What Is HELLP Syndrome?

The conditions that are part of HELLP syndrome are in its name, which stands for hemolysis, elevated liver enzymes, and low platelet count. Though the specific cause of HELLP syndrome is not known, it is thought to be associated with preeclampsia, a condition involving high blood pressure during later stages of pregnancy. HELLP syndrome typically occurs in the last trimester, but can also start after delivery.

Signs and Symptoms of HELLP Syndrome

HELLP syndrome itself is a group of these three symptoms in pregnant individuals:

• H: Hemolysis (the breakdown of red blood cells)
• EL: Elevated liver enzymes
• LP: Low platelet count

More specific signs and symptoms of HELLP syndrome include:

• Fatigue or feeling unwell
• Fluid retention and excess weight gain
• Headache
• Nausea and vomiting that continues to get worse
• Pain in the upper right or mid part of the abdomen
• Blurry vision
• Jaundice
• Seizures or convulsions (which are also rare)

Diagnosis

Prior to being diagnosed with HELLP syndrome, many people will first notice abdominal pain—which may be in the right upper quadrant or in the epigastric region—along with nausea and vomiting, sometimes accompanied by malaise, headache, and visual disturbance. These symptoms are typically what causes them to seek medical treatment or evaluation for what may be later diagnosed as HELLP syndrome.

At the doctor's office or hospital, the healthcare provider will perform a physical exam, and in the process may discover:

• Abdominal tenderness, especially in the right upper side or in the mid-chest region above the umbilicus (belly button)
• High blood pressure
• Swelling in the legs

Lab work will also be involved to check on whether the pregnant person has:

• High levels of liver enzymes
• A low platelet count
• Excessive protein in their urine

If a liver function test indicates potential problems with the organ, the healthcare professional may order a CT scan to see whether there is bleeding into the liver. Additionally, they'll also perform tests on the fetus, including a fetal non-stress test, and/or an ultrasound, among others.

Treatment

Given the severity of HELLP syndrome, the main goal of the treatment is to deliver the baby as safely and soon as possible—even in cases where they might be premature. One of the challenges of treating HELLP syndrome is that the pregnant person's symptoms can get far more serious in a very short period of time—especially any problems related to the liver.

When a delivery is required, the medical professional will either give the pregnant person medication to induce labor or perform a cesarean section. The pregnant person may also receive:

• A blood transfusion if bleeding problems become severe
• Corticosteroid medicines to help the baby's lungs develop faster
• Medicines to treat high blood pressure
• Magnesium sulfate infusion to prevent seizures

Even with a medical team's best efforts to deliver the baby as quickly and safely as possible, HELLP syndrome could result in a number of complications for both the pregnant person and the fetus.

Complications for the pregnant person can include:

• Eclampsia
• Placental abruption
• Cesarean section
• DIC
• Recurrent thrombosis
• Liver rupture
• Cerebral infarction
• Cerebral hemorrhage
• Pulmonary/cerebral edema
• Cardiovascular instability
• Acute renal failure
• Infection/sepsis
• Death

Complications for the fetus can include:

• Perinatal death
• Intrauterine growth restriction (IUGR)
• Preterm delivery
• Neonatal thrombocytopenia
• Respiratory distress syndrome

Risk Factors

Although the cause of HELLP syndrome is unknown, there are some known risk factors for developing the condition. The most common is having preeclampsia, or pregnancy-induced high blood pressure, although not every person who develops HELLP syndrome has preeclampsia.

Other risk factors that may increase a person's chance of being diagnosed with HELLP syndrome include:

• Having a previous pregnancy with HELLP syndrome
• Having preeclampsia or pregnancy-induced hypertension
• Being over the age of 25
• Multiparous (given birth two or more times)
• LCHAD deficiency in the fetus (though only in approximately 2% of HELLP syndrome cases)

Additionally, genetic factors—in both the pregnant person and fetus—may also have an impact on whether the pregnant individual develops HELLP syndrome. And while scientists are progressively learning more about HELLP syndrome, many think that it is likely multifactorial, meaning that a combination of interacting genetic and environmental factors causes HELLP syndrome.

Currently, it is thought that there is no single gene that's responsible for the condition. And, even though some people may have a genetic predisposition to developing conditions like preeclampsia and HELLP syndrome, many who have the same genetic risk factors never develop the pregnancy condition.

A Word From Verywell

There are so many unknowns involved with pregnancy. Even if someone does everything "by the book," there are still some aspects of the gestation process that are beyond our control—including developing HELLP syndrome.

The good news is that when HELLP syndrome is diagnosed early, there is usually a positive outcome for both the pregnant person and the child. This serves as yet another example of why attending regular prenatal checkups and testing appointments is so important.

Catching something like HELLP syndrome sooner rather than later can make a significant difference, and even save lives.