HELLP Syndrome

 

Original Editors - Carolyn S. Furdek from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Carolyn Furdek, Nicole Hills, Lucinda hampton, Admin, Elaine Lonnemann, Kim Jackson, 127.0.0.1, Wendy Walker and WikiSysop  

Definition/Description

File:Liver.jpg

A liver affected by HELLP Syndrome. Image courtesy of The Internet Journal of Anesthesiology.

HELLP syndrome is an acronym for several life-threatening symptoms that occur together during a woman’s pregnancy.

These symptoms are:

H - Hemolysis

EL – Elevated Liver enzymes

LP – Low Platelet count. 

Prevalence

As of Nov 2010, for every 1,000 pregnancies, 1 to 2 (0.5%-0.9%) women were diagnosed with HELLP syndrome.  Furthermore, 10-20% of women diagnosed with severe preeclampsia will be diagnosed with HELLP. Mortality rates associated with HELLP syndrome have been reported as high as 25%.

Patients have a 19-27% chance of reoccurrence on subsequent pregnancies.

Characteristics/Clinical Presentation

Clinical symptoms of HELLP include discomfort in the upper right quadrant of the abdomen, pain in the epigastric area, vomiting, and nausea. The abdominal discomfort can increase and decrease throughout the day. Patients can report extreme fatigue prior to presentation or ‘feeling unwell’. Other symptoms include: headache, fluid retention, excess weight gain, blurry vision, nosebleeds (or bleeding that does not stop easily), and seizures/convulsions.

Approximately 7 out of 10 patients with HELLP syndrome will experience the symptoms prior to delivery between the 27th and 37th week of gestation. The remaining patients will develop the symptoms within 48 hours postpartum.

There are two classifications of HELLP Syndrome:

• Classification 1:  Basis of 3 classic lab values

    - Partial: one/two of the classic values present 

    - Full:  all three abnormalities present

(Full HELLP syndrome classifications have a higher mortality rate and should be delivered within 48 hours)

• Classification 2:  Basis of platelet count

    - Class 1:  Platelet count < 50,000 mm³

    - Class 2:  Platelet count 50,000-100,000 mm³

    - Class 3:  Platelet count 100,000-150,000 mm³

(Class 1 patients have a higher maternal morbidity and mortality rate)

Associated Comorbidities

Patients who present with HELLP syndrome may have a higher risk for the following conditions:

• Renal Failure - loss of the kidney’s ability to function properly. The body will no longer be capable of filtering excess fluid, waste, and salts from the blood. This leads to dangerous levels in the system.
• Consumptive coagulopathy  - (also known as disseminated intravascular coagulation (DIC)) - clotting factors reduced
• Abruptio placentae - the placenta nourishing the fetus abruptly separates from the uterine wall prior to delivering the baby
• Pulmonary edema - fluid build up in the lungs - can lead to shortness of breath.
• Cerebral edema - build up of fluid around the brain
• Subcapsular liver hematoma - pooling of blood just outside of the liver
• Hypovolemic shock - excessive fluid and blood loss that can lead to organ failure
  

Medications

Maternal Medications

• Magnesium sulfate – anticonvulsant to prevent seizures
• Antihypertensive medications – high blood pressure
• Blood product – if necessary
• Dexamethasone – corticosteroid used for fetus lung maturity prior to delivery 

Diagnostic Tests/Lab Tests/Lab Values

Hemolysis: 

• Low haptoglobin concentration (< 1 g/L – < 0.4 g/L)  - more specific indicator
• High LDH
• presence of unconjugated bilirubin

   (if Hematocrit normal: decreased serum haptoglobin levels may be present indicating HELLP)

Liver Enzymes: As high as 4,000 U per L

Platelets: As low as 6,000 per mm³ (anything less than 150,000 per mm³ should be of concern)

Plasma fibrogen: levels less than 300 mg per dL (DIC suspected) 

Etiology/Causes

In 1982 L Weinstein identified cardinal signs and symptoms that were a variant of severe preeclampsia and named the condition HELLP.

The overall cause of HELLP syndrome in pregnant women is unknown at this time. However, researchers do have a better understanding of the three main characteristics that are known to occur with HELLP. These symptoms are: haemolysis, elevation of liver enzymes, and thrombocytopenia.

• Haemolysis occurs due to microangiopathic haemolytic anaemia (MAHA). Blood smears have shown contracted red cells w/ spicula, polychromatic red cells, and increased reticulocyte counts. All these findings lead researches to suspect the development of MAHA. Increased LDH levels and decreased haemoglobin concentrations further show Haemolysis.
  
• Elevated liver enzymes indicates the involvement of the liver as well as reflecting the haemolytic process.
• Thrombocytopenia has been associated to the destruction of platelets. 

**A recent study out of Turkey (published in March 2011) found that Homocysteine levels were significantly higher and a increased likelihood of deficiency in antithrombin III were found in women diagnosed with HELLP. (Further research in this area is needed in order to validate this report)

Systemic Involvement

HELLP syndrome mainly involves the liver and the blood therefore is part of the digestive and circulatory system as described in the Etiology/Causes section. However, if left untreated, multiple organ systems can go into failure as described in the Associated Comorbidities section. 

Medical Management (current best evidence)

In patients diagnosed with HELLP syndrome prior to delivery, the immediate treatment is delivery of the fetus. If the fetus is earlier than 34 weeks gestation, steroid injections and close monitoring for 24-48 hours may be provided to allow the fetus’ lungs to mature. 

Figure: Suggested protocol in treating pts with HELLP Syndrome

[Chart courtesy of Journal of The American Family Physician: HELLP Syndrome]

Prognosis

Current research concurs that following delivery of the fetus and subsequent stabilization of the mother, there are no residual effects to the mother.  Patients should be advised that there is a 43% chance of developing preeclampsia in subsequent pregancies.  As mentioned in the Description section there is a 19 to 27% chance of reocurrance of HELLP syndrome in subsequent pregnancies. Patients diagnosed with Class I HELLP syndrome are at the highest risk of reoccurance. If the sydrome reoccurs, it tends to develop later in gestation and with less severity.

Physical Therapy Management (current best evidence)

Due to the severity and risk of maternal mortality, conservative management is not recommended in the treatment of HELLP syndrome.  Abdominal weakness and deconditioning may be seen in patients after a caesarean section.  Most patients are advised to wait 4-6 weeks before resuming physical activity and exercise.   

Differential Diagnosis

HELLP Syndrome my be misdiagnosed as any of the below conditions:

• Viral Hepatitis
• Cholangitis
• Acute fatty liver of pregnancy
• Haemolytic uremic syndrome
• Thrombotic thrombocytopenic purpura
• Systemic lupus erythematosus

Case Reports/ Case Studies

• HELLP syndrome in a pregnant patient with a past history of splenectomy for idiopathic thrombocytopenic purpura. Case Report. [view article in Journal of Gynecology and Obstetrics]  
• Subscapular Liver Hematoma in HELLP Syndrome: Case Report. [view article in Gastroenterology Research]
• Case Report: post partum class 1 HELLP syndrome.  [view article in Journal of Gynecology and Obstetrics]

Resources

• American Pregnancy Association - www.americanpregnancy.org
• Preeclampsia Foundation - www.preeclampsia.org