HELLP syndrome

Answer 1

Definition

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

• H -- hemolysis
• EL -- elevated liver enzymes
• LP -- low platelet count

Causes, incidence, and risk factors

HELLP syndrome occurs in approximately 10% of pregnant women with preeclampsiaor eclampsia. HELLP syndrome is associated with more serious cases.

Many women have high blood pressure and are diagnosed with preeclampsia before they develop HELLP syndrome. However, in some cases, HELLP symptoms are the first warning of preeclampsia and the condition is misdiagnosed as hepatitis, gallbladder disease, idiopathic thrombocytopenic purpura, or thrombotic thrombocytopenic purpura.

Symptoms

• Headache
• Nausea and vomiting that continues to get worse
• Upper abdominal pain
• Vision problems

Signs and tests

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

Liver function tests (liver enzymes) 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, even if premature, since liver function in the mother gets worse very quickly. Problems with the liver can be harmful to both mother and child.

Expectations (prognosis)

When the disease is not treated early, up to 25% of women develop serious complications. Without treatment, a small number of women die. The Death Rate among babies born to mothers with HELLP syndrome varies and depends on birth weight and the development of the baby's organs, especially the lungs. (See also: Prematurity)

Complications

The mother's liver may bleed (hemorrhage). Permanent liver damage may occur if delivery is delayed. Such damage can lead to death.

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.

Although there is no known way to prevent HELLP syndrome, it is important for all pregnant women to start prenatal care early and continue it through the pregnancy. This allows the health care provider to find and treat conditions such as HELLP syndrome early.

References

Sibai BM. Hypertension. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Obstetrics - Normal and Problem Pregnancies. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2007:chap 33.

Martin JN, Rose CH, Briery CM. Understanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child. American Journal of Obstetrics and Gynecology. 2006; 195(914-34).

Answer 2

Definition

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

• H -- hemolysis(the breakdown of red blood cells)
• EL -- elevated liver enzymes
• LP -- low platelet count

Causes, incidence, and risk factors

A cause for HELLP syndrome has not been found.

HELLP syndrome occurs in about 1 to 2 out of 1,000 pregnancies, and in 10-20% of pregnant women with severe preeclampsia or eclampsia.

Most often HELLP develops before the pregnancy is 37 weeks along. Sometimes it can develop in the week after the baby is born.

Many women have high blood pressure and are diagnosed with preeclampsia before they develop HELLP syndrome. However, in some cases, HELLP symptoms are the first warning of preeclampsia and the condition is misdiagnosed as:

• Flu or other viral illness
• Gallbladder disease
• Hepatitis
• Idiopathic thrombocytopenic purpura (ITP)
• Lupus flare
• Thrombotic thrombocytopenic purpura

Symptoms

• Fatigue or feeling unwell
• Fluid retention and excess weight gain
• Headache
• Nausea and vomiting that continues to get worse
• Pain in the upper right part of the abdomen
• Blurry vision
• Nosebleed or other bleeding that won't stop easily (rare)
• Seizures or convulsions (rare)

Signs and tests

During a physical examination, the doctor may discover:

• Abdominal tenderness, especially in the right upper side
• Enlarged liver
• High blood pressure
• Swelling in the legs

Liver function tests (liver enzymes) may be high. Platelet counts may be low. A CT scan may show bleeding into the liver.

Tests of the baby's health will be done.

Treatment

The main treatment is to deliver the baby as soon as possible, even if the baby is premature. Problems with the liver and other complications of HELLP syndrome can quickly get worse and be harmful to both the mother and child.

Your doctor may induce labor by giving you drugs to start labor, or may perform a C-section.

You may also receive:

• A blood transfusion if bleeding problems become severe
• Corticosteroid medications to help the baby's lungs develop faster
• Medications to treat high blood pressure

Expectations (prognosis)

When the disease is not treated early, up to 1 out of 4 women develop serious complications. Without treatment, a small number of women die.

The death rate among babies born to mothers with HELLP syndrome depends on birth weight and the development of the baby's organs, especially the lungs. (See also: Prematurity)

HELLP syndrome may return in up to 1 out of 4 future pregnancies.

Complications

There can be complications before and after the baby is delivered, including:

• Disseminated intravascular coagulation (DIC) -- a clotting disorder that leads to excess bleeding (hemorrhage)
• Fluid in the lungs (pulmonary edema)
• Kidney failure
• Liver hemorrhage and failure
• Separation of the placenta from the uterine wall (placental abruption)

After the baby is born and HELLP syndrome has time to improve, most of these complications will go away.

Calling your health care provider

If symptoms of HELLP syndrome occur during pregnancy:

• See your obstetrician immediately
• Call the local emergency number (such as 911)
• Get to the emergency room

Although there is no known way to prevent HELLP syndrome, it is important for all pregnant women to start prenatal care early and continue it through the pregnancy. This allows the health care provider to find and treat conditions such as HELLP syndrome early.

References

Sibai BM. Hypertension. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Obstetrics - Normal and Problem Pregnancies. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2007:chap 33.

Wakim-Fleming J. Liver disease in pregnancy. In: Carey WD, ed. Cleveland Clinic: Current Clinical Medicine 2010. 2nd ed. Philadelphia, Pa: Saunders Elsevier;2010:section 6.

Reviewed By

Review Date: 11/21/2010

Linda Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine; and Susan Storck, MD, FACOG, Chief, Eastside Department of Obstetrics and Gynecology, Group Health Cooperative of Puget Sound, Redmond, Washington; Clinical Teaching Faculty, Department of Obstetrics and Gynecology, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.