Case Series and Review of Hematological and Non-Hematological Malignancies in Aging Patients with Sickle Cell Disease in the Hydroxyurea Era

Abstract

Survival of adult patients with sickle cell disease has increased progressively since the 1970s. Aging patients with sickle cell disease are at risk of developing comorbidities that are not due to sickle cell disease itself, including malignancies. Many studies tried to assess the incidence of malignancy in patients with sickle cell disease. However, no studies have been done to evaluate cancer incidences in aging sickle cell patients, especially in the hydroxyurea (HU) era. In this review, we assessed the prevalence of malignancies in aging patients with sickle cell disease at our institution with or without HU therapy. Retrospective analysis of hospital records identified patients who had been diagnosed to carry sickle cell disease and malignancies before 2020 using the International Statistical Classification of Diseases and Related Health Problems (ICD-10) coding. Four hundred and eighty-three sickle cell disease patients were seen in our inpatients/outpatients offices. Among these, 12 sickle cell disease patients had a confirmed diagnosis of malignancy. The patients were classified into three categories based on age groups: four patients who were 60 years and older had multiple myeloma. Solid tumors were found in 5/6 patients, aged 40–60 who had the Hb S (HBB: c.20A>T) (β^S/β^S) genotype with signs of iron overload. Two patients, aged 25 and 35, had hematological malignancies. The number of patients on HU was too small to make any comment on relationship to malignancy or mortality. This study is only one institution’s experience, further investigation on a larger scale is needed to look into cancer incidences in this population.

Keywords:

• Aging sickle cell patients
• hydroxyurea (HU)
• malignancies

Acknowledgments

The authors acknowledge all the team members of Thomas Jefferson Hospital, Philadelphia, PA, USA sickle cell program. Ethics approval and consent to participate: The review was approved by the Institutional Review Board of Thomas Jefferson University with a request for waiver of subjects’ authorization to collect protected health information. The datasets generated during and/or analyzed during the current study are available from the corresponding author upon reasonable request. Authors’ contributions: S. Rizk, S.K. Ballas and D.J. Axelrod took care of the patients involved in the study. R. Olaosebikan reviewed the charts and made the data collection. S. Rizk and S.K. Ballas reviewed the literature, did data analysis and wrote the review. D.J. Axelrod reviewed the review. The manuscript was read and approved by all the authors.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Additional information

Funding

NB: Dr. Rasaq Olaosebikan was supported by a National Institutes of Health training fellowship grant No. T32GM008562.