What is HELLP Syndrome?

HELLP syndrome is a life threatening variant of preeclampsia, characterized by a group of serious symptoms and complications that generally occur late in pregnancy or after delivery. Dr. Louis Weinstein named the condition using the initials of its main features [1], with the full form being:

  • Hemolysis (abnormal break down of RBC)
  • Elevated Liver enzymes
  • Low Platelet count

HELLP Syndrome Classification

It is classified into three categories [2] using the Mississippi classification system which determines the severity of the condition depending on the reduction in the patient’s blood platelet count.

  • Class I (characterized by severe thrombocytopenia): Platelet count under 50,000/mm3
  • Class II (characterized by moderate thrombocytopenia): Platelet count between 50,000/mm3 and 100,000/mm3
  • Class III (characterized by mild thrombocytopenia): Platelet count between 100,000/mm3 and 150,000/mm3

What Causes HELLP Syndrome in Pregnancy?

Researchers are still trying to find out the exact causes responsible for this pregnancy related problem. However, certain risk factors have been identified by doctors [3].

  • Having been suffered from preeclampsia in previous pregnancies
  • Conceiving at ages over 25 years
  • Suffering from obesity
  • Suffering from diabetes
  • Having a poor and unhealthy diet
  • Lack of regular exercise
  • Suffering from PIH (pregnancy-induced hypertension)
  • Multiple pregnancies
  • Being Caucasian

HELLP Syndrome Pathophysiology

Although the triggering factors are still unknown, experts believe the condition to occur when the coagulation cascade gets activated due to abnormal functioning of the body. Fibrin creates conjoined networks within the small blood vessels, leading to microangiopathic hemolytic anemia. The mesh formed by the fibrin destroys red blood cells as they pass through it. Considerable numbers of platelets are destroyed as well. As the whole process takes place in the liver, the downstream liver cells develop ischemia which leads to periportal necrosis. The disorder may equally affect cells in various other organs. In some cases, it may become quite difficult to perform an emergency surgery as HELLP syndrome gradually leads to a certain form of DIC (disseminated intravascular coagulation), resulting in paradoxical bleeding.

What are the Symptoms of HELLP Syndrome?

During the initial stages, the patient may or may not have the symptoms of high blood pressure, proteinuria (protein in the urine) and high uric acid levels in urine. These are the characteristic symptoms of preeclampsia. The onset of the symptoms occurs during the second or third trimester. Women suffering from HELLP syndrome often experience:

  • Headache [4]
  • Nausea and vomiting
  • Indigestion
  • Heartburn
  • Pain in the upper right abdomen
  • Chest pain (particularly behind the sternum)
  • Chest and abdominal tenderness
  • Shoulder and neck pain
  • Pain experienced when taking a deep breath
  • Pain originating in the liver (due to liver enlargement) and spreading in other regions
  • Vision problems
  • Edema or swelling of different body parts
  • Seizure

How to Prevent HELLP Syndrome?

Although it is not possible to prevent the condition from occurring, taking the following measures may reduce the risks [2]:

  • Follow a regular exercise schedule to stay in excellent physical shape before conceiving
  • Make sure to visit the physician regularly, especially in case of first pregnancy
  • Inform the doctor about any family history of preeclampsia and HELLP syndrome
  • In case of second pregnancy, inform the doctor about any complication that may have occurred during the previous pregnancy
  • Monitor the signs of pregnancy carefully and contact the physician if there are any abnormal symptoms as these may be the early warning signs of HELLP syndrome

HELLP Syndrome Diagnosis and Diagnostic Criteria

The diagnostician should look for the laboratory criteria [5] mentioned below to confirm the diagnosis:

1) Hemolysis, accompanied by burr cells, polychromasia and schistocytes shown on a peripheral blood smear test. However, peripheral smears are not always used for this diagnosis as the presence of Hemolysis can be confirmed from the following evidence:

  • Elevated LDH (lactate dehydrogenase): >600 IU/L
  • Elevated bilirubin levels: > 1.2 mg/ dL (5.1 micromol/ L)
  • Low serum haptoglobin levels (rarely used)

2) Elevated liver transaminases: Aspartate aminotransferase (AST) and/or alanine transaminase (ALT) >70 IU/L

  • Thrombocytopenia: platelet count <100,000/ microL (<100 x 10^9/ L).

3) Diagnostic tests used for confirming the presence of HELLP Syndrome include:

  • Blood tests (coagulation panel, full blood count, electrolytes, kidney function study and liver enzyme levels)
  • Blood pressure measurement
  • Urine tests (for evidence of excessive protein in the urine)

Radiology and CT scan may be necessary in case of any possible complications.

HELLP Syndrome Differential Diagnosis

Sometimes, the absence of the primary preeclampsia symptoms may lead the diagnostician to mistake the condition for some other disorder [6]. The initial symptoms of HELLP Syndrome are similar to those caused by:

  • Gastritis
  • Acute hepatitis
  • Gall bladder diseases
  • Haemolytic uraemic syndrome
  • Thrombotic Thrombocytopenic Purpura
  • Acute fatty liver in pregnancy

How to Treat HELLP Syndrome?

The treatment mainly focuses on management of the symptoms and prevention of any serious complication. Delivering the baby is the most effective way to prevent the disorder from leading to any complication in the mother or the baby.

In cases where the HELLP syndrome occurs within 34 weeks of gestation, the baby’s lung functioning is evaluated by doctors for determining the possible effects of delivery on the baby. Treatment guidelines for managing the condition until the baby is mature enough for delivery usually include:

  • Complete bed rest [7]
  • Monitoring the symptoms closely for any sign of deterioration
  • Monitoring the fetal development closely, using biophysical tests, fetal movement evaluation, non stress tests and ultrasounds (Doppler flow studies) for any abnormality
  • Blood transfusions may be required in case the platelet count becomes too low

Drugs and medications used for the purpose include

  • Corticosteroids (to ensure proper lung development of the baby)
  • Magnesium Sulfate (to manage the symptom of seizure)
  • Blood pressure medications for lowering the elevated blood pressure

Postpartum HELLP syndrome treatment may involve administration of heparin shots.

HELLP Syndrome Prognosis

The symptoms of HELLP Syndrome tend to worsen postpartum before resolving entirely (within 2 to 3 days) with proper treatment and medications. The time required to reach complete recovery may vary in cases where complications are present.

Maternal mortality rate is as low as 1% with proper treatment. The number of prenatal and infant death ranges from 73 to 119 per 1000 cases. Around 40% babies born to HELLP affected mothers are delivered prematurely, hence are smaller than the ideal birth size. The outcome and long term effects of HELLP in the baby are determined by factors like the gestational age rather than the severity of the disorder itself.

HELLP Syndrome Complications

  • Placental abruption [8]
  • Pulmonary edema
  • Stroke (cerebral hemorrhage or cerebral edema)
  • Lung failure in the mother or the baby
  • Permanent heart failure
  • Kidney damage
  • Acute renal failure
  • Intrauterine Growth Restriction or IUGR
  • Permanent liver damage
  • Placental abruption
  • Eye problems like retinal detachment in the baby
  • Ruptured liver hematoma
  • Diseminated intravascular coagulation or DIC
  • Stillbirth
  • Death of the mother

HELLP Syndrome Recurrence

The recurrence rate ranges between 2% and 19% depending on whether one suffered from preeclampsia in an earlier pregnancy.

HELLP Syndrome ICD-10 Code

The ICD-10 Code for HELLP syndrome is O14.2.

HELLP Syndrome Support

Preeclampsia Foundation

6767 N Wickham Road,

Suite 400

Melbourne, Florida 32940

Phone: 321.421.6957

Toll Free: 800.665.9341

Fax: 321.821.0450

Email: [email protected]

Website: http://www.preeclampsia.org/

  1. http://www.nlm.nih.gov/medlineplus/ency/article/000890.htm
  2. http://www.preeclampsia.org/health-information/hellp-syndrome
  3. http://umm.edu/health/medical/pregnancy/specialcare-pregnancies/hellp-syndrome
  4. http://www.webmd.com/baby/tc/hellp-syndrome-and-preeclampsia-topic-overview
  5. http://bestpractice.bmj.com/best-practice/monograph/1000/diagnosis/criteria.html
  6. http://www.patient.co.uk/doctor/hellp-syndrome
  7. http://www.chp.edu/CHP/P02454
  8. http://americanpregnancy.org/pregnancycomplications/hellpsyndrome.html [/ref]