. The preceding discussion does not exhaust the current state of knowledge on pathophysiologic processes in these diseases but highlights areas where experimental evidence exists for perturbations of similar processes in both conditions. It is important to point out that the existence of hemostatic activation, for example, in both conditions does not establish
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The utility of thromboelastography and thrombin generation in assessing the prothrombotic state of adults with sickle cell disease
2017, Thrombosis ResearchCitation Excerpt :This acquired thrombophilic state appears to be a risk factor for premature death in SCD [7,8]. The pathophysiology of VOC is thought to be multifactorial, not only due to polymerisation of HbSS but also involvement of endothelial, platelet and white cell activation [9,10]. Hydroxycarbamide (HC) therapy and regular blood transfusion therapy have both shown to decrease the frequency of VOC [11–14].
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Association between adenotonsillar hypertrophy and sickle cell disease in adolescents
2019, Pediatria PolskaAdenotonsillar hypertrophy in pre-school children with sickle cell disease and diagnostic accuracy of the sleep disturbance scale for children
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2015, Revista Cubana de Hematologia, Inmunologia y Hemoterapia