scholarly journals High incidence of venous thromboembolism recurrence in patients with sickle cell disease

2019 ◽  
Vol 94 (8) ◽  
pp. 862-870 ◽  
Author(s):  
Ann Brunson ◽  
Theresa Keegan ◽  
Anjlee Mahajan ◽  
Richard White ◽  
Ted Wun
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
High Incidence ◽  
Venous Thromboembolism Recurrence

scholarly journals The effect of anticoagulant choice on venous thromboembolism recurrence and bleeding in sickle cell disease

2020 ◽  
Author(s):  
Vishal K. Gupta ◽  
Rachel Strykowski ◽  
Brittany Scarpato ◽  
Romy Lawrence ◽  
Sarah L. Khan ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Venous Thromboembolism Recurrence

Sickle cell trait and the risk of venous thromboembolism among blacks

Blood ◽  
2007 ◽  
Vol 110 (3) ◽  
pp. 908-912 ◽  
Author(s):  
Harland Austin ◽  
Nigel S. Key ◽  
Jane M. Benson ◽  
Cathy Lally ◽  
Nicole F. Dowling ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Odds Ratio ◽  
Sickle Cell Trait ◽  
Coagulation System ◽  
Cell Disease ◽  
Hemoglobin C ◽  
Increased Risk ◽  
S Allele

Abstract People with sickle cell disease have a chronically activated coagulation system and display hemostatic perturbations, but it is unknown whether they experience an increased risk of venous thromboembolism. We conducted a case–control study of venous thromboembolism that included 515 hospitalized black patients and 555 black controls obtained from medical clinics. All subjects were assayed for hemoglobin S and hemoglobin C genotypes. The prevalence of the S allele was 0.070 and 0.032 for case patients and controls, respectively (P < .001). The odds that a patient had sickle cell trait were approximately twice that of a control, indicating that the risk of venous thromboembolism is increased approximately 2-fold among blacks with sickle cell trait compared with those with the wild-type genotype (odds ratio = 1.8 with 95% confidence interval, 1.2-2.9). The odds ratio for pulmonary embolism and sickle cell trait was higher, 3.9 (2.2-6.9). The prevalence of sickle cell disease was also increased among case patients compared with controls. We conclude that sickle cell trait is a risk factor for venous thromboembolism and that the proportion of venous thromboembolism among blacks attributable to the mutation is approximately 7%.

scholarly journals Sickle cell disease, sickle trait and the risk for venous thromboembolism: a systematic review and meta-analysis

2018 ◽  
Vol 16 (1) ◽  
Author(s):  
Jean Jacques Noubiap ◽  
Mazou N. Temgoua ◽  
Ronni Tankeu ◽  
Joel Noutakdie Tochie ◽  
Ambroise Wonkam ◽  
...  
Keyword(s):  
Systematic Review ◽  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Cell Disease

Overcoming challenges of venous thromboembolism in sickle cell disease treatment

2019 ◽  
Vol 12 (3) ◽  
pp. 173-182 ◽  
Author(s):  
Foluso Joy Ogunsile ◽  
Rakhi Naik ◽  
Sophie Lanzkron
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Disease Treatment

Venous Thromboembolism In Pregnant Women With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2217-2217
Author(s):  
Craig D. Seaman ◽  
Margaret V. Ragni ◽  
Charity G. Moore ◽  
Jie Li ◽  
Jonathan Yabes
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Pregnant Women ◽  
Sickle Cell ◽  
Pregnancy Complications ◽  
Categorical Variables ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Discharge Data

Abstract Background The risk of venous thromboembolism (VTE) is increased in pregnancy and sickle cell disease (SCD), yet the rates of pregnancy-related VTE are not firmly established in SCD, nor are other SCD-related complications, including pneumonia, vasooclusive crisis (VOC), and acute chest syndrome (ACS), which may be clinically indistinguishable from VTE. Moreover, the American College of Chest Physicians has made no recommendations regarding thromboprophylaxis in pregnant women with SCD. Methods Inpatient hospital discharge data for PE for the most recent 5-year period available, 2007-2011, were obtained from the Pennsylvania Health Care Cost Containment Council (PHC4). We compared VTE, pregnancy complications, medical co-morbidities, and mortality among pregnant women with and without SCD. Among pregnant SCD women with and without VTE, we also compared rates of pneumonia, vasooclusive crisis (VOC), and acute chest syndrome (ACS). All patient identifiers were removed. Diagnostic categories and co-morbidities were obtained using ICD-9 codes. Data from categorical variables were analyzed by chi-square or Fisher's exact test; and from continuous variables by two-sample Student t-test. Results The prevalence of VTE was 2.8% among pregnant women with SCD. Among pregnant women with SCD and VTE, the rate of pneumonia, 28.6% vs. 4.4%, p=0.043, was significantly higher than in those without VTE. While somewhat higher, the rates of VOC, 57.1% vs. 24.7%, p=0.073, and ACS, 14.3% vs. 2.4%, p=0.177, were not significantly different between pregnant SCD VTE and non-VTE groups. Comparing VTE and non-VTE pregnant SCD women, the rate of pregnancy complications did not differ, p=0.999; nor did the rates of medical co-morbidity, other than diabetes, 28.6% vs. 3.6%, p=0.031. Among the subset of pregnant SCD with pneumonia, the prevalence of VOC, 80.0% vs. 36.1%, p=0.001; ACS, 35.0% vs. 2.9%, p<0.001; and length of stay, 12.5 vs. 4.6 days, p=0.030, were significantly greater than in those without pneumonia. Among the subset of SCD pregnancies with VOC, the prevalence of preeclampsia, 28.4% vs. 13.5%, p=0.003; pneumonia, 15.7% vs. 2.6%, p=0.001; ACS, 12.8% vs. 0.6%, p<0.001; and length of stay, 7.7 vs. 3.6 days, p<0.001, were significantly more common than in those without VOC. Among the subset of SCD pregnancies with ACS, the rates of intrauterine fetal death, 14.3% vs. 1.6%, p=0.036; postpartum infection, 28.6% vs. 6.6%, p=0.016; pneumonia, 50.0% vs. 5.3%, p<0.001; and VOC, 92.9% vs. 36.5%, p<0.001, were significantly higher than in those without ACS. Overall, the rate of VTE, among SCD women with pneumonia, VOC, and/or ACS, 6.6%, was significantly higher than among those without these conditions, 2.2%, p<0.001. Discussion The prevalence of pregnancy-related VTE in women with SCD, while low, 2.8%, appears to be at least 10-fold greater than the general 0.2% incidence of pregnancy-related VTE in the unaffected population. Further, the higher rates of VTE we observed among pregnant women with SCD-related complications, including pneumonia, VOC, or ACS, and the well-recognized potential clinical overlap with VTE, suggest that VTE may be missed in such women, and that VTE rates in pregnant women with SCD may be higher than herein reported or previously recognized. The presence of pneumonia, VOC, or ACS appears to be associated with VTE and may be a potential marker(s) of its occurrence. Prospective studies, however, are needed to determine the incidence of VTE in pregnant women with SCD with and without complicating pneumonia, VOC, and/or ACS. We conclude that pregnancy-related VTE may be more common than previously recognized in pregnant women with SCD and, if confirmed, such women might be candidates for thromboprophylaxis. Disclosures: No relevant conflicts of interest to declare.

scholarly journals The molecular basis for the prothrombotic state in sickle cell disease

Haematologica ◽  
2020 ◽  
Vol 105 (10) ◽  
pp. 2368-2379
Author(s):  
Arun S. Shet ◽  
Maria A. Lizarralde-Iragorri ◽  
Rakhi P. Naik
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Molecular Basis ◽  
Cell Disease ◽  
Prothrombotic State ◽  
Thrombotic Risk ◽  
Increased Risk ◽  
Innovative Therapies ◽  
History Of

The genetic and molecular basis of sickle cell disease (SCD) has long since been characterized but the pathophysiological basis is not entirely defined. How a red cell hemolytic disorder initiates inflammation, endothelial dysfunction, coagulation activation and eventually leads to vascular thrombosis, is yet to be elucidated. Recent evidence has demonstrated a high frequency of unprovoked/recurrent venous thromboembolism (VTE) in SCD, with an increased risk of mortality among patients with a history of VTE. Here, we thoroughly review the molecular basis for the prothrombotic state in SCD, specifically highlighting emerging evidence for activation of overlapping inflammation and coagulation pathways, that predispose to venous thromboembolism. We share perspectives in managing venous thrombosis in SCD, highlighting innovative therapies with the potential to influence the clinical course of disease and reduce thrombotic risk, while maintaining an acceptable safety profile.

scholarly journals Red blood cells modulate structure and dynamics of venous clot formation in sickle cell disease

Blood ◽  
2019 ◽  
Vol 133 (23) ◽  
pp. 2529-2541 ◽  
Author(s):  
Camille Faes ◽  
Anton Ilich ◽  
Amandine Sotiaux ◽  
Erica M. Sparkenbaugh ◽  
Michael W. Henderson ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Plasminogen Activator ◽  
Sickle Cell ◽  
Whole Blood ◽  
Blood Cells ◽  
Cell Disease ◽  
Increased Risk ◽  
Blood Clots ◽  
Tissue Plasminogen

Abstract Sickle cell disease (SCD) is associated with chronic activation of coagulation and an increased risk of venous thromboembolism. Erythrocyte sickling, the primary pathologic event in SCD, results in dramatic morphological changes in red blood cells (RBCs) because of polymerization of the abnormal hemoglobin. We used a mouse model of SCD and blood samples from sickle patients to determine if these changes affect the structure, properties, and dynamics of sickle clot formation. Sickling of RBCs and a significant increase in fibrin deposition were observed in venous thrombi formed in sickle mice. During ex vivo clot contraction, the number of RBCs extruded from sickle whole blood clots was significantly reduced compared with the number released from sickle cell trait and nonsickle clots in both mice and humans. Entrapment of sickled RBCs was largely factor XIIIa–independent and entirely mediated by the platelet-free cellular fraction of sickle blood. Inhibition of phosphatidylserine, but not administration of antisickling compounds, increased the number of RBCs released from sickle clots. Interestingly, whole blood, but not plasma clots from SCD patients, was more resistant to fibrinolysis, indicating that the cellular fraction of blood mediates resistance to tissue plasminogen activator. Sickle trait whole blood clots demonstrated an intermediate phenotype in response to tissue plasminogen activator. RBC exchange in SCD patients had a long-lasting effect on normalizing whole blood clot contraction. Furthermore, RBC exchange transiently reversed resistance of whole blood sickle clots to fibrinolysis, in part by decreasing platelet-derived PAI-1. These properties of sickle clots may explain the increased risk of venous thromboembolism observed in SCD.

scholarly journals Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: an administrative database study

2018 ◽  
Vol 2 (3) ◽  
pp. 285-291 ◽  
Author(s):  
Riten Kumar ◽  
Joseph Stanek ◽  
Susan Creary ◽  
Amy Dunn ◽  
Sarah H. O’Brien
Keyword(s):  
Risk Factors ◽  
Retrospective Study ◽  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Administrative Database ◽  
Similar Data ◽  
Cell Disease ◽  
Database Study ◽  
Key Points

Key Points Adults with SCD have an increased incidence of VTE, but similar data in children are lacking. In this 7-year, multicenter retrospective study, 1.7% of children with SCD developed VTE.

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