Incidence of Malignancies in Californians with Sickle Cell Disease

Project: Research project

Project Details


Small case series have suggested increased incident cancer in patients with sickle cell disease(SCD), which, if confirmed, might affect cancer surveillance recommendations for thesepatients. Experimental data have linked chronic inflammation and high cellular turnover, bothhallmarks of SCD, to increased risk of malignancy. Because increased incident cancer in SCDhas important implications for management of these patients, it is necessary to rigorously testthis finding in a large patient cohort. We propose to determine the incidence of cancer inCalifornians with SCD by linking two existing databases created for surveillance purposes?theCalifornia Cancer Registry (CCR) and the California Office of Statewide Health Planning andDevelopment (OSHPD) Patient Discharge and Emergency Department Utilization. We estimatebetween 6,000-8,000 cases with SCD will be found. We will test the hypothesis that cancer riskis increased in the SCD population through the following specific aims. Specific Aim 1:Determine the standardized incidence ratio of cancer amongst Californians with SCD. The SCDcohort will be created using a search algorithm informed by the Registry and SurveillanceSystem for Hemoglobinopathies (RuSH) project, a collaboration between the Centers forDisease Control and Prevention (CDC) and the National Heart Lung and Blood Institute. Thealgorithm has been validated by the CDC-funded Public Health Research, Surveillance andEpidemiology in Hemoglobinopathies (PHRESH) study. Specific Aim 2: Describe theepidemiology of cancer in patients with SCD, including types of malignancies, demographics,and potential risk factors associated with incident cancer. These data will have significantimpact on our knowledge of the complications of SCD, and important implications for cancerscreening as survival for patients with SCD improves. It will also provide insight into how SCDaffects cancer-specific survival. Assessment of potential risk factors associated withdevelopment of malignancy in the SCD population that can be pursued in future, moremechanistic studies. An example might be an association between malignancy and the numberof transfusions, severity of SCD as measured by frequency of admissions or othercomplications, or co-morbidities such as autoimmune disease. These results will also serve aspreliminary data for an R01 proposal that would determine the epidemiology of cancer and riskfactors for cancer in patients with SCD from the seven states that participated in the RuSHproject (California, Georgia, Florida, Michigan, North Carolina, New York, and Pennsylvania).
Effective start/end date8/6/166/30/18


  • National Institutes of Health: $121,361.00


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