Tiny Tip: HELLP Syndrome

If you train in a tertiary care center with obstetrical triage, you may not assess many pregnant women beyond the first trimester of pregnancy. However, in community emergency departments without a primary obstetrics triage department, you will often encounter pregnancy-induced hypertension (PIH; systolic pressure 140 mmHg, or diastolic pressure 90 mmHg), a common complication occurring in 7-9% of pregnancies. HELLP syndrome is an important subset of PIH that comes with its own built-in mnemonic to help remember its features[1].

The Mnemonic: HELLP Syndrome

HELPP syndrome is comprised four component parts [2]:

Haemolysis

Elevated

Liver enzymes

Low

Platelets

Applying the Mnemonic

If you encounter a pregnant woman with hypertension in the emergency department, be on the look out for signs of haemolysis (the peripheral blood smear and elevated LDH are your clues), elevated AST and ALT, and thrombocytopaenia. Clinical symptoms of acute hypertensive disease, including epigastric or right upper quadrant pain, headache, paraesthesias, and blurred vision may contribute to the clinical picture. Prolonged prothrombin time and low fibrinogen level are indicators of severity, and uric acid is used as an adjunct used for diagnosis in some centers.

The only cure for HELLP syndrome is delivery, but patients may be temporized with fluids, antihypertensive agents, and platelet transfusion, depending on the clinical picture. Consult obstetrics early.

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Review by an Attending

by Teresa Chan MD FRCPC

Thank you very much for your piece. I enjoyed this review, and thought it represented the topic well. One thing I would suggest is that it is critical to consider the key differences between HELLP and other PIH-related entities.

Of note, within this recent update of the Society of Obstetricians and Gynecologists of Canada (SOGC) guidelines they state that:

“Definitions of severe preeclampsia vary, but most include multi-organ involvement. We modified our definition of severe preeclampsia to preeclampsia associated with one or more severe complications. Severe preeclampsia now warrants delivery regardless of gestational age. Our definition excludes heavy proteinuria and HELLP syndrome, which are not absolute indications for delivery, and includes stroke and pulmonary edema, which are leading causes of maternal death in preeclampsia.” – Pg 422, SOGC guidelines [2]

For Canadian learners, it is best to understand that there is controversy about definitions of severe preeclampsia internationally. The SOGC, however, is the main body that sets the national standards in Canada. Hence, it is worthwhile being familiar with what our Canadian experts have defined as Severe Preeclampsia – and with the 2014 guideline, it is noted that they excluded heavy proteinuria and HELLP syndrome from the definition for preeclampsia because these two diagnoses are not absolute indications for delivery.

That said, on page 421 they state that:

“HELLP syndrome is represented by its component parts (hemolysis, elevated liver enzymes, and low platelets), to which we react to by initiating delivery.”

This statement implies that usually a patient presenting with HELLP may very well require emergent delivery. As such, it is imperative, as you have stated, to alert obstetrics early and involve them in the decision making for this case.

One more note, the 125^th recommendation urges practitioners to bear in mind the delay between ordering and receiving platelets (Level III-B) – and hence, if you are a first line practitioner with a patient whose platelets are low, it will be important to alert the obstetrical team and perhaps even begin ordering (+/- transfusing) platelets if the patient has HELLP syndrome (and particularly if the platelet count is < 20 x 10⁹/L as detailed in recommendation 126 (Level III-B). [2, p. 432]).

Addendum (Aug 14, 2014 12:03pm): Of note, the pregnancy-induced hypertension pathologies can occur in women in the postpartum period. At the 6 week postpartum check up it is important to consider this critical diagnosis. (Thanks to Taylor Zhou for requesting this amendment as a post-publication peer review.)

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References

1. Nabhan, A., & Elsedawy, M. (2011). Tight control of mild-moderate pre-existing or non-proteinuric gestational hypertension. The Cochrane Collaboration. Retrieved from http://onlinelibrary.wiley.com/store/10.1002/14651858.CD006907.pub2/asset/CD006907.pdf?v=1&t=hxvw2the&s=71494c9ecf58b33d930f62ee724ce5a38fff3029

2. Rey, E., Pels, A., von Dadelszen, P., Helewa, M., & Magee, L. (2014, May). Diagnosis, evaluation, and management of the hypertensive disorders of pregnancy: Executive summary. Retrieved from http://sogc.org/wp-content/uploads/2014/05/gui307CPG1405Erev.pd PMID: 21735406

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