Leg ulcer in a case of thalassemia minor (a case report).SR Vagaskar, RJ Fernandez, FP Mistry
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Source of Support: None, Conflict of Interest: None
A rare case of leg ulcer in a 28 years old woman with thalassemia minor is reported.
Keywords: Adult, Case Report, Female, Human, Leg Ulcer, etiology,Thalassemia, complications,
Leg ulcers very often have a haematological cause, sickle cell anemia and congenital spherocytosis being the most frequent. However, thalassemia minor as a cause of leg ulcers is very rare, the first case being reported by Estes et al. Thalassemia intermedica (5% of cases) is often associated with leg ulcers, but not the major form.
A 28 year old female patient came with a complaint of a leg ulcer over the left medial malleolus. which had been present intermittently since 9 months. There was no history of trauma, pedal edema, varicose veins, calf pain, or a history suggestive of collagen vascular disease.
Physical examination revealed the patient to have pallor. There was a single shallow irregular shaped ulcer of 7.5 cm x 5 cm size over the left medial malleolus with a granulating floor The surrounding skin showed minimal erythema and hyperpigmentation. There were no varicose veins. The dorsalis pedis and posterior tibial arterial pulsations were well felt. There was no splenomegaly.
The haemoglobin was 10 gm/100 ml with a reticulocyte count of 0.5%. The peripheral smear showed hypochromic, microcytic anemia with poikilocytes and target cells. Sickling was absent and osmotic fragility was decreased. Haemoglobin A2 was 4.5%, haemoglobin F 1.9%, Hb A 97.6% and serum iron was 55 microgram/100 ml (normal-70 microgram-200 microgram/100 ml). Total iron binding capacity was 545 microgram/100 ml (normal-250 microgram-404 microgram/100 ml) and transferrin saturation was 10.1% (normal-25%-43%). All the other investigations were within normal limits. The patient's father and elder sister showed a thalassemia trait. The other family members were not available for screening.
The patient was admitted to the hospital and treated with systemic antibiotics for 14 days. The ulcer was treated with zinc acetate spray and aluminium hydroxide cream. Within 3 weeks, the ulcer decreased to one third its original size and had completely healed at the time of discharge, 2 weeks later.
Reports of leg ulcers in association with thalassemia have been few, possibly because the underlying cause was not identified. Estes, et al were the first to report its association in three patients who had the minor form of the disease. March et al reported the same association in a patient with thalassemia minor as did Cooper and Wacker.
Most of the reported cases were females of Italian descent, with unilateral or bilateral malleolar ulcers. The average age of onset of the ulcers was 25.6 years with a range of 14-44 years. The haemoglobin ranged from 6.3-10.0 gm/100 ml.
Very few cases of leg ulcers due to thalassemia minor have been reported from India. Raha and Sur Roychaudhury reported one such case in association with thalassemia- E. Five cases of leg ulcers in association with thalassemia of intermediate severity have been reported by Mehta and Agarwal and Agarwal and Mehta.
The pathogenesis of leg ulcers in thalassemia minor is not known. A splenectomy does not influence the occurrence of the ulcers, which are believed to occur due to relative hypoxia from chronic anemia in tissues where orthostatic pressures delay the return of blood. Secondary infection may also play a part.
Our patient was asymptomatic for 27 years and the leg ulcer was the presenting manifestation of the disease. Apart from the ulcer, the patient had pallor and showed altered haematological picture. She had no other findings. We are reporting this case in view of its rarity and to highlight the importance of ruling out a haematological cause in a non-healing leg ulcer.