National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

HELLP syndrome

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Other Names:
Hemolysis, Elevated Liver Enzymes, Lowered Platelets
Categories:
HELLP syndrome is a life-threatening condition that can potentially complicate pregnancy.[1] It is named for 3 features of the condition: Hemolysis, Elevated Liver enzyme levels, and Low Platelet levels. It typically occurs in the last 3 months of pregnancy (the third trimester) but can also start soon after delivery.[2] A wide range of non-specific symptoms may be present in women with HELLP syndrome. Symptoms may include fatigue; malaise; fluid retention and excess weight gain; headache; nausea and vomiting; pain in the upper right or middle of the abdomen; blurry vision; and rarely, nosebleed or seizures.[1][3] The cause of HELLP syndrome is not known, but certain risk factors have been associated with the condition.[1] It is most common in women with preeclampsia or eclampsia.[3] If not diagnosed and treated quickly, HELLP syndrome can lead to serious complications for the mother and baby.[2] The main treatment is to deliver the baby as soon as possible, even if premature, if there is distress of the mother or the baby.[3][1] Treatment may also include medications needed for the mother or baby, and blood transfusion for severe bleeding problems.[3]
Last updated: 6/21/2018
Women with HELLP syndrome may feel tired, have pain in the upper right part of the belly, have bad headaches, and nausea or vomiting. They may also experience swelling, especially of the face and hands.[2][3] Vision problems may also be observed.[3] Rarely, they may have bleeding from the gums or other places.[2] 

Because healthy pregnant women may also have these symptoms late in pregnancy, it may be hard to know for sure if they are attributable to HELLP syndrome. A doctor may order blood tests to determine if these symptoms are the result of HELLP syndrome.[2]
Last updated: 2/23/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance 0000006
Eclampsia 0100601
Edema
Fluid retention
Water retention
[ more ] 		0000969
Elevated hepatic transaminase
High liver enzymes
0002910
Hypertension 0000822
Intrauterine growth retardation
Prenatal growth deficiency
Prenatal growth retardation
[ more ] 		0001511
Maternal hypertension 0008071
Preeclampsia 0100602
Proteinuria
High urine protein levels
Protein in urine
[ more ] 		0000093
Seizure 0001250
Thrombocytopenia
Low platelet count
0001873
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Last updated: 7/1/2020
The cause of HELLP syndrome is unclear.[2][4] Although it is more common in women who have preeclampsia or pregnancy induced hypertension (high blood pressure during pregnancy), some women develop HELLP syndrome without showing signs of these conditions.[3][5]

The following risk factors may increase a woman's chance to develop HELLP syndrome:[2][5]

  • Having a previous pregnancy with HELLP syndrome
  • Having preeclampsia or pregnancy induced hypertension
  • Being over age 25
  • Being Caucasian
  • Multiparous (given birth 2 or more times)
In less than 2 percent of women with HELLP syndrome, the underlying cause appears to be related to LCHAD deficiency in the fetus.[4]
Last updated: 5/2/2016
A variety of genetic factors (both in the mother and fetus) have been found to play a role in the development of preeclampsia and HELLP syndrome.[6] However, the condition is likely multifactorial. This means that several genetic and environmental factors likely interact to cause HELLP syndrome, and no one gene is thought to be responsible for the condition.

Some women may have a genetic predisposition to developing preeclampsia and related conditions, such as HELLP syndrome. This means that certain genetic factors increase a woman's risk to develop HELLP syndrome. However, many women with a genetic predisposition will never develop HELLP syndrome.
Last updated: 5/2/2016
The long-term outlook (prognosis) for mothers with HELLP syndrome varies from woman to woman, but HELLP syndrome is rarely fatal. The outcome for mothers with HELLP is generally good, but serious complications are relatively common. In most cases, after delivery, a mother's signs and symptoms begin to improve within 2 days. However, some women experience complications. The risk of serious complications generally is higher for women with severe symptoms and for those with more severe laboratory abnormalities. Examples of complications include disseminated intravascular coagulation (DIC), placental abruption, acute kidney failure, and pulmonary edema. However, these complications are related. For example, placental abruption can cause DIC, which then may cause acute kidney failure; acute kidney failure may lead to pulmonary edema. Of note, HELLP syndrome with or without kidney failure does not affect long-term kidney function. Other complications that have been reported include adult respiratory distress syndrome, sepsis, and stroke.[7]

The prognosis for a fetus or newborn of a mother with HELLP syndrome is thought to largely depend on the gestational age at delivery and birth weight (rather than the fact that HELLP syndrome was present, or the severity of signs and symptoms). This means that in many cases, the longer the pregnancy continues, the better the chance for survival and good health for the baby. Premature birth is common in mothers with HELLP syndrome.[7] The rate of preterm delivery is 70%, with 15% occurring before 28 weeks of gestation.[8] This is the leading cause of fetal or newborn death, with the overall risk of death ranging from 7 to 20 percent.[7][8]

The mother's laboratory abnormalities are not thought to be related to the chance of survival of a fetus or newborn. Additionally, HELLP syndrome does not affect liver function in the fetus or newborn.[7][8] Unfortunately, there is very limited data available regarding the long-term follow-up of children born to mothers with HELLP syndrome.
Last updated: 5/23/2018

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
  • ClinicalTrials.gov lists trials that are related to HELLP syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with HELLP syndrome. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

    Registries for HELLP syndrome:
    The Preeclampsia Registry
     

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The American Pregnancy Association provides information about HELLP syndrome.
  • FamilyDoctor.org provides additional information about HELLP syndrome. Click on the link above to access this information.
  • March of Dimes has information on HELLP syndrome.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The Merck Manual provides information on this condition for patients and caregivers. 
  • The Preeclampsia Foundation provides information about HELLP syndrome. The information includes the signs and symptoms of the syndrome, risks, prognosis, and more. 

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss HELLP syndrome. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Khan H. HELLP Syndrome. Medscape Reference. 2018; http://emedicine.medscape.com/article/1394126-overview.
  2. HELLP Syndrome. FamilyDoctor.org. September 27, 2017; http://familydoctor.org/familydoctor/en/diseases-conditions/hellp-syndrome.printerview.all.html.
  3. White CD. HELLP syndrome. MedlinePlus. 2018; http://www.nlm.nih.gov/medlineplus/ency/article/000890.htm.
  4. Sibai BM. HELLP syndrome. UpToDate. Waltham, MA: UpToDate; 2018;
  5. HELLP Syndrome. American Pregnancy Association. 2018; http://americanpregnancy.org/pregnancy-complications/hellp-syndrome/.
  6. Kjell Haram, Jan Helge Mortensen, and Bálint Nagy. Genetic Aspects of Preeclampsia and the HELLP Syndrome. Journal of Pregnancy. 2014; 2014:
  7. Sibai BM. HELLP syndrome. UpToDate. Waltham, MA: UpToDate; Feb 20, 2018; https://www.uptodate.com/contents/hellp-syndrome.
  8. Cavaignac-Vitalis M, Vidal F, Simon-Toulza C, Boulot P, Guerby P, Chantalat E, Parant O. Conservative versus active management in HELLP syndrome: results from a cohort study. J Matern Fetal Neonatal Med. December, 2017; 21:1-7. https://www.ncbi.nlm.nih.gov/pubmed/29228827.