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Common, But Neglected: A Comprehensive Review of Leg Ulcers in Sickle Cell Disease
Tarun Sahu1, Henu Kumar Verma, Satyaki Ganguly
1Tarun Sahu, PhD, is Research Fellow, Department of Physiology, All India Institute of Medical Sciences, Raipur, India. Henu Kumar Verma, PhD, is Researcher, Stem Cell Laboratory, Institute of Experimental Endocrinology and Oncology, National Research Council, Naples, Italy. At the All India Institute of Medical Sciences, Satyaki Ganguly, MD, DNB, is Associate Professor, Department of Dermatology; Meenakshi Sinha, PhD, is Associate Professor, Department of Physiology; and Ramanjan Sinha, PhD, is Professor and Head, Department of Physiology. The authors have disclosed no financial relationships related to this article. Submitted August 26, 2020; accepted in revised form October 22, 2020.
Objective:
To compile available evidence to better understand the management of leg ulcers in sickle cell disease (SCD), as well as describe potential therapeutic steps that may be required to improve the quality of life of patients with SCD leg ulcers.
Data Sources:
MEDLINE, PubMed, EMBASE, and Web of Science databases.
Study Selection:
A comprehensive search was conducted to retrieve relevant studies using the keywords "sickle cell disease and leg ulcer," "ulcer treatments, diagnosis and sickle cell," and "wound sickle cell." Studies published through July 2020 were included.
Data Extraction:
Two independent authors selected all studies that assessed the relationship between leg ulcer and SCD identified from online databases.
Data Synthesis:
The authors have summarized updated information on pathophysiology (vasculopathy linked to chronic hemolysis and endothelial dysfunction), diagnosis, and available treatment options to unravel the dermohematologic connection between leg ulcers and SCD.
Conclusions:
It is the authors' hope that this detailed discussion of the information available on leg ulcers and SCD will lead to a better appreciation of this clinical problem by the clinicians and researchers and in turn have a long-term positive effect on the quality of life of patients with SCD. Researchers should design new trials considering these insights and potential therapeutic approaches based on current knowledge.
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