A study of the geographic distribution and associated risk factors of leg ulcers within an international cohort of sickle cell disease patients: the CASiRe group analysis

Charles Antwi-Boasiako, Biree Andemariam, Raffaella Colombatti, Eugenia Vicky Asare, Crawford Strunk, Connie M. Piccone, Deepa Manwani, Donna Boruchov, Fatimah Farooq, Rebekah Urbonya, Samuel Wilson, Gifty Dankwah Boatemaa, Silverio Perrotta, Laura Sainati, Angela Rivers, Sudha Rao, William Zempsky, Ivy Ekem, Fredericka Sey, Catherine SegbefiaBaba Inusa, Immacolata Tartaglione, Andrew D. Campbell

Research output: Contribution to journalArticle

Abstract

Vasculopathy is a hallmark of sickle cell disease ultimately resulting in chronic end organ damage. Leg ulcer is one of its sequelae, occurring in ~ 5–10% of adult sickle cell patients. The majority of leg ulcer publications to date have emanated from single center cohort studies. As such, there are limited studies on the geographic distribution of leg ulcers and associated risk factors worldwide. The Consortium for the Advancement of Sickle Cell Research (CASiRe) was formed to improve the understanding of the different phenotypes of sickle cell disease patients living in different geographic locations around the world (USA, UK, Italy, Ghana). This cross-sectional cohort sub-study of 659 sickle cell patients aimed to determine the geographic distribution and risk factors associated with leg ulcers. The prevalence of leg ulcers was 10.3% and was associated with older age, SS genotype, male gender, and Ghanaian origin. In fact, the highest prevalence (18.6%) was observed in Ghana. Albuminuria, proteinuria, increased markers of hemolysis (lower hemoglobin, higher total bilirubin), lower oxygen saturation, and lower body mass index were also associated with leg ulceration. Overall, our study identified a predominance of leg ulcers within male hemoglobin SS patients living in sub-Saharan Africa with renal dysfunction and increased hemolysis.

Original languageEnglish (US)
JournalAnnals of Hematology
DOIs
StateAccepted/In press - 2020

Keywords

  • Hemolysis
  • International
  • Leg ulcers
  • Sickle cell

ASJC Scopus subject areas

  • Hematology

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    Antwi-Boasiako, C., Andemariam, B., Colombatti, R., Asare, E. V., Strunk, C., Piccone, C. M., Manwani, D., Boruchov, D., Farooq, F., Urbonya, R., Wilson, S., Boatemaa, G. D., Perrotta, S., Sainati, L., Rivers, A., Rao, S., Zempsky, W., Ekem, I., Sey, F., ... Campbell, A. D. (Accepted/In press). A study of the geographic distribution and associated risk factors of leg ulcers within an international cohort of sickle cell disease patients: the CASiRe group analysis. Annals of Hematology. https://doi.org/10.1007/s00277-020-04057-8