ARTERIAL CALCIFICATIONS IN BETA-THALASSEMIA

The purpose of this study was to define the incidence of arterial calc ifications in patients with beta-Thalassemia. beta-thalassemia patient s have been shown to present a high prevalence of angioid streaks and skin lesions characteristic of pseudoxanthoma elasticum (PXE). Given t he fact that vascular involvement in the form of arterial calcificatio ns is also a common manifestation of PXE, the authors investigated rad iographically the presence of arterial calcifications in beta-thalasse mia patients. They studied 40 patients with beta-thalassemia over 30 y ears 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 lesion s and of angioid streaks (AS) was also investigated. Arterial calcific ations were detected in the posterior tibial artery in 22 (55%) beta-t halassemia patients and in six (15%) controls (P<0.01 for the comparis on). PXE skin lesions and AS were found in eight (20%) and 21 (52%) pa tients respectively. A total of 34 patients (85%) had at least one of the three lesions, namely, arterial calcifications, angioid streaks, a nd/or PXE-like skin lesions. Stepwise logistic regression analysis did not reveal prognostic value in independent variables such as transfus ions, 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 acq uired PXE syndrome associated with beta-thalassemia, and consequently, vascular events complicating PXE should be expected in these patients .