HELLP Syndrome

HELLP Syndrome

Article
Women's Health
Diseases & Conditions
+2
Contributed byMaulik P. Purohit MD MPHSep 25, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Haemolysis - Elevated Liver Enzymes - Low Platelet Count Syndrome
  • HELLP - Syndrome of Hemolysis, Elevated Liver Enzymes and Low Platelet
  • Hemolysis - Elevated Liver Enzymes - Low Platelet Count Syndrome (HELLP Syndrome)

What is HELLP Syndrome? (Definition/Background Information)

  • HELLP Syndrome is a potential life-threatening complication of pregnancy that occurs in the 3rd trimester or after childbirth; generally in women of childbearing age
  • HELLP Syndrome is a term that is coined per the following parameters or conditions:
    • H - Hemolysis (destruction of red blood cells)
    • EL - Elevated Liver enzymes
    • LP - Low Platelet counts
  • The exact cause of HELLP Syndrome is unknown. Although, being pregnant or having a history of HELLP Syndrome due to previous pregnancy/pregnancies, increases the risk for developing the condition
  • The signs and symptoms of HELLP Syndrome include fatigue, malaise, headache, and worsening nausea. A healthcare professional can utilize a physical examination, medical history evaluation, and blood and urine tests to aid in the diagnosis
  • For women who are pregnant and diagnosed with HELLP Syndrome, the healthcare provider may recommend an early delivery of the baby, even if it is before term. This can help avoid potential complications
  • Also, women who are treated early are typically known to have a good prognosis. If HELLP Syndrome remains undiagnosed, it may result in complications, such as clotting disorders, fluid buildup in the lungs, and kidney failure, which can even lead to death

According to Mississippi classification, there are 3 categories of HELLP Syndrome (based on its severity or blood platelet count level):

  • Class I: Blood platelet count is less than 50,000/ml
  • Class II: Blood platelet count is less than 50,000-100,000/ml
  • Class III: Blood platelet count is less than 100,000-150,000/ml, with liver enzyme aspartate aminotransferase (AST) greater than 40IU/l

Who gets HELLP Syndrome? (Age and Sex Distribution)

  • HELLP Syndrome affects pregnant women of different ages. The condition mostly occurs in women who are in their third trimester, or who have recently given birth to a child
  • Women of all racial and ethnic backgrounds may be affected, though Caucasian are affected more than women of any other race/ethnic group
  • Reports estimate that worldwide up to 0.5% of all pregnancies (or 1 in 200 pregnancies) may be affected by HELLP Syndrome

What are the Risk Factors for HELLP Syndrome? (Predisposing Factors)

The risk factors for HELLP Syndrome include the following factors:

  • Pregnancy: Expectant mothers who are in their 3rd trimester are at an increased risk
  • Postpartum period: The period immediately following childbirth
  • A previous history of HELLP Syndrome greatly increases the risk in subsequent pregnancies; the recurrence rate can be as high as 25%
  • Pre-eclampsia, which is high blood pressure and high protein content in urine, during pregnancy

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of HELLP Syndrome? (Etiology)

The cause of HELLP Syndrome is currently unknown.

  • The disorder only occurs in pregnant women, or in women who have just given birth to a child
  • Some researchers say that HELLP Syndrome is a variant of pre-eclampsia (a complication of pregnancy with high blood pressure and high urine protein levels)

What are the Signs and Symptoms of HELLP Syndrome?

The signs and symptoms of HELLP Syndrome are manifested as the following:

  • Hemolysis, which can cause paleness, fever, weakness, and confusion
  • Elevated liver enzymes that may indicate damage to the liver
  • Low platelet count resulting in severe bleeding upon injury

Other signs and symptoms of HELLP Syndrome may include:

  • Fatigue and malaise (feeling unwell)
  • Headache
  • Worsening nausea and vomiting
  • Pain in the upper right abdomen
  • Blurry vision

The less common signs and symptoms of HELLP Syndrome may include:

  • High blood pressure
  • Vaginal bleeding
  • Fluid retention leading to weight gain in individuals with HELLP Syndrome
  • Proteinuria: Presence of abnormal levels of proteins in the urine

The signs and symptoms generally vary among pregnant women, due to which the condition may be underdiagnosed. These can include:

  • Chest pain
  • Abnormal bleeding
  • Abdominal pain
  • Vision defects
  • Nausea and vomiting

How is HELLP Syndrome Diagnosed?

HELLP Syndrome can be elusive to diagnosis, because it can mimic the signs and symptoms of many other conditions. Some of the diagnostic tools that may be used include:

  • Physical examination for abdominal tenderness, enlarged liver, and swelling in the legs, along with an analysis of previous medical history
  • Blood and urine tests; the presence of proteins in urine may be detected
  • Blood pressure measurements; an increase in BP level may be noted
  • Liver function tests

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of HELLP Syndrome?

Complications of HELLP Syndrome can arise before and after childbirth and these may include:

  • A clotting disorder, known as disseminated intravascular coagulation (DIC) that can lead to excessive bleeding
  • Pulmonary edema or fluid in the lungs
  • Acute kidney failure in the mother
  • Placental abruption: The premature detachment of the placenta from the walls of the uterus, before the baby is delivered
  • Acute respiratory distress syndrome in mother; a type of lung failure
  • Infant respiratory distress syndrome causing lung failure in newborn
  • Complications due to blood transfusion, when necessitated
  • Retinal detachment in the expectant mother
  • Bleeding of the liver and subsequent liver failure; liver rupture
  • Brain stroke
  • Expectant mothers diagnosed with the syndrome generally give birth to smaller babies (small for gestational age)

There is a higher incidence of stillbirths or neonatal deaths associated with HELLP Syndrome.

How is HELLP Syndrome Treated?

HELLP Syndrome usually resolves itself following childbirth. A healthcare provider will recommend that the baby be delivered as early as possible, even if pre-term, because the syndrome can quickly develop to become dangerous to the baby and mother.

The following treatment measures may be recommended:

  • Blood and blood product transfusion
  • Medication to treat high blood pressure
  • Medication to help the baby develop its lungs faster
  • Magnesium sulfate may be administered to prevent seizures in the mother (termed as eclampsia)
  • Corticosteroid therapy, administered to the mother, can help in baby’s lung development
  • Bed rest, close monitoring of pregnancy, and continuous fetal monitoring

How can HELLP Syndrome be Prevented?

  • There are currently no known preventative measures for HELLP Syndrome, because the cause of the condition is unknown. However, early recognition of the signs and symptoms is important for prompt treatment and prevent complications
  • Regular prenatal and antenatal visits to a healthcare provider is strongly recommended, especially if there is a history of HELLP Syndrome in previous pregnancy(pregnancies)

What is the Prognosis of HELLP Syndrome? (Outcomes/Resolutions)

  • The prognosis for women with HELLP Syndrome is good, if regular prenatal checkups are scheduled and the condition is diagnosed early
  • If there is a delay in diagnosis or misdiagnosis of the condition, then it may give rise to serious complications that can even result in death, if immediate treatment is not provided
  • Babies born to mothers with HELLP Syndrome can have a variable prognosis; it depends on the birth weight, organ development, and several other factors. The global mortality rate without treatment is 25%

Additional and Relevant Useful Information for HELLP Syndrome:

Please visit our Pregnancy-Related Disorders Health Center for more physician-approved health information:

http://www.dovemed.com/diseases-conditions/pregnancy-related-disorders/

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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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