HELLP syndrome: an atypical presentation
Caroline L. Stella, MD; Khurram M. Malik, MD; Baha M. Sibai, MD
ELLP (hemolysis, elevated liver enzymes and low platelets) syndrome accounts for 0.2-0.6% of all pregnancies. This condition comprises 10% of the cases of severe preeclampsia and approximately 50% of eclampsia cases. Preeclampsia, eclampsia, and HELLPsyndrome afﬂict many women and result in a large percentage of maternal and perinatal morbidities. It is important that clinicians make an accurate diagnosis of these conditions, to prevent any adverse outcome. In general, most women will have a classical presentation of preeclampsia (hypertension and proteinuria) at 20 weeks gestation. However, recent studies suggested that some women will developpreeclampsia without the manifestations of classical symptoms. Atypical cases are those that may develop prior to 20 weeks, or present with signs and symptoms of preeclampsia without hypertension or proteinuria, or those cases with subtle abnormalities in laboratory ﬁndings.
HELLP syndrome without proteinuria or hypertension is rare. We present the case of a 33-year-old primigravid who initiallywas diagnosed with pulmonary embolism. The patient was readmitted with a diagnosis of subcapsular hematoma and eventually diagnosed with HELLP syndrome with a sequela of hepatic rupture. Key words: atypical presentation, HELLP syndrome, hepatic rupture
C ASE R EPORT
We present the case of a 33-year-old primigravida at 31 weeks gestation with a chief complaint of sudden shortness of breath.The patient also complained of an intense, sharp, stabbing midsternal chest pain (intensity 9/10) radiating to the back. The patient indicated that the pain intensiﬁed with deep breathing or when lying ﬂat on her back/stomach. The patient also has a history of smoking, which continued during pregnancy. In the emergency room, the patient presented with a blood pressure of
From the Division ofMaternal-Fetal Medicine, University of Cincinnati College of Medicine, Cincinnati, OH.
Received Sept. 21, 2007; revised Dec. 11, 2007; accepted Dec. 21, 2007. Reprints not available from the authors. 0002-9378/free © 2008 Mosby, Inc. All rights reserved. doi: 10.1016/j.ajog.2007.12.034
122/82 mmHg, a pulse of 84, and a respiratory rate of 20. Physical examination was pertinent with reproduciblemidsternal chest wall tenderness. Other ﬁndings included hemoglobin of 13.7 g/dL, hematocrit of 41%, platelet count of 179,000 mm3, creatinine of 0.8 mg/dL, aspartate aminotransferase (AST) of 94 IU/L, alanine transaminase (ALT) of 153 IU/L, and total bilirubin of 0.5 mg/dL. Subsequently, urinalysis was ordered and noted to be negative for protein. A helical chest CT scan showed decreasedattenuation in the distal branches of both right and left lower lobe pulmonary arteries, suspicious of bilateral pulmonary emboli. Venous Doppler studies of the lower extremities, however, were negative. Fetal ultrasound revealed an estimated fetal weight of 10th percentile and a biophysical proﬁle of 8/8. The perinatologist consulted recommended corticosteroids for fetal lung maturity and twice weeklyfollow-up. The diagnosis at this time was pulmonary embolism. The patient was initially anticoagulated with intravenous heparin (therapeutic dose) and was subsequently maintained on low molecular weight heparin at 10,000 units every 12 hours. The following day, the patient’s platelet count subsequently dropped to 100,000 mm3. This was attributed to heparin use. Platelet count subsequently increased to139,000 mm3 (Table). The patient was discharged home 2 days later on low molecular weight heparin at 10,000 units every 12 hours. The patient returned to the emergency room 5 days later with a chief complaint of acute epigastric pain radiating to the back. The patient indicated that the pain began 6 hours prior and with moderate
to severe intensity, duration of 6 hours and intensity of 8/10....