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HELLP Syndrome

  • Rare, life-threatening condition that can affect pregnant women
  • Symptoms include fatigue, abdominal pain or discomfort, headaches, nausea, vomiting
  • Treatment includes medications, blood transfusion, inducing early labor
  • Involves Maternal-Fetal Medicine, High-Risk Pregnancy Program, Obstetrics, Gynecology & Reproductive Sciences

HELLP Syndrome

Overview

HELLP syndrome is an acronym for a rare, life-threatening condition. The acronym stands for Hemolysis, Elevated Liver enzyme levels, and Low Platelet levels. The condition can cause health complications, such as hemorrhage or excessive bleeding. Up to 24% of pregnant women with HELLP syndrome and up to 34% of babies die from the condition.

HELLP is very rare. Fewer than 1% of all pregnant women experience HELLP syndrome. It’s most common during the third trimester, but it may also arise during labor and delivery. HELLP syndrome is felt by some to be a severe form of preeclampsia. Preeclampsia is a condition of pregnancy that is diagnosed by high blood pressure and includes protein in the urine. In some cases, HELLP and preeclampsia may even occur after a woman delivers her baby.

In most cases, HELLP syndrome resolves shortly after delivery. But treatments can help resolve symptoms. Because HELLP can be life-threatening, it’s important for the condition to be monitored to ensure the health of baby and mother.

What is HELLP syndrome?

HELLP syndrome (Hemolysis, Elevated Liver enzyme levels, and Low Platelet levels) is a serious health condition that can affect pregnant women. The condition may result in high blood pressure, liver dysfunction, and blood problems, including clotting difficulties.

HELLP syndrome can be seen in women who also have preeclampsia, a condition marked by high blood pressure (their blood pressure readings are 140/90 mmHg or higher on two or more occasions during the second half of pregnancy) and protein in their urine. However, 15%-20% of patients with HELLP syndrome do not have elevated blood pressure.

HELLP is an acronym that stands for the following:

  • Hemolysis, or a breakdown of red blood cells, leaving fewer red blood cells to carry oxygen to organs throughout the body
  • Elevated liver enzymes, typically a sign of liver malfunction
  • Low platelet levels, which indicate bleeding problems—platelets help with clotting
  • Low red blood cell levels, in which the body destroys red blood cells faster than it produces them, leading to fatigue and organ dysfunction

A pregnant woman with HELLP syndrome should be closely monitored by doctors to ensure the condition does not lead to injury or death of her or the baby. In most cases, the condition is managed safely.

What causes HELLP syndrome?

Doctors aren’t sure what causes HELLP syndrome. It is often associated with preeclampsia and may be linked to problems with the placenta.

What are the symptoms of HELLP syndrome?

Pregnant women with HELLP syndrome may experience:

  • Fatigue
  • Pain or discomfort in the upper-right abdomen
  • Headaches
  • Nausea
  • Vomiting
  • Dizziness
  • Jaundice
  • Swelling in the face, hands, feet, and legs
  • Fluid retention
  • Weight gain
  • Vision problems, including blurred vision
  • Nosebleeds
  • Bleeding gums
  • Hemorrhage or excessive bleeding
  • Fluid in the lungs
  • Kidney failure
  • Liver failure
  • Detached retina
  • Placental abruption (when the placenta separates from the uterine wall)
  • Seizures
  • Death

Babies may experience:

  • Low birth weight
  • Neonatal respiratory distress syndrome
  • Low platelet count, which may cause severe bleeding
  • Death

What are the risk factors for HELLP syndrome?

Women are more likely to experience HELLP syndrome if they:

  • Are age 35 or older
  • Have obesity
  • Are pregnant with twins or other multiples
  • Have diabetes
  • Have chronic high blood pressure
  • Had preeclampsia or HELLP syndrome in a previous pregnancy
  • Were previously pregnant with twins or other multiples
  • Have a family history of preeclampsia or HELLP syndrome

How is HELLP syndrome diagnosed?

A medical history, physical exam, and diagnostic testing are all tools used in the diagnosis of HELLP syndrome.

Pregnant women with a personal or family history of preeclampsia or HELLP syndrome should share that information with their doctor. They should also share if they have new abdominal pain or nosebleeds that are hard to control.

During the physical exam at a prenatal appointment, doctors typically look for swelling in the face, hands, and feet. Significant weight gain and jaundice may also be signs of HELLP syndrome. Gentle pressure may be placed on different areas of the abdomen to see if anything causes pain; tenderness in the upper right portion of the abdomen (where the liver is located) may be a sign of HELLP syndrome.

Doctors offer diagnostic tests when they suspect HELLP syndrome, which may include:

  • Blood tests, to look at the levels of red blood cells, white blood cells, platelets, and bilirubin (for jaundice), and to check for bleeding disorders
  • Urine test, to check for the presence of protein in the urine
  • Liver function test
  • An imaging test—computed tomography (CT) or magnetic resonance imaging (MRI)—to look at the liver for signs of enlargement, bleeding, or liver failure

How is HELLP syndrome treated?

Patients with HELLP syndrome are cared for in a hospital setting, where their condition can be monitored closely. The following treatments may be offered:

  • Anticonvulsant medications, such as IV magnesium sulfate, may be used to reduce the risk of seizures (eclampsia).
  • Blood pressure medications, such as labetalol, hydralazine, or nifedipine, may be used if a woman has severe preeclampsia or very high blood pressure (160/110 mmHg or higher). The medication may be given intravenously or orally.
  • Blood transfusions, if a woman with HELLP syndrome loses too much blood as a result of a hemorrhage
  • Steroids, to help increase platelet levels

Ultimately, it’s recommended that pregnant women with HELLP syndrome deliver their babies to alleviate symptoms, as the condition typically goes away after delivery. The timing of the delivery is based on the health of the mother and baby and how far along the pregnancy is.

In serious cases, a woman may need to deliver the baby immediately. If this occurs sooner than 34 weeks of pregnancy, corticosteroids may be prescribed to help the baby’s lungs develop more quickly; the baby will then be delivered 24 to 48 hours later. In extreme circumstances, when a woman is less than 34 weeks pregnant, delivery is implemented immediately, without taking time for corticosteroid administration.

Cesarean delivery may be preferable because it is quicker, but in some cases, doctors may proceed with an induction of labor with the goal of a vaginal birth.

What is the outlook for people with HELLP syndrome?

In most cases, HELLP syndrome resolves shortly after delivery. But some women experience postpartum hemorrhages that require emergency blood transfusions, which underlines the importance of monitoring the condition.

Once you have had HELLP syndrome, you are more likely to develop it in future pregnancies. You are also at increased risk of hypertension and cardiovascular disease in the future.

This article was medically reviewed by Yale Medicine Maternal-Fetal Medicine specialist Katherine Kohari, MD, who specializes in high-risk pregnancies.