Abstract
OBJECTIVE: HELLP syndrome is a severe preeclampsia spectrum disorder diagnosed when laboratory evidence of hemolysis, liver dysfunction and thrombocytopenia are present. The presence of epigastric pain with laboratory criteria for HELLP syndrome may indicate higher maternal-fetal risk. Thus we explored maternal outcomes in 42 pregnant/postpartum women that had HELLP syndrome by laboratory criteria in addition to sudden severe epigastric pain. METHODS: A database was constructed from the medical files of all patients with medical/hypertensive disorders evaluated by the first author from 1986-2015 for medicolegal purposes. All patient files of women who presented to their physicians with a diagnosis of presumptive HELLP syndrome were examined. RESULTS: Fifty-eight pregnant/postpartum women fulfilled study inclusion criteria; they presented to physicians for evaluation usually in non-tertiary care hospital settings. Clinical presentation and care including lab data and details of pregnancy outcome were evaluated. A correct diagnosis of HELLP syndrome was verified for 42 women (72.4%); others were determined to have AFLP-Acute Fatty Liver of Pregnancy (n = 8) or TTP-aHUS-Thrombotic thrombocytopenic purpura-adult/atypical hemolytic uremic syndrome (n = 8). All 42 HELLP women (100%) in our cohort had severe epigastric pain. Treatment modalities for maternal HELLP syndrome included magnesium sulfate (n = 35, 83%), antihypertensives (n = 17, 40%); corticosteroids (CORT) for HELLP were not utilized. Major maternal morbidity (21 strokes, 9 liver ruptures/hematomas) affected 41 HELLP women (98%); 22 died, 7 were permanently disabled. CONCLUSION: HELLP syndrome patients presenting with sudden, severe epigastric pain in this highly selected cohort experienced very high maternal morbidity and mortality. Laboratory evidence of HELLP syndrome in association with epigastric pain is a dangerous combination that portends great danger to safe motherhood. We speculate that the absence of CORT use in this cohort contributed to poor maternal outcome (word count = 280 as modified for reviewers).
Highlights
Four decades ago, Dr Louis Weinstein proposed the acronym “HELLP syndrome” to characterize a special group of pregnant or postpartum patients with a severe preeclampsia-spectrum disorder characterized by laboratory evidence of hemolysis, elevated liver enzymes and moderate to severe thrombocytopenia [1]; epigastric pain was not a criterion for the diagnosis nor had it become a criterion in the intervening years (ACOG 2013, 2020)
Based on the very high major maternal morbidity and maternal mortality experienced by the 42 women with HELLP syndrome in this cohort, all of whom presented with severe epigastric pain, it seems likely that the presence of this significant symptom identifies a subset of HELLP patients who are at much greater risk of morbidity and mortality compared to asymptomatic HELLP patients
HELLP Syndrome is an obstetric emergency which poses a significant threat to safe motherhood [5]
Summary
Dr Louis Weinstein proposed the acronym “HELLP syndrome” to characterize a special group of pregnant or postpartum patients with a severe preeclampsia-spectrum disorder characterized by laboratory evidence of hemolysis, elevated liver enzymes and moderate to severe thrombocytopenia [1]; epigastric pain was not a criterion for the diagnosis nor had it become a criterion in the intervening years (ACOG 2013, 2020). Almost four decades after the acronym HELLP was formalized, the most recent meta-analysis of worldwide prospective trials comparing potent corticosteroids (CORT) versus matched controls as principal therapy for HELLP was able to aggregate only 22 qualifying studies [11] [12]. These were not separated for analysis according to the presence or absence of severe epigastric pain in the study subjects
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