Antiphospholipid antibodies, proteins C and S, and coagulation changes in sickle cell disease

Westerman, Maxwell P.; Gilman‐Sachs, Alice; Beaman, Kenneth D.; Freels, Sally; Boggio, Lisa; Allen, Sandra; Zuckerman, L; Schlegel, Robert; Williamson, Patrick

doi:10.1016/s0022-2143(99)90149-x

Cited by 128 publications

(139 citation statements)

References 37 publications

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“…10,11,[13][14][15][16][19][20][21][22] In some of these studies, the authors described differences between Table 2. Tissue factor expression, markers of coagulation, endothelial and inflammation activation in controls, hemoglobin SC and sickle cell anemia patients.…”

Section: Discussionmentioning

confidence: 99%

“…10,11,[19][20][21][22] Since the focus of these studies was not HbSC disease, only a few compared the HbSC patients with normal controls. 11,19,21,22 When this comparison was made, the results were variable and no conclusions could be drawn. It is important to emphasize that, since our study was focused specifically on HbSC disease, we included a large cohort of these patients (n=56), whereas only very small numbers were included in the previous studies (<18 cases).…”

Section: Discussionmentioning

confidence: 99%

“…It is important to emphasize that, since our study was focused specifically on HbSC disease, we included a large cohort of these patients (n=56), whereas only very small numbers were included in the previous studies (<18 cases). 10,11,19,21,22 Since we included a larger number of patients, we were able to evaluate associations of hyperoagulability markers with clinical complications in HbSC patients.…”

Section: Discussionmentioning

confidence: 99%

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Elevated hypercoagulability markers in hemoglobin SC disease

et al. 2015

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ABSTRACTincluded in the study was being treated with hydroxyurea. Patients who were on anticoagulation were excluded. A total of 56 HbSC patients were compared to a population of 39 HbSS patients, of whom 15 were described in a previous study. 17 We also selected 27 healthy age-matched HbAA controls. The University's ethics committee approved the study and all of the patients included signed a written informed consent form.Venous blood samples for all of the study analyses, including peripheral blood counts and hemolysis markers, were obtained simultaneously.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Elevated hypercoagulability markers in hemoglobin SC disease

et al. 2015

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“…We found a relation of total protein S to hemoglobin levels in our HbSS patients. Both protein S and protein C can exert their anticoagulant effects on phosphatidylserine expressing sickle red blood cell membranes [7,8,10]. In patients with lower hemoglobin levels, there are more dense cells and membrane-shed vesicles, as well as reticulocytes (which all have high phosphatidylserine exposure) [44][45][46][47][48].…”

Section: Discussionmentioning

confidence: 99%

Protein C and S and inflammation in sickle cell disease

et al. 2004

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Reduced activity of naturally occurring anticoagulants (NOAC) protein C and protein S may contribute to vaso-occlusion in sickle cell disease (SCD). We studied whether protein C and S are related to clinical vaso-occlusion, hematological markers of disease severity (hemoglobin levels, leukocyte counts, and percentage of fetal hemoglobin), and inflammation in SCD. Protein C activity, protein S (free and total) antigen, endothelial activation markers (soluble vascular cell adhesion molecule-1 [sVCAM-1], von Willebrand antigen [vWF]), and high sensitive C-reactive protein (hsCRP) levels were measured in 30 HbSS and 20 HbSC patients and in race-matched HbAA controls. NOAC levels were reduced in patients, and endothelial activation markers and hsCRP were elevated (except vWF in HbSC patients). Protein C activity and vWF levels were lower in HbSC patients who experienced painful crises compared to HbSC patients who were clinically asymptomatic. No other differences were observed between patients who did and did not experience vaso-occlusive events (painful crises, stroke, acute chest syndromes) or leg ulcers. A significant positive correlation between total protein S with hemoglobin levels and a significant negative correlation between total and free protein S and sVCAM-1 were detected in HbSS patients. Except perhaps for protein C in relation to painful crises in HbSC patients, these markers were not associated with the occurrence of clinical events. The protein S, hemoglobin, and sVCAM-1 associations may suggest decreased endothelial protein S production due to the more severe endothelial perturbation in HbSS patients with lower hemoglobin levels. Am.

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“…Indeed, despite the consumption already present in these patients at steady state, levels of some pro-and anticoagulant factors such as factors V, X, XI, IX, II and antithrombin often remain in normal ranges whereas others such as protein C and protein S are significantly decreased. In addition, factor VIII levels, phospholipid-bearing microparticles, and the prevalence of antiphospholipid antibodies are often increased [7][8][9][10][11][12]. It remains unclear whether the global clotting capacity of SCD patients is altered.…”

Section: Introductionmentioning

confidence: 99%

Thrombin generation reveals high procoagulant potential in the plasma of sickle cell disease children

et al. 2011

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Changes in several components of the clotting system are well documented in sickle cell disease (SCD) patients. However, whether the global hemostatic potential of these patients is altered is still unclear. Calibrated automated thrombogram 1 method of thrombin generation (TG) was used to characterize the hemostatic potential of 83 SCD children (75 SS, 6 SC, and 2 Sb thal ) at steady-state as compared with 50 controls of the same range of age. TG was triggered using 1 pM tissue factor and 4 lM phospholipids with and without thrombomodulin. Thirteen SCD children were also evaluated during vaso-occlusive crisis. Protein C activity, free protein S and D-dimers levels were measured in parallel. SCD patients showed higher rates of thrombin formation, higher thrombin peak height (with and without thrombomodulin), and higher endogenous thrombin potential (ETP) than controls in the presence of thrombomodulin. Reduction of ETP (RETP) in the presence of thrombomodulin was lower in SCD group compared with controls and correlated both with protein C and protein S levels. ETP, RETP, peak height, and velocity index of TG correlated with D-dimers. Compound heterozygous patients showed an intermediate hemostatic phenotype at steady-state. No significant difference was observed when comparing TG parameters during vaso-occlusive crisis to those obtained at steady-state in the same patients. The global hemostatic potential is increased and reflects the hypercoagulable state of SCD patients even at steady-state. The relevance of this finding with respect to the risk of thrombotic complications of the disease needs further investigation. Am. J. Hematol. 87:145-149, 2012. V

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Antiphospholipid antibodies, proteins C and S, and coagulation changes in sickle cell disease

Cited by 128 publications

References 37 publications

Elevated hypercoagulability markers in hemoglobin SC disease

Elevated hypercoagulability markers in hemoglobin SC disease

Protein C and S and inflammation in sickle cell disease

Thrombin generation reveals high procoagulant potential in the plasma of sickle cell disease children

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