HELLP Syndrome: Hemolysis,

Elevated Liver Enzymes, and Low

Platelets
Nneoma Nwachuku, PGY-1
Surgery Intensive Care Unit
February 27, 2014
Introduction
HELLP Syndrome characterized by:
Hemolysis
Elevated Liver Enzymes
Low platelet count
Occurs in 0.5 0.9% of pregnancies
Peak incidence is weeks 27-37 weeks gestation
About one third occur in the post-partum period

HELLP and Pre-eclampsia
Pre-eclampsia hypertension in pregnancy,
returning to normal after the post-partum
period, with proteinuria.

Distinct entity vs Severe manifestation of pre-
eclampsia
HELLP found in 10-20% of cases
Risk Factors
Previous history of pre-eclampsia or HELLP
syndrome
Family history
Antiphospholipid-antibody syndrome
Risk factors seen in pre-eclampsia but NOT
HELLP
Obesity
Nulliparity

Etiology
Abnormal placentation in the
first trimester

Placental dysfunction leads to
endothelial dysfunction end
organ damage

Immune reaction/ rejection of
fetal allograft?
Increase in HLA-DR in
maternal circulation in
HELLP not seen in
preeclampsia or controls

Etiology
Microangiopathic hemolytic anemia results from
damaged endothelium
Hepatic injury
Placental FasL injurious to hepatocytes
Microangiopathy poor portal blood flow
Thrombocytopenia
Damaged endothelium activated von
Willebrand factor decreased ADAMT13 in
HELLP
Elevated vWF leads to thrombocytopenia

Other Signs and Symptoms
Elevated blood pressures
Abdominal pain right upper quadrant or
epigastric pain
Nausea/vomiting
Headache
Visual symptoms
Excessive weight gain
Generalized edema
Diagnosis
Microangiopathic hemolytic anemia
Peripheral smear: schistocytes, Burr cells
Elevated indirect bilirubin
Low serum haptoglobin
Anemia and increased LDH
Serum AST 70 IU/L, total bilirubin 1.2
mg/dL
AST > 40IU/L and/or ALT > 40IU/L
Platelet count 100,000 cells/L
Treatment
Delivery, especially if:
Greater than 34 weeks gestation
Prior to this, ensure corticosteroid administration
for fetal lung maturity
Severe complications
Non-reassuring fetal status

Anti-hypertensive medications
Treatment
Magnesium sulfate
Eclampsia prophylaxis
Fetal neuroprotection if 24-32 weeks gestation

Corticosteroids?
Improvement in thrombocytopenia for most
severe category; shorter hospital duration
No improvement in maternal morbidity
Complications
Bleeding
Platelet transfusion if under 20,000 cells/L
Hepatic hematoma formation and rupture
Volume replacement
Percutaneous embolization of the hepatic arteries
If persistent bleeding, hemodynamically unstable,
continued expansion surgery

Other complications DIC, abruptio placentae, acute
renal failure, pulmonary edema, Retinal detachment,
chronic hypertension
Fetal Complications
High perinatal mortality

Premature delivery

Intrauterine growth restriction
Recurrence and Prevention
Risk of recurrence is 2 6 %
Twenty percent develop pre-eclampsia in
subsequent pregnancies

Prevention: Low-dose aspirin for pre-eclampsia
prevention
Selected References
Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis,
elevated liver enzymes, and low platelet count (HELLP): a review.
Eur J Obstet Gynecol Reprod Biol. 2013 Feb;166(2):117-23.

Fonseca JE, Mendez F, Catano C, Arias F: Dexamethasone treatment
does not improve the outcome of women with HELLP syndrome: a
double-blind, placebo-controlled, randomized clinical trial. Am J
Obstet Gynecol 2005, 193:1591-1598.

Jebbink J, Wolters A, Fernando F, et al. Molecular genetics of
preeclampsia and HELLP syndrome - a review. Biochim Biophys
Acta 2012; 1822:1960.

Sibai BM: The HELLP syndrome (hemolysis, elevated liver enzymes,
and low platelets): much ado about nothing? Am J Obstet Gynecol
1990, 162:311-316.