Hemostatic abnormalities in sickle cell disease

Lim, Ming Y.; Ataga, Kenneth I.; Key, Nigel S.

doi:10.1097/moh.0b013e328363442f

Cited by 71 publications

(42 citation statements)

References 46 publications

(20 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Moreover, Vesconi et al [26] did not confirm a prognostic significance of filling defects in ARDS; they reported instead a correlation of the microthrombi presence with ARDS etiology (posttraumatic syndrome) rather than with ARDS severity. However, immunothrombosis cannot easily be depicted by imaging studies since it evolves in microvessels that are not completely occluded, and the size of thrombi can be as small as <10 μm [29,30] .…”

Section: Immunothrombosis In Ards Pathophysiologymentioning

confidence: 99%

Immunothrombosis in Acute Respiratory Distress Syndrome: Cross Talks between Inflammation and Coagulation

Frantzeskaki¹,

Armaganidis²,

Orfanos³

2016

Respiration

216

231

View full text Add to dashboard Cite

Acute respiratory distress syndrome (ARDS) is defined as a syndrome of acute onset, with bilateral opacities on chest imaging and respiratory failure not caused by cardiac failure, leading to mild, moderate, or severe oxygenation impairment. The syndrome is most commonly a manifestation of sepsis-induced organ dysfunction, characterized by disruption of endothelial barrier integrity and diffuse lung damage. Imbalance between coagulation and inflammation is a predominant characteristic of ARDS, leading to extreme inflammatory response and diffuse fibrin deposition in vascular capillary bed and alveoli. Activated platelets, neutrophils, endothelial cells, neutrophil extracellular traps, microparticles, and coagulation proteases, participate in the complex process of immunothrombosis, which is a key event in ARDS pathophysiology. The present review is focused on the elucidation of immunothrombosis in ARDS and the potential therapeutic implications.

show abstract

Section: Immunothrombosis In Ards Pathophysiologymentioning

confidence: 99%

Immunothrombosis in Acute Respiratory Distress Syndrome: Cross Talks between Inflammation and Coagulation

Frantzeskaki¹,

Armaganidis²,

Orfanos³

2016

Respiration

216

231

View full text Add to dashboard Cite

show abstract

“…39 Acute red blood cell transfusion led to improved flow-mediated vasodilation in this study and chronic transfusion, which lowers plasma-free hemoglobin in SCD, 40 was associated with both lower TRV and improved flow-mediated vasodilation, suggesting improved vascular function over time. 39 Thromboembolism and precapillary pulmonary hypertension SCD is a condition with a predilection for thrombotic complications including pulmonary embolism, 41 and CTEPH is a major category of pulmonary hypertension (Table 1). Therefore, consideration needs to be given to the possibility that chronic thromboembolism may contribute to pulmonary hypertension in SCD.…”

Section: Hemolysis and Precapillary Pulmonary Hypertensionmentioning

confidence: 99%

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Gordeuk

Castro

Machado

2016

Blood

115

101

View full text Add to dashboard Cite

Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments improve SCD-related pulmonary hypertension.

show abstract

“…In parallel with SCT, the risk appears to be particularly elevated for PE rather than isolated DVT (Lim, et al 2013, Naik, et al 2014b, Novelli, et al 2012, Stein, et al 2006). Mechanistically, many more studies have evaluated the laboratory manifestations of activation of coagulation and platelets in SCA (Ataga and Key 2007, Lim, et al 2013) compared to SCT (Lawrie, et al 2012, Westerman, et al 2002). Although it is premature to assume a common mechanism, the proximal event is presumably related to the abnormal sickle haemoglobin.…”

Section: Sickle Cell Trait and Venous Thromboembolismmentioning

confidence: 99%

Negative health implications of sickle cell trait in high income countries: from the football field to the laboratory

2015

Self Cite

View full text Add to dashboard Cite

Worldwide, sickle cell trait is a highly prevalent gene carrier state. While generally a benign condition with a normal life expectancy, it is becoming increasingly clear that the sickle trait is associated with certain adverse outcomes. This article will focus on three of these outcomes, namely exertional rhabdomyolysis and sudden death, chronic renal dysfunction, and venous thromboembolism. In each case, the epidemiological evidence for the association is reviewed, together with the existing data on potential underlying mechanisms. Because newborn screening programmes for sickle cell anaemia also identify those with sickle cell trait, it is imperative that further studies determine what, if any, preventive measures can be taken to reduce the burden of these uncommon but potentially morbid complications in affected individuals.

show abstract

Hemostatic abnormalities in sickle cell disease

Cited by 71 publications

References 46 publications

Immunothrombosis in Acute Respiratory Distress Syndrome: Cross Talks between Inflammation and Coagulation

Immunothrombosis in Acute Respiratory Distress Syndrome: Cross Talks between Inflammation and Coagulation

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Negative health implications of sickle cell trait in high income countries: from the football field to the laboratory

Contact Info

Product

Resources

About