HELLP Syndrome

What is HELLP Syndrome?

HELLP syndrome is a life-threatening pregnancy complication usually considered to be a variant of preeclampsia. Both conditions usually occur during the later stages of pregnancy, or sometimes after childbirth.

HELLP syndrome was named by Dr. Louis Weinstein in 1982 after its characteristics:

H (hemolysis, which is the breaking down of red blood cells),
EL (elevated liver enzymes) and
LP (low platelet count).

A suspicion of HELLP syndrome can be frustrating to the physician when all requirements for its certain diagnosis are not apparent. In some patients who are developing HELLP syndrome the primary preeclampsia indicators of high blood pressure and protein in the urine may not be present, and its symptoms can be mistaken for gastritis, flu, acute hepatitis, gall bladder disease, or other conditions. While some of these other conditions may also be present, there is no evidence they are related.

Early diagnosis is critical because the morbidity and mortality rates associated with the syndrome have been reported to be as high as 25%. As a result, patient awareness of HELLP syndrome, and how it relates to preeclampsia, is helpful to ensure optimal and timely medical care for mother and baby.

Symptoms of HELLP Syndrome

The physical symptoms of HELLP Syndrome may seem at first like preeclampsia. Symptoms reported by the pregnant woman developing HELLP syndrome may include one or all of the following:

• headache
• nausea/vomiting/indigestion with pain after eating
• epigastric (abdominal) or substernal (chest) tenderness and right upper quadrant pain (from liver distention)
• shoulder pain or pain when breathing deeply
• bleeding
• visual disturbances
• swelling

Signs to look for include:

• high blood pressure
• protein in the urine

The most common reasons for the mother to become critically ill or die are liver rupture or stroke (cerebral edema or cerebral hemorrhage). These can usually be prevented when caught in time. If you or someone you know has any of these symptoms, please see a doctor immediately.

Most often, the definitive treatment for women with HELLP Syndrome is delivery of the baby. Transfusion of some form of blood product (red cells, platelets, plasma) is often needed. Corticosteroids can be used to improve fetal lung maturation in the very preterm pregnancy; the University of Mississippi MFM group have reported beneficial maternal effects to slow disease progression, to improve laboratory parameters, to lessen liver and central nervous system morbidity, and to shorten hospitalization.

Who is at risk of getting HELLP syndrome?

Among pregnant women in the United States, 5 - 8% develop preeclampsia; 15% of those women develop evidence of HELLP syndrome (15-20% of those with severe preeclampsia), meaning as many as 48,000 women per year will develop HELLP syndrome in the US. There is still some difference of opinion among experts as to what lab values constitute HELLP syndrome, so these estimates are approximate and also reflect the higher end of the spectrum.

These numbers will vary with attention to the mother's care. If preeclampsia is diagnosed early and the baby is delivered, HELLP syndrome may not develop. The rate of HELLP and the mortality will then be lower than stated. If the diagnosis of preeclampsia was delayed or it was managed too conservatively, a woman's likelihood of developing HELLP syndrome is even higher.

What can I do to prevent HELLP syndrome?

Unfortunately at this time, there is no way to prevent this illness. The best thing to do is:

• Get yourself in the best physical shape before getting pregnant
• Have regular prenatal visits
• Inform the doctor about any previous high-risk pregnancies or family history of HELLP syndrome, preeclampsia, etc.
• Understand the warning signs and do not delay reporting them to your healthcare provider, including trusting yourself when "something just doesn't feel right"

The severity of HELLP syndrome is measured according to the blood platelet count of the mother and divided into three categories, according to a system termed "the Mississippi classification."

• Class I (severe thrombocytopenia): platelets under 50,000/mm3
• Class II (moderate thrombocytopenia): platelets between 50,000 and 100,000/mm3
• Class III (AST > 40 IU/L, mild thrombocytopenia): platelets between 100,000 and 150,000/mm3

If the baby weighs over 1000 grams (approx. 2 lbs.), at birth, his or her survival rate and length of hospital stay is similar to non-HELLP babies of comparable sizes, and there doesn't seem to be many long-term adverse outcome.

If the baby weighs less than 1000 grams at delivery, the news is not as good. Several studies have suggested longer hospital stays and more chance of needing ventilator care. Unfortunately, right now doctors can't predict the scope of the medical problems that these small babies encounter.

How likely is the baby to die from HELLP Syndrome? In developed countries, the stillbirth rate (in utero death of the baby after 20 weeks of gestation) is 51/1,000 a rate higher than both severe preeclampsia and eclampsia. Overall perinatal mortality from HELLP Syndrome (stillbirth plus neonatal death) ranges from 7.7 to 60%. Most of these deaths are attributed to abruption of the placenta (placenta prematurely separating from the uterus), placental failure with intrauterine asphyxia (fetus not getting enough oxygen), and extreme prematurity.

Women with a history of HELLP syndrome are at increased risk of all forms of preeclampsia in subsequent pregnancies. The rate of preeclampsia in subsequent pregnancies ranges from 16 - 52%, with higher rates if the onset of HELLP syndrome was in the second trimester. The rate of recurrent HELLP syndrome ranges from 2 - 19% depending upon the patient population studied.