Cancer specific survival in patients with sickle cell disease

Brunson, Ann; Keegan, Theresa H.M.; Mahajan, Anjlee; Paulukonis, Susan; Wun, Theodore

doi:10.1111/bjh.15687

Cited by 8 publications

(7 citation statements)

References 8 publications

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“…During the follow up period, 3 (2.9%), 11 (2.7%), 27 (3.1%), and 10 (3.2%) individuals had a malignant neoplasm diagnosis code in the Both Drugs, Neither Drug, HU Only, and DFX Only cohorts, respectively, which is similar to previous reported prevalence of cancer in those with SCD. 13 …”

Section: Resultsmentioning

confidence: 99%

Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

Wong

Valle

Paulukonis

2021

Health Science Reports

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Background and aims When patients with sickle cell disease have appropriate indications, they can be prescribed hydroxyurea (HU) and deferasirox (DFX) concurrently despite little knowledge about how the two medications interact. We wished to analyze whether there was evidence of adverse interaction between HU and DFX when taken simultaneously and hypothesized that those who took both drugs together had similar clinical complications when compared to those who took only one or neither drug. Methods We conducted this retrospective cohort investigation between 2009 and 2016 of persons with SCD in the California Sickle Cell Data Collection Program, a validated database of Californians with SCD a statewide. People in the database who took HU and DFX simultaneously for at least 3 months as compared to those who took either HU or DFX alone or to matched persons who took neither drug were eligible. Results We identified 104 people who were prescribed both HU and DFX concurrently, 877 who were prescribed HU only, and 314 who were prescribed DFX only during the study period. We identified 416 matched controls who took neither HU nor DFX. People who took both HU and DFX concurrently had similar rates of ED and inpatient encounters and had similar rates and distribution of adverse effects compared to those who took either HU or DFX alone or took neither drug. Conclusion Three months of concurrent use of DFX and HU appears safe, but further studies are required to better understand the safety and effectiveness of this medication combination. (Funded by CDC, CDC Foundation, and others).

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Section: Resultsmentioning

confidence: 99%

Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

Wong

Valle

Paulukonis

2021

Health Science Reports

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“…In addition, the lack of disease surveillance may delay the diagnosis of rare comorbid complications that can be sufficiently evaluated only with large data sets (eg, the worse outcome of cancer in persons with SCD). 22 Furthermore, as new therapies are developed for these rare conditions and translated into clinical care, it is imperative to evaluate their positive and negative impacts at the population level. These data cannot be obtained using 1 data set (eg, individual hospital electronic health record data) and instead require multiple sources of combined, linked, and deduplicated data.…”

Section: The Value Of Surveillance For Rare Diseasesmentioning

confidence: 99%

Improving Outcomes for Patients With Sickle Cell Disease in the United States

et al. 2021

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IMPORTANCEAlthough considered a rare disease with fewer than 200 000 cases annually in the US, sickle cell disease (SCD) is the most common and clinically significant inherited blood disorder in the US and worldwide. Despite the relatively high prevalence of this rare disease, there is a paucity of longitudinal data available to evaluate access to care or to identify quality metrics.OBSERVATIONS This review discusses why systematic data collection for SCD through populationwide surveillance programs can help to facilitate progress in treatment. It also explores the importance of having both a longitudinal clinical registry and a national surveillance program to improve resource utilization, clinical outcomes, and provide an equitable foundation for care. CONCLUSIONS AND RELEVANCEFederal funding should be appropriately allocated to establish and maintain a national SCD surveillance system supported by the Centers for Disease Control and Prevention, as well as a longitudinal registry available at recognized sickle cell centers.

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“…The SCD cohort was identified using longitudinal records from the California Patient Discharge Data (PDD) and the Emergency Department Utilization (EDU) databases from the Office of Statewide Health Planning and Development, as previously reported. [9][10][11][12][13] Since July 1990, the State of California has required that nonfederal hospitals report up to 25 diagnoses and up to 21 procedures associated with each hospitalization, coded using the International Classification of Disease, Ninth Revision, Clinical Modification (ICD-9-CM). Since 2005, the EDU database of all hospital-associated emergency department (ED) encounters has also been mandated.…”

Section: Cohort Definitionmentioning

confidence: 99%

“…41, 282.42, 282.60, 282.61, 282.62, 282.63, 282.64, 282.68, 282.69) for all hospitalizations available in the California PDD and EDU databases. [9][10][11][12][13][14] To be included in the SCD cohort, patients had to meet 1 of the following criteria: 2 separate admissions with SCD as the principal diagnosis, or 1 admission with SCD as the principal diagnosis and 2 additional admissions with SCD as a secondary diagnosis. To increase specificity, all patients had to be younger than 65 years at entry into the cohort.…”

Section: Cohort Definitionmentioning

confidence: 99%

“…Frequency of hospitalization was defined using a patient's average number of visits per year for any cause; patients with 3 or more visits per year were defined as frequent users, as this number of hospitalizations has previously been shown to be associated with an increase in mortality. [9][10][11][12][13] For this analysis, we excluded visits with bleeding as the principal diagnosis from calculation of hospitalization frequency. Patients with fewer than 3 visits per year were defined as less frequent users.…”

Section: Covariatesmentioning

confidence: 99%

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Bleeding in patients with sickle cell disease: a population-based study

Hariharan¹,

Brunson

Mahajan

et al. 2020

Blood Advances

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Bleeding is a known complication of sickle cell disease (SCD) and includes hemorrhagic stroke, hematuria, and vitreous hemorrhage. However, the incidence of bleeding events in patients with SCD has not been well described. We present a retrospective, population-based study examining the cumulative incidence of bleeding in 6423 patients with SCD from 1991 to 2014. We also studied risk factors associated with bleeding and the effects of bleeding on mortality, using Cox proportional hazards regression models. We used California emergency department and hospitalization databases to identify patients with SCD with intracranial hemorrhage, gastrointestinal (GI) bleeding, hemophthalmos, gross hematuria, epistaxis, menorrhagia, and other bleeding events. The cumulative incidence of any first bleeding event at age 40 years was 21% (95% confidence interval [CI], 19.8%-22.3%), increasing with age to 41% by age 60 years (95% CI, 38.8%-43.1%). The majority of bleeding events were GI (41.6%), particularly from the upper GI tract. A higher bleeding risk was associated with increased frequency of hospitalization (hazard ratio [HR], 2.16; 95% CI, 1.93-2.42), venous thromboembolism 180 days before bleeding event (HR, 4.24; 95% CI, 2.86-6.28), osteonecrosis of the femoral head (HR, 1.25; 95% CI, 1.08-1.46), and ischemic stroke (HR, 1.65; 95% CI, 1.20-2.26). Bleeding was also associated with a twofold increased risk for death (HR, 2.09; 95% CI, 1.82-2.41) adjusted for other SCD-related complications. Our novel finding of a high incidence of bleeding in patients with SCD, particularly from the upper GI tract, suggests that patients with SCD may be predisposed to bleeding, with possible etiologies including increased use of nonsteroidal anti-inflammatory drugs, mucosal infarction from vascular occlusion by sickled red blood cells, and increased stress ulceration from frequent hospitalization.

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Cancer specific survival in patients with sickle cell disease

Cited by 8 publications

References 8 publications

Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

Improving Outcomes for Patients With Sickle Cell Disease in the United States

Bleeding in patients with sickle cell disease: a population-based study

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