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Results for “Hemoglobinuria, Paroxysmal”

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[Recurrent acute myocardial infarction in a patient with nocturnal paroxysmal hemoglobinuria].

Paroxysmal nocturnal hemoglobinuria is a form of acquired hemolytic anemia with a high incidence of thrombotic complications, generally in the venous district; arterial thrombosis is rare, and exceptional in the coronary tree. We describe the case of a man who had two episodes of myocardial infarction, both during a hemoglobinuric crisis; this patient was free from cardiovascular risk factors and angiography revealed that there was no coronary stenosis. The clinical course was favorable and the patient's response to thrombolytic and dicumarol therapy was satisfactory. To our knowledge, this is the second case of coronary involvement in paroxysmal nocturnal hemoglobinuria described in the literature.

Hemoglobinuria, Paroxysmal↗

[Diagnostic role of magnetic resonance in the renal involvement in nocturnal paroxysmal hemoglobinuria].

Paroxysmal nocturnal haemoglobinuria or Machiafava-Micheli disease is an acquired clonal stem cell disorder characterized by defective haematopoiesis, which results in an increased sensitivity of the erythrocytes to complement-mediated intravascular haemolysis. Renal damage is rare but it can lead to chronic renal failure. Micro-infarctions due to repeated episodes of microvascular thrombosis and cortical haemosiderosis are thought to be the main contributors to the development of chronic renal failure in paroxysmal nocturnal haemoglobinuria. Magnetic resonance imaging provides characteristic images of the kidney. We describe a patient with cortical haemosiderosis and show the magnetic resonance features.

Adult↗