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Arterial calcifications in beta-thalassemia
A Aessopos1, M Samarkos, E Voskaridou
1First Department of Internal Medicine, University of Athens, Greece.
Insights
Arterial calcifications are common in older patients with beta-thalassemia, potentially indicating an acquired pseudoxanthoma elasticum (PXE) syndrome. This suggests a higher risk of vascular events in these individuals.
Area of Science:
- Vascular Medicine
- Genetics
- Dermatology
Background:
- Beta-thalassemia patients often exhibit angioid streaks and skin lesions similar to pseudoxanthoma elasticum (PXE).
- Arterial calcifications are a known manifestation of PXE, suggesting a potential link in beta-thalassemia.
Purpose of the Study:
- To determine the incidence of arterial calcifications in beta-thalassemia patients.
- To investigate the association between arterial calcifications, angioid streaks, and PXE-like skin lesions in beta-thalassemia.
Main Methods:
- Radiographic assessment of tibial arteries for calcifications in 40 beta-thalassemia patients (age >30) and 40 controls.
- Clinical examination for PXE skin lesions and angioid streaks (AS).
Main Results:
- Arterial calcifications were found in 55% of beta-thalassemia patients versus 15% of controls (P < 0.01).
- PXE skin lesions and AS were present in 20% and 52% of patients, respectively.
- 85% of patients had at least one of the three conditions: arterial calcifications, AS, or PXE-like skin lesions.
Conclusions:
- Arterial calcifications are prevalent in older beta-thalassemia patients.
- This may represent an acquired PXE syndrome associated with beta-thalassemia.
- Vascular complications similar to those in PXE should be anticipated in these patients.
Abstract:
The purpose of this study was to define the incidence of arterial calcifications in patients with beta-thalassemia. Beta-thalassemia patients have been shown to present a high prevalence of angioid streaks and skin lesions characteristic of pseudoxanthoma elasticum (PXE). Given the fact that vascular involvement in the form of arterial calcifications is also a common manifestation of PXE, the authors investigated radiographically the presence of arterial calcifications in beta-thalassemia patients. They studied 40 patients with beta-thalassemia over 30 years of age. Forty healthy, age- and sex-matched subjects were chosen as a control group. Radiographs of the tibias were performed in order to disclose arterial calcifications. The occurrence of PXE skin lesions and of angioid streaks (AS) was also investigated. Arterial calcifications were detected in the posterior tibial artery in 22 (55%) beta-thalassemia patients and in six (15%) controls (P < 0.01 for the comparison). PXE skin lesions and AS were found in eight (20%) and 21 (52%) patients respectively. A total of 34 patients (85%) had at least one of the three lesions, namely, arterial calcifications, angioid streaks, and/or PXE-like skin lesions. Stepwise logistic regression analysis did not reveal prognostic value in independent variables such as transfusions, chelation therapy, pseudoxanthoma elasticum skin lesions and/or angioid streaks, diabetes, hemoglobin, serum ferritin, and uric acid. It was concluded that arterial calcifications are common in older beta-thalassemia patients. This finding could be a manifestation of an acquired PXE syndrome associated with beta-thalassemia, and consequently, vascular events complicating PXE should be expected in these patients.