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

Mehta, Vikita, Kirubarajan, Abirami, Sabouhanian, Amir, Jayawardena, Sanasi M, Chandrakumaran, Priya, Thangavelu, Nila, Cader, Refai, Mettananda, Sachith, Bandara, Dayananda, Khan, Shawn, Weatherall, David J, Allen, Angie, Premawardhena, Anuja P and Olivieri, Nancy F (2022) 'Leg Ulcers: A Report in Patients with Hemoglobin E Beta Thalassemia and Review of the Literature in Severe Beta Thalassemia'. Acta haematologica, Vol 45, Issue 3, pp. 334-343.

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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.

Item Type:	Article
Subjects:	QZ Pathology > Manifestations of Disease > QZ 140 General manifestations of disease WA Public Health > Statistics. Surveys > WA 900 Public health statistics WH Hemic and Lymphatic Systems > Hematologic Diseases. Immunologic Factors. Blood Banks > WH 170 Hemolytic anemia (e.g., Sickle cell anemia)
Faculty: Department:	Biological Sciences > Department of Tropical Disease Biology
Digital Object Identifer (DOI):	https://doi.org/10.1159/000520731
Depositing User:	Julie Franco
Date Deposited:	14 Dec 2021 09:59
Last Modified:	29 Jun 2022 10:56
URI:	https://archive.lstmed.ac.uk/id/eprint/19641

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