A Rare Blood Malignancy in a Genetic Hematological Disorder: Polycythemia Vera (PV) in Sickle Cell Disease (SCD)

Journal of clinical haematology Pub Date : 2023-08-12 DOI:10.33696/haematology.4.053

C. L. Edwards, Sharena Scott, M. Boggan, J. Meek, W. J. Bryson, Alexandria McDougald, Milo Broadnax, C. Barker, Jessica Miller, J. Sollers, Tanisha I. Burford, J. Livingston, E. Whitworth, G. Byrd, Kelvin Williams, Sherry C. Eaton, Malik Muhammad, George Cliette, Dana Jones, Brianna Downey, Hilary T. Dietahin, Merrell Turner, Roland Thorpe, Keith Whitfield, Debra O. Parker, E. Robinson, M. Wood, Kenyon Railey, Shiv Sudhakar, Wandy Morel Cubilete, N. Shah

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Abstract

Objective: To delineate the etiology, symptomatology, and treatment of Polycythemia Vera in adults with Sickle Cell Disease. The current review contains a review of the 4 case reports that we found on the topic. To our knowledge, no other case reports exist. Methods: We reviewed the scientific literature to discover case reports that included the topic of PV. We noted consistencies in presentation, evaluation, treatment, and clinical outcomes. Results: We reviewed 4 case reports and a limited number of clinical papers on PV in SCD. We found and reported on consistencies in clinical presentation and the diversity of treatments. We reported hematological, bone marrow, and radiographic findings. Conclusions: There is great variability in the evaluation and treatment of cases of PV in SCD. We advocate for more research and deconstructing the complicated relationship between these two comorbid disorders.

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遗传性血液病中一种罕见的血液恶性肿瘤:镰状细胞病(SCD)中的真性红细胞增多症(PV)

目的:探讨成人镰状细胞病真性红细胞增多症的病因、症状及治疗。当前的综述包括对我们发现的关于该主题的4个病例报告的综述。据我们所知，没有其他病例报告存在。方法:我们回顾科学文献，发现包括PV主题的病例报告。我们注意到在表现、评估、治疗和临床结果方面的一致性。结果:我们回顾了4例病例报告和有限数量的关于SCD PV的临床论文。我们发现并报告了临床表现的一致性和治疗的多样性。我们报告了血液学、骨髓学和放射学的发现。结论:在SCD的PV病例的评估和治疗方面存在很大的差异。我们提倡对这两种共病之间的复杂关系进行更多的研究和解构。

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Journal of clinical haematology

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