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1
Question:

A 19-year-old woman, gravida 1 para 0, at 29 weeks gestation comes to the emergency department due to severe right upper quadrant pain.  The patient suddenly developed the pain this evening after dinner.  The pain was associated with acute nausea and vomiting.  She has no headaches or visual changes.  The patient has no chronic medical conditions and takes no daily medications.  Temperature is 36.7 C (98.1 F), blood pressure is 160/100 mm Hg, and pulse is 84/min.  The abdomen is tender over the right upper quadrant, but no rebound or guarding is present.  Hemoglobin is 8 g/dL.  Urinalysis shows 3+ protein.  Which of the following associated laboratory changes are most likely to be seen in this patient?

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Explanation:

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HELLP syndrome

Pathogenesis

  • Abnormal placental development
  • Maternal endothelial dysfunction

Clinical
features

  • Hypertension
  • Right upper quadrant pain
  • Nausea/vomiting
  • Possible headache or vision changes

Laboratory
findings

  • Microangiopathic hemolytic anemia
  • Thrombocytopenia
  • Elevated liver enzymes & bilirubin
  • Proteinuria

HELLP = Hemolysis, Elevated Liver enzymes, Low Platelet count.

This patient at >20 weeks gestation with hypertension, proteinuria, and right upper quadrant pain likely has HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome.  HELLP syndrome is most often diagnosed in patients with preeclampsia, which likely occurs due to abnormal placental development (insufficient cytotrophoblast invasion in early pregnancy) and subsequent maternal endothelial dysfunction.

Unlike preeclampsia, HELLP syndrome is associated with excessive activation of the coagulation cascade, likely because of severe endothelial injury.  This causes widespread thrombi formation in the microvasculature (ie, microangiopathic thrombotic microangiopathy).  As platelets are consumed and numerous thrombi form, patients develop severe thrombocytopenia and mechanical shearing of circulating red blood cells, resulting in hemolytic anemia.

A hallmark of both preeclampsia and HELLP syndrome is hepatocellular injury (due to impaired hepatic perfusion), which leads to increased alanine aminotransferase release, increased indirect bilirubin accumulation (exacerbated by the ongoing hemolysis), and clinical features of right upper quadrant pain with nausea/vomiting.  More serious complications include liver necrosis, hepatic hemorrhage, subcapsular hematoma, and liver rupture.

Educational objective:
HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome is a thrombotic microangiopathic disorder that causes microangiopathic hemolytic anemia (eg, increased indirect bilirubin levels), thrombocytopenia, and hepatocellular injury (eg, increased alanine aminotransferase).  Patients may have hypertension and right upper quadrant pain with nausea/vomiting.