Introduction: Beta thalassemia major is a hereditary anemia secondary to deficient production of the beta-globins in structurallynormal hemoglobin, with a consequent precocious destruction of red globules present in the circulation. Coagulation and cardiaccomplications due to the accumulation of iron are the main causes of death in these patients.Clinical case: The authors present a case of cardiac iron overload complicated by fatal multi-organ embolism due to a left ventricularthrombus in a beta-thalassemia major patient.Discussion: The presence of chronic hypercoagulability related to pro-thrombotic anomalies in beta-thalassemia major patientsmay be complicated by thrombolytic embolism events. On the basis of our experience, we believe that to prevent rapid deterioration ofthe patient to fatal multi-organ embolism secondary to thrombolytic detachment it's advisable to perform systemic thrombolysis ratherthan surgery for a single occlusion which precludes the use of thrombolytic agents.

Clinical case: Fatal multi-organ left ventricular embolism in a patient with beta thalassemia major undergoing endovascular treatment for stenosis of the sub-renal abdominal aorta

CANTARELLA, Giovanni;LA CAMERA, Giuseppa;
2015

Abstract

Introduction: Beta thalassemia major is a hereditary anemia secondary to deficient production of the beta-globins in structurallynormal hemoglobin, with a consequent precocious destruction of red globules present in the circulation. Coagulation and cardiaccomplications due to the accumulation of iron are the main causes of death in these patients.Clinical case: The authors present a case of cardiac iron overload complicated by fatal multi-organ embolism due to a left ventricularthrombus in a beta-thalassemia major patient.Discussion: The presence of chronic hypercoagulability related to pro-thrombotic anomalies in beta-thalassemia major patientsmay be complicated by thrombolytic embolism events. On the basis of our experience, we believe that to prevent rapid deterioration ofthe patient to fatal multi-organ embolism secondary to thrombolytic detachment it's advisable to perform systemic thrombolysis ratherthan surgery for a single occlusion which precludes the use of thrombolytic agents.
beta-thalassemia; echocardiography; thrombophilia
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/20.500.11769/36279
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