HELLP syndrome

Definition

HELLP syndrome is a group of symptoms that occur in pregnant women who have:

     
  • H - hemolytic anemia  
  • EL - elevated liver enzymes  
  • LP - low platelet count

Causes, incidence, and risk factors

HELLP syndrome occurs in approximately 10% of pregnant women with pre-eclampsia or eclampsia. Pre-eclampsia may be mild or severe. Severe cases involve high blood pressure and protein in the urine and can progress to seizures (eclampsia). Severe cases are life-threatening to both mother and fetus. HELLP syndrome is associated with more serious cases.

It may occur long before a pregnancy reaches term (for example, HELLP at 30 weeks gestation). Many women have high blood pressure and are diagnosed with pre-eclampsia before they get HELLP syndrome. However, in some cases, HELLP symptoms are the first warning of pre-eclampsia and the condition is misdiagnosed as hepatitis, gallbladder disease, idiopathic thrombocytopenic purpura, or thrombotic thrombocytopenic purpura.

Symptoms

     
  • Progressive nausea and vomiting  
  • Upper abdominal pain  
  • Headache

Signs and tests

During a physical examination, the doctor may discover upper abdominal tenderness, especially in the right upper quadrant. The liver may be enlarged.

Liver function tests (liver enzyme tests) may be elevated. Red blood cell and platelet counts may be low.

Treatment

The main treatment is to deliver the baby as soon as possible, since liver function in the mother rapidly deteriorates in this condition, and this is harmful to both mother and child.

In more severe cases, the baby has to be delivered before its due date. If this is the case, a cesarian section may be necessary. In less severe cases, the physician will monitor the mother and wait as long as is possible to deliver the baby either through natural or induced labor.

Expectations (prognosis)

When the disease is not treated early, up to 25% of affected women develop serious complications. Without treatment, approximately 1.1-3.5% of patients die from HELLP syndrome. The fatality rate among babies born to mothers with HELLP syndrome varies, depending on factors like birth weight. (See also prematurity.)

Complications
The mother’s liver may hemorrhage (bleed). Permanent liver damage, which can be fatal, may occur if delivery is delayed.

Calling your health care provider
See your obstetrician immediately, call the local emergency number (such as 911), or get to the emergency room if the symptoms above occur during pregnancy. HELLP syndrome can rapidly progress to an emergency condition!

Prevention
Early and continuing prenatal care to facilitate early identification and treatment of conditions such as pre-eclampsia and eclampsia may help to prevent the disorder. Some cases are not preventable.

Johns Hopkins patient information

Last revised: December 8, 2012
by Armen E. Martirosyan, M.D.

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