Zusammenfassung
Thrombotische Mikroangiopathien sind durch Thrombozytenaktivierung, Endothelzellschädigung, Hämolyse und mikrovaskuläre Okklusionen gekennzeichnet. Es handelt sich hierbei um eine Gruppe von Erkrankungen, deren Hauptvertreter die thrombotische thrombozytopenische Purpura (TTP) und das hämolytisch-urämische Syndrom (HUS) sind. Klinisch bestehen bei den Patienten eine mikroangiopathische hämolytische Anämie mit Thrombozytopenie und okklusionsbedingte Organischämien in variabler Ausprägung. Die Symptomatik der einzelnen Krankheitsbilder überschneidet sich häufig, sodass eine eindeutige Zuordnung anhand klinischer Kriterien oft schwierig ist. Aufgrund einer hohen Mortalität, insbesondere der TTP, sind eine schnelle Diagnostik und Therapie erforderlich. Es wird über 2 Patienten mit thrombotischen Mikroangiopathien nach kardiochirurgischen Eingriffen berichtet. Da TTP, HUS und eine medikamentöse Ätiologie weitgehend ausgeschlossen wurden, wurde ein Zusammenhang zwischen der extrakorporalen Zirkulation während dem herzchirurgischen Eingriff und der thrombotischen Mikroangiopathie vermutet.
Abstract
Thrombotic microangiopathies are characterized by platelet activation, endothelial damage, hemolysis and microvascular occlusion. This group of diseases is primary represented by thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Patients present with microangiopathic hemolytic anemia and thrombocytopenia as well as occlusion-related organ ischemia to a variable degree. A deficiency of the metalloprotease ADAMTS-13 is a major risk for acute disease manifestation as this is a regulator of unusually large von Willebrand factor (vWF) multimers, which are extremely adhesive and secreted by endothelial cells. In classical TTP an ADAMTS-13 activity below 5% is specific, whereas in other forms of thrombotic microangiopathies activity of ADAMTS-13 ranges from very low to normal. Symptoms of different forms of thrombotic microangiopathy are frequently overlapping and a clear classification according to clinical criteria is often difficult. Due to a high mortality, particularly of TTP, immediate diagnosis and therapy are essential. In this article two cases of thombotic microangiopathy after cardiac surgery are reported. After exclusion of TTP and HUS as well as other etiologies of thrombotic microangiopathy a relationship between the use of extracorporeal circulation and the pathogenesis of thrombotic microangiopathy is assumed.
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Schmidt, T., Tsakiris, D., Grapow, M. et al. Thrombotische Mikroangiopathien nach extrakorporaler Zirkulation. Anaesthesist 60, 451–456 (2011). https://doi.org/10.1007/s00101-010-1819-2
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DOI: https://doi.org/10.1007/s00101-010-1819-2
Schlüsselwörter
- Thrombotische Mikroangiopathie
- Purpura, thrombotisch thrombozytopenisch
- Extrakorporale Zirkulation
- ADAMTS-13-Protein, human
- Kardiovaskuläre Eingriffe