Hemolysis in Sickle Cell Disease

Bensinger, Thomas A.

doi:10.1001/archinte.1974.00320160118010

Cited by 91 publications

(30 citation statements)

References 63 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…However, disorders of intravascular haemolysis disrupt these diffusion barriers, releasing haemoglobin into plasma, where it reacts rapidly with NO to oxidize it to nitrate (Figs 1B and 2) (Reiter et al, 2002). Although this is a stoichiometric and not catalytic reaction, the intravascular component of haemolysis in an adult with SCD can release over 3 g of haemoglobin into plasma per day (Bensinger & Gillette, 1974), equivalent to over 125 mg/h, capable of neutralising more than 7 mmol of NO per hour. This is often surpassed in PNH (Hill et al, in press) and severe haemolytic malaria.…”

Section: Nitric Oxide Scavengingmentioning

confidence: 99%

Pleiotropic effects of intravascular haemolysis on vascular homeostasis

Kato

Taylor

2010

Br J Haematol

View full text Add to dashboard Cite

Summary The breakdown of senescent or defective red blood cells releases red cell contents, especially hemoglobin, which scavenges nitric oxide (NO) and decomposes to heme and free iron. These are potent oxidants, all of which have promoted the evolution of inducible and vasculoprotective compensatory pathways to rapidly clear and detoxify hemoglobin, heme and iron. Chronic hemolytic red cell disorders as diverse as sickle cell disease, thalassemia, unstable hemoglobinopathy, cytoskeletal defects and enzymopathies have been linked to a clinical constellation of pulmonary hypertension, priapism, leg ulceration and possibly cerebrovascular disease and thrombosis. Besides free hemoglobin, hemolysis has been associated with extracellular arginase that limits substrate availability to NO synthase, endogenous inhibitors of NO synthase activity, and inappropriate activation of hemostatic pathways. This article reviews the hemolytic disorders that have been reported to manifest vascular complications, and explores the speculative possibility that hemolysis mediates some of the vascular complications of inflammation and diabetes.

show abstract

Section: Nitric Oxide Scavengingmentioning

confidence: 99%

Pleiotropic effects of intravascular haemolysis on vascular homeostasis

Kato

Taylor

2010

Br J Haematol

View full text Add to dashboard Cite

show abstract

“…CO inhibits the expression of plasminogen activator inhibitor, thereby attenuating the role of this procoagulant species in tissue injury (42). Interestingly, increased production of CO and increased circulating levels of carboxyhemoglobin are well recognized in SCD (16,21,33) and likely reflect increased expression of HO-1 in these patients (59,82).…”

Section: The Heme Oxygenase System As An Adaptive Response In Scdmentioning

confidence: 99%

The Perfusion Paradox and Vascular Instability in Sickle Cell Disease

2004

View full text Add to dashboard Cite

Sickle cell disease (SCD) exhibits a curious coexistence of contrasting perfusion profiles in the circulatory system: hypoperfusion is endemic in microcirculatory beds occluded by hemoglobin S-containing erythrocytes while hyperperfusion characterizes the systemic (macro)circulation and a number of regional vascular circuits. This review highlights this perfusion paradox of SCD, focusing on forearm blood flow and the renal circulation, and exploring the extent to which alterations in vasoactive systems (such as nitric oxide and prostanoids) are involved. Also reviewed are the mechanisms and pathways that contribute to altered vascular reactivity and vascular instability observed in SCD. Finally, the possibility that the induction of heme oxygenase-1, recently described in SCD, may confer a protective response in the vasculature and other tissues is discussed.

show abstract

“…Odonkor et a1 did find significantly higher basal metabolic rates in 8 teenage sickle cell patients when they were compared to 25 normal age-matched controls with similar thyroid hormone levels [ 131. Finally, an increase in endogenous carbon monoxide production has been documented in sickle cell disease, indicating that the bone marrow functions at a rate 6 times (range [3][4][5][6][7][8][9][10][11][12][13][14] that of normal subjects [ 121.…”

Section: Introductionmentioning

confidence: 99%

Nutrition and sickle cell disease

Reid

2012

Comptes Rendus. Biologies

View full text Add to dashboard Cite

Hemolysis in Sickle Cell Disease

Cited by 91 publications

References 63 publications

Pleiotropic effects of intravascular haemolysis on vascular homeostasis

Pleiotropic effects of intravascular haemolysis on vascular homeostasis

The Perfusion Paradox and Vascular Instability in Sickle Cell Disease

Nutrition and sickle cell disease

Contact Info

Product

Resources

About