Malignancy in patients with sickle cell disease: A single center experience

JOURNAL OF CLINICAL ONCOLOGY(2023)

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e22551 Background: The development of malignancy in patients with sickle cell disease has been documented in multiple case reports and case series. The incidence of malignancy in patients with sickle cell disease is unknown. Large national databases like the National Cancer Database, North American Association of Central Cancer Registries, and SEER do not collect data on the presence of sickle cell disease. This has made it difficult to calculate the incidence of malignancy in this patient population. In our study, we aim to calculate the prevalence of malignancy in sickle cell patients following our institution. Methods: After IRB approval, a retrospective analysis was conducted of adult sickle cell disease patients being followed at our Hematology outpatient clinic. We included patients aged > / = 18 years with sickle cell disease (HBSS, HBSC, HBS-beta thalassemia) as per hemoglobin electrophoresis, who were followed at the clinic from 1/1/2017 to 10/1/2022. We excluded prisoners. We did an extensive chart review to identify patients with a diagnosis of malignancy by pathology. Variables of age, sex, race, type of sickle cell disease, type of malignancy, age at the diagnosis of malignancy, and treatment were collected. Continuous descriptive data were summarized as means. Categorical data were summarized with a sample percentage. The prevalence of malignancy was calculated. SAS for windows 9.4 was used for analysis. Results: 438 patients with sickle cell disease were identified from 2017 to 2022. All the patients were African American. Their ages ranged from 16 to 84 with a mean of 35 years. Of these patients 53 % were females and 47 % were male. 62% had HBSS disease, 25 % had HBSC and 13% had HBS beta thalassemia genotype. 4 patients were diagnosed with malignancy. All 4 patients were female. The prevalence of malignancy was calculated as 0.91%. Conclusions: We found four patients with malignancy in the patient population with sickle cell disease at our hematology clinic. This data is based on limited sample size. The incidence of malignancy in sickle cell patients should be calculated in a larger population base. Further studies can be done to determine if a single type of sickle cell genotype increases the risk of malignancy. All the centers where sickle cell patients are followed should monitor the patients prospectively for the development of malignancy.[Table: see text]
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sickle cell disease
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