Vikita Mehta1, Abirami Kirubarajan2, Amir Sabouhanian2, Sanasi M Jayawardena2, Priya Chandrakumaran2, Nila Thangavelu3, Refai Cader4, Sachith Mettananda5, Dayananda Bandara6,7, Shawn Khan2, David J Weatherall8, Angela Allen8, Anuja P Premawardhena9, Nancy F Olivieri10. 1. Arts and Science, McMaster University, Hamilton, Ontario, Canada. 2. Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada. 3. Health Sciences, McMaster University, Hamilton, Ontario, Canada. 4. Policy Analysis and Development, Ministry of Health, Colombo, Sri Lanka. 5. Department of Paediatrics, Faculty of Medicine, University of Kelaniya, Kelaniya, Sri Lanka. 6. National Thalassaemia Centre, Kurunegala, Sri Lanka. 7. Teaching Hospital Kurunegala, Kurunegala, Sri Lanka. 8. MRC Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom. 9. Faculty of Medicine, University of Kelaniya, Kelaniya, Sri Lanka. 10. Pediatrics, Medicine and Public Health Sciences, University of Toronto, Toronto, Ontario, Canada.
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.
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.
Authors: Caterina P Minniti; James Eckman; Paola Sebastiani; Martin H Steinberg; Samir K Ballas Journal: Am J Hematol Date: 2010-10 Impact factor: 10.047
Authors: Vikki G Nolan; Adeboye Adewoye; Clinton Baldwin; Ling Wang; Qianli Ma; Diego F Wyszynski; John J Farrell; Paola Sebastiani; Lindsay A Farrer; Martin H Steinberg Journal: Br J Haematol Date: 2006-06 Impact factor: 6.998