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Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes.

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Sickle cell disease

SCD is characterized by a remarkable phenotypic complexity; common acute complications are acute pain events, acute chest syndrome and stroke; chronic complications (including chronic kidney disease) can damage all organs.
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Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease.

It is proposed that LDH elevation identifies patients with a syndrome of hemolysis-associated NO resistance, endothelial dysfunction, and end-organ vasculopathy, as well as a clinical subphenotype of pulmonary hypertension, leg ulceration, priapism, and risk of death in patients with sickle cell disease.
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Platelet activation in patients with sickle disease, hemolysis-associated pulmonary hypertension, and nitric oxide scavenging by cell-free hemoglobin.

In patients with SCD, administration of sildenafil, a phosphodiesterase-5 inhibitor that potentiates NO-dependent signaling, reduced platelet activation, supports a role for NO-based therapeutics for SCD vasculopathy.
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Intravascular hemolysis and the pathophysiology of sickle cell disease

Intravascular hemolysis represents an intrinsic mechanism for human vascular disease that manifests clinical complications in sickle cell disease and other chronic hereditary or acquired hemolytic anemias.
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Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease.

These data support a novel mechanism of disease in which hemolysis contributes to reduced nitric oxide bioavailability and endothelial dysfunction via release of erythrocyte arginase, which limits arginine bioavailability, and release of ______ hemoglobin, which scavengesNitric oxide.
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Levels of soluble endothelium‐derived adhesion molecules in patients with sickle cell disease are associated with pulmonary hypertension, organ dysfunction, and mortality

It is found that higher levels of soluble vascular cell adhesion molecule‐1 (sVCAM‐1) were associated with markers indicating renal dysfunction and hepatic impairment, and increased soluble adhesion molecules expression correlated with severity of pulmonary hypertension, a clinical manifestation of endothelial dysfunction.
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An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.

Evidence-based recommendations for the management of patients with SCD with increased mortality risk are provided, but will require frequent reassessment and updating.
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Lipid levels in sickle‐cell disease associated with haemolytic severity, vascular dysfunction and pulmonary hypertension

Evaluated lipid and lipoprotein levels and their relationship to markers of intravascular haemolysis, vascular dysfunction and PH characterize elevated plasma triglyceride levels as a potential risk factor for PH in SCD.
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Elevated tricuspid regurgitant jet velocity in children and adolescents with sickle cell disease: association with hemolysis and hemoglobin oxygen desaturation

The hypotheses that elevated jet velocity affects 10% of pediatric patients, is associated with both hemolysis and hypoxia, and has clinical correlates with acute chest syndrome, stroke, transfusion requirement and abnormal 6-minute walk test results are tested.
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