[Spontaneous liver rupture as a rare complication of the HELLP syndrome].

The HELLP syndrome (haemolysis, elevated liver enzymes, low platelet count) was first referred to by Weinstein in 1982 as an extremely progressive form of gestosis. In addition to the more common gestotic symptoms, such as oedema, proteinuria and hypertension, the clinical picture is characterized by microangiopathic haemolysis, thrombocytopenia and, especially, impaired hepatic function. Within this clinical picture severe complications can occur, such as eclamptic attacks, renal dysfunction, intracranial haemorrhage, intrahepatic haemorrhage and coagulopathy. An imbalance in prostanoid metabolism has been implicated in the pathogenesis. A decrease in synthesis of the vasodilator and thrombocyte aggregation inhibitor prostacyclin leads to a preponderance of the vasoconstrictor thromboxane A2, which promotes thrombocyte aggregation. This results in local vascular spasms and endothelial lesions, which in the case of hypercoagulopathy are accompanied by the formation of fibrin deposits with resultant vascular constriction. Intravascular fibrin deposits indicate that the coagulation system has been compromised and can lead to consumption coagulopathy in approximately 10% of cases. In the majority of cases, however, one finds low-grade disseminated intravascular coagulation (DIC), i.e. mild hypercoagulopathy with thrombocytopenia, a tendency to thrombocyte aggregation and fibrinogen deficiency in the presence of usually normal plasmatic coagulation. These vascular changes occur particularly in organs that have high blood flow, such as liver, kidneys and placenta. In the liver, sinusoidal obstruction causes vascular congestion, leading to an increase in intrahepatic pressure, dilatation of Glisson's capsule, development of subcapsular hepatic haematomas and hepatic rupture. Hepatic haematoma virtually always requires surgical treatment, and otherwise the patient has hardly any chance of survival. Nevertheless, mortality is around 35%.(ABSTRACT TRUNCATED AT 250 WORDS)