HELLP syndrome is a pregnancy-related condition and is an abbreviation for:
haemolysis
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.
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Epidemiology
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.
Clinical presentation
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.
Pathology
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
Microscopic appearance
On liver histology, there is a combination of deposited fibrin, hemorrhage, and hepatocellular necrosis surrounding portal areas 6.
Radiographic features
General features predominantly involve hepatic sequelae:
hepatomegaly: especially the right lobe
hemorrhage, subcapsular hematoma, rupture
Ultrasound
Ultrasound is usually preferred over CT to avoid ionizing radiation.
CT
The place of CT is mainly to assess for complications. When considering a CT scan the radiologist needs to discuss with the obstetrician the radiation risk to the fetus versus the 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.
Complications
disseminated intravascular coagulation: reported to occur in ~30% (range 20-40%) of patients 5
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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