HELLP syndrome

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.

Epidemiology

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.

Clinical presentation

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 .

Pathology

The etiology and pathophysiology remain incompletely understood with multiple theories :

Histology

On liver histology, there is a combination of deposited fibrin, hemorrhage, and hepatocellular necrosis surrounding portal areas .

Complications

Radiographic features

General features predominantly involve hepatic sequelae:

Ultrasound

Generally preferred over CT, an ultrasound is usually preferred 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 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 .