CASE HISTORY – September 2006

HELLP syndrome


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Hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome has been recognized as a complication of preeclampsia for several decades. Both the etiology and pathogenesis of HELLP syndrome remain unknown. The presence of this syndrome is associated with increased risk of adverse outcome for both mother and fetus. Right upper quadrant/epigastric pain is the most important symptom suggestive of underlying HELLP syndrome. A majority of patients will also have hypertension and proteinuria. 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. Most patients benefit from corticosteroid therapy. Mortality rate for women with HELLP syndrome is approximately 1.1 percent. Patients who have had HELLP syndrome should be counselled for increased risk of developing the syndrome in subsequent pregnancies.
A 27-year-old primigravid woman was referred to the hospital by emergency at 27 weeks of gestation because of sudden onset vaginal bleeding. Until then the pregnancy had been uneventful. On arrival at the hospital the patient was hypotonic, vaginal bleeding continued. Abruptio placentae was suspected and an emergency caesarean section was performed. A dead girl fetus of 720 gram was born. On the following day the patient had generalized epileptic seizure. During 24 hours her platelet count had fallen from 226 x 109/l to 19 x 109/l. The patient had also hemolysis (low haemoglobine, high LDH level), increased activity of liver enzymes – AST 1686 U/l, ALT 749 U/l, proteinuria. Because of the impairment of consciousness, a CT scan of the brain was performed, which showed a right frontal intracerebral hematoma with a ventricular breakthrough and a distended ventricular system. Clinically, the patient had left hemiparesis and left n. abducens paresis. She was treated in a neurological intensive care unit with intravenous dexamethasone and magnesium sulphate. She also received erythrocytes, platelets and fresh frozen plasma. Occlusive hydrocephalus was treated with ventricular drainage. During two weeks of treatment the patient`s condition improved, left hemiparesis disappeared and she was sent to the rehabilitation clinic. During the investigation of the patient`s coagulation system we found that she had activated protein C resistance, which may be associated with increased risk of preeclampsia.