Venous Thrombosis Biomarkers in Sickle Cell Disease and Sickle Cell Trait

The natural history of SCD 04-H-0161 protocol (NCT00081523) will specifically be leveraged to recruit participants with VTE. In this study we will assess feasibility of establishing a prospective natural history study of thrombosis in SCD. Establishing feasibility could lead to recruitment of SCD patients experiencing thrombosis, particularly those that are understudied including patients with HbSC disease.

• INCLUSION CRITERIA:

Sickle cell disease with and without VTE

• Sickle cell disease (HbSS, HbSC and HbS/beta-thalassemia genotypes) in steady state.
• Diagnosis of at least one or more VTE within 5 years of study enrolment confirmed by radiologic imaging (for SCD patients with VTE).
• Absence of clinical history of VTE (for SCD controls)
• Between 18 and 80 years of age.
• Ability to provide informed written consent.

Sickle cell trait

• Sickle cell disease (HbAS genotype).
• Absence of clinical history of VTE
• Between 18 and 80 years of age.
• Ability to provide informed written consent.

Ethnically matched controls

• Between 18 and 80 years of age.
• African, or of African descent.
• Ability to provide informed written consent.
• Absence of clinical history of VTE

EXCLUSION CRITERIA:

SCD with and without VTE

• Pregnancy (test done at enrollment; if a subject becomes pregnant during the study period, samples will not be obtained while the subject is pregnant and the subject will be taken off study).
• Patients on exchange transfusion or having received a simple blood transfusion in the past 60 days.
• Active viral infection as evidenced by testing positive for hepatitis B surface antigen or hepatitis C virus (HCV) antibody (Ab) with signs of active hepatitis B or C virus infection. If the subject is positive for HCV Ab, a reverse transcriptasepolymerase chain reaction test will be conducted. Subjects with hepatitis C may be rescreened after receiving appropriate hepatitis C treatment.
• Testing positive for human immunodeficiency virus 1 or 2 Ab with evidence for ongoing active infection (i.e., CD 4 count <400/microL and viral load >100,000 copies/ml) on antiretroviral therapy.
• Active acute inflammatory disorders rheumatoid arthritis or systemic lupus erythematosus on disease modifying therapy.
• Diabetes mellitus judged to be under poor control by the Investigator evidenced by a single fasting sugar value >250gm/dl or requiring >3 antidiabetic agents, including insulin (all insulins are considered 1 agent); use of insulin per se is not exclusionary.

SCT and ethnically matched controls

• Diagnosis of any of the following chronic disease or conditions: Sickle cell disease (HbSS, HbSC and HbS/beta-thalassemia genotypes).
• Clinical history of VTE.
• Pregnancy (test done at enrollment; if a subject becomes pregnant during the study period, samples will not be obtained while the subject is pregnant and the subject will be taken off study.
• Active viral infection as evidenced by testing positive for hepatitis B surface antigen or hepatitis C virus (HCV) antibody (Ab) with signs of active hepatitis B or C virus infection. If the subject is positive for HCV Ab, a reverse transcriptasepolymerase chain reaction test will be conducted. Subjects with hepatitis C may be rescreened after receiving appropriate hepatitis C treatment.
• Testing positive for human immunodeficiency virus 1 or 2 Ab with evidence for ongoing active infection (i.e., CD 4 count <400/microL and viral load >100,000 copies/ml) on antiretroviral therapy.
• Active acute inflammatory disorders rheumatoid arthritis or systemic lupus erythematosus on disease modifying therapy.
• Diabetes mellitus judged to be under poor control by the Investigator evidenced by a single fasting sugar value >250gm/dl or requiring >3 antidiabetic agents, including insulin (all insulins are considered 1 agent); use of insulin per se is not exclusionary.

National Heart, Lung, and Blood Institute (NHLBI)

hypercoagulable state Recurrence Biomarkers	Venous Thromboembolism Vascular Mortality Natural History

Layout table for MeSH terms

Thrombosis Thromboembolism Venous Thromboembolism Venous Thrombosis Anemia, Sickle Cell Sickle Cell Trait Thrombophilia Embolism and Thrombosis	Vascular Diseases Cardiovascular Diseases Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn