HELLP Syndrome

Hemolysis, elevated liver enzymes, and low platelet count associated with severe preeclampsia.

Affects approximately 0.1% of pregnant women, and occurs almost exclusively in the third trimester.

Associated with an increased risk of maternal outcomes.

Can lead to substantial fetal and maternal morbidity and mortality.

Multisystem disease with abnormal vascular tone, vasospasm and coagulation defects.

Approximately 40% of patients have germline variants in genes that regulate complement.

Hemolysis in HELLP is mechanical and increases in bilirubin, transaminases and LDH levels are due to hemolysis rather than a liver disease.

Thrombocytopenia is consumptive.

Red blood cell destruction is microangiopathic in nature with associated peripheral smear changes with spherocytes, schistocytes and burr cells.

Can lead to fibrin deposition in hepatic sinusoids with secondary hemorrhage, hematoma and liver rupture.

Initially patients have normal coagulation studies, but DIC can be a late complication.

Platelet counts are usually below 100,000/mm3.

Complications include severe hypertension, pulmonary edema, acute renal insufficiency, liver rupture, and abruptio placenta.

When platelets counts are below 40,000mm3 maternal mortality rates are as high as 3-5%.

Liver rupture occurs in 1.8% of cases and is associated with a mortality rate of 58-70%.

Almost one third of patients present in the postpartum period and 15% of these patients do not have preeclampsia, hypertension or proteinuria.

Management is largely supportive.

Delivery of the fetus is the treatment of choice and leads to resolution of signs and symptoms in mother.

Glucocorticoids are not recommended for management.

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