Erythropoietin levels were determined in 50 Greek females: 20 beta-thalas-saemia (beta-thai) heterozygotes, 15 with a diagnosis of iron-deficiency anaemia and 15 normal controls. In beta-thai trait carriers, the erythropoietin levels were slightly higher than in normal controls (16.65 ± 4.43 vs. 12.84 ± 2.47 mU/ml); these levels were significantly lower than those in iron-deficient subjects with the same degree of anaemia (55.24 ± 31.35 mU/ml). In both groups, the erythropoietin levels are statistically correlated with the severity of anaemia (r = -0.537 p < 0.05 for iron deficiency; r = -0.610 p < 0.01 for beta-thai heterozygotes). In beta-thai heterozygotes, a close inverse correlation with red cell number and erythropoietin levels was also noted. It is suggested that microcytosis accompanying beta-thai trait constitutes an additional factor intervening in the regulation of erythropoiesis.
The coexistence of a pseudoxanthoma elasticum (PXE)-like syndrome in beta-thalassemia and other hemoglobinopathies is a recently established clinical entity that has been observed with a significant frequency and related to some severe, even life-threatening complications. We present here a thalassemia intermedia patient who developed unstable angina in a setting of severe anemia and PXE-related coronary arterial calcification. Besides the clinical significance of this PXE-like syndrome, its acquired nature may introduce some new thoughts regarding the pathogenesis of atherosclerosis.
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