Pseudoxanthoma Elasticum Lesions and Cardiac Complications as Contributing Factors for Strokes in β-Thalassemia Patients

Abstract

Background and Purpose Pseudoxanthoma elasticum (PXE) lesions, which lead to intracranial hemorrhages and cardiac complications, predisposing to thrombotic strokes, are frequent findings in β-thalassemia. Nevertheless, the association of these lesions with strokes in thalassemic patients has not been previously discussed. Methods Ten β-thalassemic patients who developed an intracranial hemorrhage or a thrombotic stroke were reviewed. Results In the group of the four patients presenting with hemorrhage, one had PXE lesions, one had cardiac abnormalities, and one both PXE and cardiac disorders. In the group presenting with thrombotic stroke, all six patients had cardiac abnormalities and platelet count elevation due to splenectomy. Three also had PXE findings. No other predisposing factor for stroke was present. Conclusions Cardiac complications and PXE may be risk factors for strokes in β-thalassemia. Their frequent coexistence leads to a therapeutic dilemma in patients requiring antithrombotic therapy.