Abstract
The development of a diffuse elastic tissue defect resembling pseudoxanthoma elasticum (PXE) is a recently established and frequently encountered clinical entity in β-thalassemia. The clinical spectrum of this disorder is not yet completely understood as it is continuously being enriched with novel complications that are often serious. We present here a thalassemia intermedia patient with typical PXE manifestations and a fusiform aneurysmatic dilatation of the ascending thoracic aorta, a previously unknown complication in these patients. An aortic aneurysm has itself a notable morbidity, while it may also impair the particularly susceptible cardiac function of β-thalassemia patients, hence affecting the overall prognosis of the main disease.
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Farmakis, D., Vesleme, V., Papadogianni, A. et al. Aneurysmatic dilatation of ascending aorta in a patient with β-thalassemia and a pseudoxanthoma elasticum-like syndrome. Ann Hematol 83, 596–599 (2004). https://doi.org/10.1007/s00277-004-0859-6
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DOI: https://doi.org/10.1007/s00277-004-0859-6