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 . The condition often occurs in the 3 trimester of pregnancy (and postpartum ). It tends to present in young primigravid women.
The presentation can be variable and can include malaise, epigastric/right upper quadrant pain, and nausea/vomiting. Some may have non-specific viral-like symptoms. Hypertension and proteinuria (classic symptoms of preeclampsia) may be absent or slight .
The etiology and pathophysiology remain incompletely understood with multiple theories :
- 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 .
- disseminated intravascular coagulation: reported to occur in ~30% (range 20-40%) of patients
- hepatic infarction
- hepatic hematoma
- hepatic rupture
- placental abruption
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.
History and etymology
The condition was originally described Pritchard et al. in 1954 with the acronym later coined by L Weinstein in 1982 .