Extract
1Department of Nephrology, 2Department of Pathology and 3Department of Cardiology, University Hospital Aachen, Aachen, Germany
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Introduction
Thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome (TTP-HUS) represent clinical entities with the common findings: consumptive thrombocytopenia, microangiopathic haemolytic anaemia, intravascular fibrin/platelet thrombus formation and consecutive organ dysfunction. Although overlaps exist, traditionally, the TTP (Moschcowitz syndrome with predominant central nervous system involvement) and the HUS (Gasser syndrome with predominant renal involvement) have been differentiated on clinical grounds.
The introduction of plasmapheresis has provided substantiated benefit in terms of survival rate and the mortality rate of TTP-HUS has been reduced from over 90% to ∼15–20% [1–3]. However, despite the rapid initiation of up-to-date therapy, TTP-HUS remains a potentially life-threatening disorder. The causes of death depend on the variability of vital organ affection (e.g. the central nervous system or myocardial involvement). We describe two patients with fatal TTP-HUS, in whom autopsy confirmed widespread myocardial hyaline thromboses correlating well to the clinical picture of acute myocardial infarction and therapy-resistant cardiogenic shock.
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