Pocket ObGyn – HELLP Syndrome
See Abbreviations

Definition and Epidemiology (BMC Pregnancy Childbirth 2009;9:8)

Variant of sev preeclampsia characterized by microangiopathic hemolysis, elevated serum transaminases, & low platelet Partial HELLP includes those w/ sev preeclampsia & those w/ either “ELLP” (elevated liver transaminase & low Plts) or “EL” (elevated liver enzymes). “Partial HELLP” = “sev preeclampsia,” on a spectrum. See also Chapters 11 and 12.
5–0.9% of all pregnancies. 10–20% of those w/ eclampsia. See Chapter 18.
Increased risk for eclampsia, preterm birth, & perinatal mortality

Microangiopathic hemolysis leading to elevation of serum lactate dehydrogenase level & fragmented red bld cells on periph Same process as PEC, but more severe.
Decreased Plts due to increased

Signs & sx of preeclampsia (elevated BP, proteinuria, focal edema, HA, vision changes)
RUQ abdominal or midepigastric pain, nausea, vomiting
Intensity of sx characterized by exacerbation during the night & recovery during day (J Matern Fetal Neonatal Med 2006;19:93)
Sev complications: Spont rupture of subcapsular liver hematoma, placental abruption, DIC

Physical Exam and Diagnostic Workup/Studies (Am J Obstet Gynecol 2011;205:192)

Sibai criteria: Hemolysis on periph smear, LDH > 600 U/L, or total bilirubin

>1.2 mg/dL

Abdominal imaging (RUQ US, CT, MRI) to assess hepatic hemorrhage that may result in subcapsular hematoma ± Consider if elevation in transaminases.

Rx similar as that for sev preeclampsia (eg, antihypertensives, magnesium sulfate, deliv after steroids [for FLM] if <34 w or earlier depending on severity of dz)
Presence of HELLP ® immediate deliv due to mat death (1%) & increased mat morbidities: Bld xfusion (25%), DIC (15%), wound disruption (14%), placental abruption (9%), pulm edema (8%), renal failure (3%), & intracranial hemorrhage (1.5%) (Obstet Gynecol 2004;103:983)
Dexamethasone may improve sev thrombocytopenia, but probably does not improve outcomes (Cochrane Database Syst Rev 2010;(9):CD008148)

Increased risk for recurrence of HELLP in subseq pregnancies (5–25%); higher incid of preterm deliv, fetal-growth restriction, placental abruption & cesarean deliv in subseq deliveries w/o recurrence of HELLP

Acute Fatty Liver of Pregnancy (AFIP)

Accum of microvesicular fat a/w Mitoc dysfxn & impairment of hepatocyte fxn that can result in acute liver failure
1/10000 pregnancies
A/w Mitoc abnormalities of fatty acid oxidation from autosomal inherited mut (ie, LCHAD deficiency)
Occurs more often w/ nulliparas, male fetus, preeclampsia, & multifetal gest

Presents late in 3rd trimester – often w/ PTL or lack of fetal mvmt
Nonspecific sx including persistent nausea & vomiting, malaise, fatigue, anorexia, epigastric pain, progressive jaundice, low-grade fever
50% w/ sx concerning for preeclampsia including HTN, proteinuria, edema
If sev: Ascites, coagulopathy & spont bleeding, SOB due to pulm edema, stillbirth, hepatorenal syn, hepatic encephalopathy, renal failure

Labs: LFTs – bilirubin (>10 mg/dL), AST/ALT (typically less than 1000 U/L), CBC (hemoconcentration, leukocytosis, thrombocytopenia), coags (hypofibrinogenemia, hypoalbuminemia, hypocholesterolemia, prolonged clotting times, prolonged PT), hypoglycemia, or hyperglycemia secondary to pancreatitis
Mother should undergo testing for LCHAD; can be lifesaving for neonate/inform risk for future pregnancies
Imaging – RUQ US shows increased echogenicity; CT &/or MRI demonstrates lower liver density
Liver bx, std for confirming dx but rarely used in clinical practice, shows microvesicular steatosis

Differentiating between AFLP and HELLP
Signs & sx	AFLP (%)	HELLP (%)
HTN	50	85
Proteinuria	30–50	90–95
Fever	25–32	Absent
Jaundice	40–90	5–10
Nausea & vomiting	50–80	40
Abdominal pain	35–50	60–80
Hypoglycemia	Present	Absent
From Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol. 2007;109(4):956–966.

Supportive care: Gluc infusion, reverse coagulopathy, fluid resusc
Deliv recommended when dx confirmed; spont resolution after deliv, typically takes 1-w postpartum for hepatic dysfxn to During recovery period, 25% w/ transient diabetes insipidus & 50% w/ acute pancreatitis.
May recur in subseq pregnancies, even if no LCHAD mut in Historically w/ 70% mat mortality rate, improved w/ early dx to <10%.
Perinatal mortality 13% due to high rate of preterm deliv