HELLP Syndrome
The pathogenesis of HELLP Syndrome is not well understood. The findings of this multisystem disease are attributed to abnormal vascular tone, vasospasm and coagulation defects.2 To date, no common precipitating factor has been found. The HELLP Syndrome seems to be the final manifestation of some insult that leads to microvascular endothelial damage and intravascular platelet activation. With platelet activation, thromboxane A and serotonin are released, causing vasospasm, platelet agglutination and aggregation, and further endothelial damage.2 Thus begins a cascade that is only terminated with delivery.
The hemolysis in HELLP Syndrome is a microangiopathic hemolytic anemia. Red blood cells become fragmented as they pass through small blood vessels with endothelial damage and fibrin deposits. The peripheral smear may reveal spherocytes, schistocytes, triangular cells and burr cells. The elevated liver enzyme levels in the syndrome are thought to be secondary to obstruction of hepatic blood flow by fibrin deposits in the sinusoids. This obstruction leads to periportal necrosis and, in severe cases, intrahepatic hemorrhage, subcapsular hematoma formation or hepatic rupture. The thrombocytopenia has been attributed to increased consumption and/or destruction of platelets.
Although some investigators speculate that disseminated intravascular coagulopathy (DIC) is the primary process in HELLP Syndrome, most patients show no abnormalities on coagulation studies. Patients who develop DIC generally do so in the setting of well-developed HELLP syndrome. All patients with HELLP Syndrome may have an underlying coagulopathy that is usually undetectable.
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HELLP Syndrome
OTHER RELATED INFORMATION
The pathogenesis of HELLP Syndrome is not well understood. The findings of this multisystem disease are attributed to abnormal vascular tone, vasospasm and coagulation defects.2 To date, no common precipitating factor has been found. The HELLP Syndrome seems to be the final manifestation of some insult that leads to microvascular endothelial damage and intravascular platelet activation. With platelet activation, thromboxane A and serotonin are released, causing vasospasm, platelet agglutination and aggregation, and further endothelial damage.2 Thus begins a cascade that is only terminated with delivery.
The hemolysis in HELLP Syndrome is a microangiopathic hemolytic anemia. Red blood cells become fragmented as they pass through small blood vessels with endothelial damage and fibrin deposits. The peripheral smear may reveal spherocytes, schistocytes, triangular cells and burr cells. The elevated liver enzyme levels in the syndrome are thought to be secondary to obstruction of hepatic blood flow by fibrin deposits in the sinusoids. This obstruction leads to periportal necrosis and, in severe cases, intrahepatic hemorrhage, subcapsular hematoma formation or hepatic rupture. The thrombocytopenia has been attributed to increased consumption and/or destruction of platelets.
Although some investigators speculate that disseminated intravascular coagulopathy (DIC) is the primary process in HELLP Syndrome, most patients show no abnormalities on coagulation studies. Patients who develop DIC generally do so in the setting of well-developed HELLP syndrome. All patients with HELLP Syndrome may have an underlying coagulopathy that is usually undetectable.
Source
HELLP Syndrome
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