Citation, DOI & article data
HELLP syndrome is a pregnancy-related condition and is an abbreviation for:
- elevated liver enzymes and
- low platelets
It is considered a severe and life-threatening form of pre-eclampsia although it can occur without co-existing pre-eclampsia.
The estimated incidence is ~0.5% (range 0.17-0.85%) of live births 9. The condition often occurs in the 3rd trimester of pregnancy (and postpartum 13). It tends to present in young primigravid women.
The presentation can be variable and can include malaise, epigastric and/or right upper quadrant pain, and nausea and vomiting. Some may have non-specific viral-like symptoms. Hypertension and proteinuria (classic symptoms of pre-eclampsia) may be absent or slight 7.
The etiology and pathophysiology remain incompletely understood with multiple theories 12:
- immune-mediated: maternal acute rejection reaction to fetal antigens
- placenta-mediated liver injury
- systemic inflammatory response syndrome in the setting of pre-eclampsia
On liver histology, there is a combination of deposited fibrin, hemorrhage, and hepatocellular necrosis surrounding portal areas 6.
General features predominantly involve hepatic sequelae:
- hepatomegaly: especially the right lobe
- hemorrhage, subcapsular hematoma, rupture
- hepatic infarction
Generally preferred over CT, an ultrasound is usually preferred to avoid ionizing radiation.
The place of CT is mainly to assess for complications. When considering a CT scan the radiologist needs to discuss with the obstetrician regarding radiation risk to fetus from radiation, versus clinical suspicion of complications in order to make a rational judgment. The CT scan may show hepatic complications as described above.
Treatment and prognosis
Management is often supportive. Patients with hepatic rupture +/- intraperitoneal bleeding require immediate surgery or selective hepatic arterial embolization.
- disseminated intravascular coagulation: reported to occur in ~30% (range 20-40%) of patients 5
- hepatic infarction
- hepatic hematoma
- hepatic rupture
- placental abruption
History and etymology
The condition was originally described by Pritchard et al. in 1954 3 with the acronym later coined by L Weinstein in 1982 4.
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