Leg Ulcers: A Report in Patients with Hemoglobin E Beta Thalassemia and Review of the Literature in Severe Beta Thalassemia

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 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 two largest treatment centres 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 two centres, 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. Discussion/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.

Non-Transfusion-Dependent Thalassemia: A Panoramic Review

“…Leg ulcers were reported to occur in 7.9% of patients with β-TI and up to 22% of patients with hemoglobin E/β-thalassemia [ 42 , 59 ]. There are multiple hypotheses regarding the etiology of leg ulcers, including chronic hypoxia due to anemia, increased oxygen affinity of HbF, hypercoagulable state, and iron overload [ 60 ].…”

“…There are multiple hypotheses regarding the etiology of leg ulcers, including chronic hypoxia due to anemia, increased oxygen affinity of HbF, hypercoagulable state, and iron overload [ 60 ]. Leg ulcers usually manifest in the second decade of life after minor trauma, most commonly on the medial or lateral malleoli [ 59 , 61 ]. They heal slowly and tend to recur or become chronic, causing pain and disability [ 59 , 60 , 61 ].…”

“…Leg ulcers usually manifest in the second decade of life after minor trauma, most commonly on the medial or lateral malleoli [ 59 , 61 ]. They heal slowly and tend to recur or become chronic, causing pain and disability [ 59 , 60 , 61 ]. Increasing age, iron overload, hypercoagulability, and splenectomy are risk factors for the development of leg ulcers [ 2 , 42 ].…”