Here’s a disturbing fact: If it looks like HELLP syndrome, and impairs the patient like HELLP syndrome, it isn’t necessarily HELLP syndrome. A plethora of diagnostic criteria from different investigators over the years has confused the issue of what constitutes this syndrome—not to mention how to manage it.
A management issue has also attracted recent attention: use of corticosteroids either antepartum to enhance maternal status so that epidural anesthesia can be administered, or postpartum to improve platelets. Such improvements are only transient, however, and we lack definitive data on the benefits.
One thing is certain, however. The combination of hemolysis, liver dysfunction or injury, and platelet consumption in women with preeclampsia makes adverse maternal and perinatal outcomes more likely and leaves no room for expectant management.
HELLP syndrome also has become a major issue in litigation against obstetricians and medical and surgical consultants. Lawsuits usually allege misdiagnosed preeclampsia, delayed delivery, or improper recognition and management of complications.
Pinning HELLP Down
One of the best tools to identify HELLP syndrome is a healthy dose of suspicion, since it can affect any pregnant woman at any time: antepartum, intrapartum, or within 1 week postpartum. Approximately 72% of cases are diagnosed before delivery, and the rest are diagnosed during the first week postpartum.
Weinstein noted that the signs and symptoms of HELLP syndrome can occur without clinical evidence of severe preeclampsia (severe hypertension and/or severe proteinuria). Indeed, he reported that hypertension can be mild or absent in most patients with HELLP, and proteinuria can be mild.
Weinstein coined the term HELLP syndrome in 1982 to describe these abnormalities in women with preeclampsia:
- H = hemolysis
- EL = elevated liver enzymes
- LP = low platelets
Another obstacle to early detection: Patients may have nonspecific signs and symptoms, none of which are diagnostic of classical preeclampsia.
However, HELLP syndrome is most common in women who have already been diagnosed with gestational hypertension and/or preeclampsia.
HELLP is more likely with severe hypertension
Overall, the incidence of HELLP syndrome in women with gestational hypertension/preeclampsia increases with the severity of the condition. HELLP syndrome also is more likely in women with early-onset hypertension/preeclampsia (before 34 weeks’ gestation).
Making The Diagnosis
HELLP syndrome is diagnosed when all 3 of the following are present:
- Hemolysis, defined as the presence of microangiopathic hemolytic anemia. This is the hallmark of the triad.
- Elevated liver enzymes (either aspartate aminotransferase [AST] or alanine aminotransferase [ALT]). This component signifies liver cell ischemia and/or necrosis.
- Low platelet count (<100,000/mm3). TABLE 1 summarizes the laboratory criteria for the diagnosis.
When to begin testing
In women with new-onset hypertension, order a complete blood count with platelets and liver enzyme analysis at the time of diagnosis and serially thereafter. The frequency of these tests depends on the initial test results, severity of disease, and onset of symptoms.
In women without hypertension, I recommend obtaining the same blood tests at the onset of any of the signs and symptoms listed in TABLE 2.
Conditions that heighten the risk of HELLP
Assessing test results
Clinicians should be familiar with the upper limit for liverenzyme tests in their laboratory. I suggest a cutoff more than twice the upper limit for a particular test.
Also keep in mind that these parameters are dynamic; some women will meet only some of the criteria early in the disease process. Moreover, maternal complications are substantially higher when all 3 components are present than when only 1 or 2 are present.