1. Leg Ulcers: A Report in Patients with Hemoglobin E Beta Thalassemia and Review of the Literature in Severe Beta Thalassemia
- Author
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Sachith Mettananda, Anuja Premawardhena, Dayananda Bandara, Priya Chandrakumaran, Amir Sabouhanian, Nancy F. Olivieri, David J. Weatherall, Sanasi M. Jayawardena, Refai Cader, Shawn Khan, Angela Allen, Nila Thangavelu, Abirami Kirubarajan, and Vikita Mehta
- Subjects
congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Severe beta thalassemia ,medicine.medical_treatment ,Thalassemia ,Hemoglobin E-beta thalassemia ,hemic and lymphatic diseases ,Internal medicine ,medicine ,Humans ,In patient ,Original Paper ,Wound Healing ,business.industry ,Hemoglobin E ,Medical record ,Leg Ulcer ,beta-Thalassemia ,wa_900 ,wh_170 ,Beta thalassemia ,Hematology ,General Medicine ,medicine.disease ,qz_140 ,digestive system diseases ,Skin grafting ,business ,Complication - Abstract
Background: Leg ulcers are a frequent complication in patients with the inherited hemoglobin disorders. In thalassemia, the literature is limited, and factors associated with the development of leg ulcers in hemoglobin E (HbE) beta thalassemia, the most common form of severe beta-thalassemia worldwide, have not previously been reported. Methods: We reviewed all available medical records of patients with HbE beta thalassemia to document the onset of leg ulcers at the 2 largest treatment centers in Sri Lanka. We reviewed the literature to identify studies reporting outcomes of interventions for ulcers in severe thalassemia. Results: Of a total of 255 actively registered patients with HbE thalassemia in the 2 centers, 196 patient charts were evaluable. A leg ulcer with a documented date of onset was recorded in 45 (22%) of 196 evaluable patients, aged (mean ± SEM) 22.2 ± 1.4 years. Most had been irregularly transfused; steady-state hemoglobin was 6.4 ± 0.2 g/dL. Treatment achieving healing in 17 patients included transfusions, antibiotics, oral zinc, wound toileting, and skin grafting. Conclusion: Leg ulcers may be more common in HbE beta thalassemia than in other forms of thalassemia. A systematic approach to treatment will be needed to document the prevalence and factors placing such patients at risk for leg ulcers. Controlled trials to evaluate the optimal treatment of this common complication are indicated.
- Published
- 2021
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