HELLP Syndrome: Etiology and Pathogenesis (HELLP Syndrome)

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

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HELLP Syndrome Characteristics (HELLP Syndrome)

HELLP Syndrome

HELLP Syndrome characterized by hemolysis, elevated liver enzyme levels and a low platelet count, is an obstetric complication that is frequently misdiagnosed at initial presentation. Many investigators consider the HELLP Syndrome to be a variant of preeclampsia, but it may be a separate entity. The pathogenesis of HELLP Syndrome remains unclear. Early diagnosis is critical because the morbidity and mortality rates associated with the HELLP Syndrome have been reported to be as high as 25 percent. Platelet count appears to be the most reliable indicator of the presence of HELLP Syndrome. The D-dimer test may be a useful tool for the early identification of patients with preeclampsia who may develop severe HELLP Syndrome. The mainstay of therapy is supportive management, including seizure prophylaxis and blood pressure control in patients with hypertension. Women remote from term should be considered for conservative management, whereas those at term should be delivered. Some patients require transfusion of blood products, and most benefit from corticosteroid therapy. Rarely, patients with refractory HELLP Syndrome require plasmapheresis. (Am Fam Physician 1999;60:829-39.)

The acronym HELLP was coined in 1982 to describe a syndrome consisting of hemolysis, elevated liver enzyme levels and low platelet count.1 The syndrome has been considered a variant of preeclampsia, but it can occur on its own or in association with preeclampsia. Pregnancy-induced hypertension, preeclampsia and HELLP Syndrome are related and overlap in their presentations. Because of the serious associated morbidity and mortality, family physicians who provide maternity care need to be aware of HELLP Syndrome so that they can identify it early.

HELLP Syndrome

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HELLP Syndrome and Your Pregnancy (HELLP Syndrome)

HELLP Syndrome

How is HELLP syndrome treated?
The main treatment for HELLP is to deliver your baby. This may have to be done before your due date. Most women with this illness start to get better a couple of days after their babies are born. If you aren't too sick, your doctor may wait a few days before delivering your baby. You may have to take a steroid. This medicine helps both you and your baby. If you have bleeding, you may need blood transfusions or other treatments in the hospital. Some women with HELLP syndrome get very sick. Rarely, this illness is fatal.

What can I do to prevent HELLP syndrome?
There is no way to prevent this illness. The best thing you can do is see your doctor regularly and tell your doctor about your symptoms at every prenatal visit. If you have HELLP syndrome during one pregnancy, you can have it again during your next pregnancy. The illness is usually less severe the second time.

HELLP Syndrome

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