HELLP syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

HELLP syndrome is a life-threatening obstetric complication considered by many to be a variant of pre-eclampsia. Both conditions occur during the later stages of pregnancy, or sometimes after childbirth.

HELLP is an abbreviation of the main findings:^[1]Hemolytic anemia, Elevated Liver enzymes, and Low Platelet count

HELLP syndrome was identified as a distinct clinical entity (as opposed to severe preeclampsia) by Dr Louis Weinstein in 1982.^[1]

The exact cause of HELLP is unknown, but general activation of the coagulation cascade is considered the main underlying problem. Fibrin forms crosslinked networks in the small blood vessels. This leads to a microangiopathic hemolytic anemia: the mesh causes destruction of red blood cells as if they were being forced through a strainer. Additionally, platelets are consumed. As the liver appears to be the main site of this process, downstream liver cells suffer ischemia, leading to periportal necrosis. Other organs can be similarly affected. HELLP syndrome leads to a variant form of disseminated intravascular coagulation (DIC), leading to paradoxical bleeding, which can make emergency surgery a serious challenge.

Rarely, post caesarean patients with HELLP may be in shock, which mimics either a pulmonary embolism or hemorrhage.

The incidence of HELPP is reported to be 0.2-0.6% of all pregnancies. Of women with preeclampsia, 4-12% also develop signs of a “superimposed” HELLP syndrome. HELLP usually begins during the third trimester, and usually in Caucasian women over the age of 25. Rarely, cases have been reported as early as 23 weeks gestation.

Often, a patient who develops HELLP syndrome has already been followed up for pregnancy-induced hypertension (gestational hypertension), or is suspected to develop pre-eclampsia (high blood pressure and proteinuria). Up to 8% of all cases present after delivery.

Patients who present with symptoms of HELLP can be misdiagnosed in the early stages, increasing the risk of liver failure and morbidity.^[2] There is gradual but marked onset of headaches (30%), blurred vision, malaise (90%), nausea/vomiting (30%), “band pain” around the upper abdomen (65%) and tingling in the extremities. Edema may occur but its absence does not exclude HELLP syndrome. If the patient develops a seizure or coma, the condition has progressed into full-blown eclampsia.

A CT scan may show bleeding into the liver.

The only effective treatment is delivery of the baby. Several medications have been investigated for the treatment of HELLP syndrome, but evidence is conflicting as to whether magnesium sulfate decreases the risk of seizures and progress to eclampsia. The DIC is treated with fresh frozen plasma to replenish the coagulation proteins, and the anemia may require blood transfusion. In mild cases, corticosteroids and antihypertensives (labetalol, hydralazine, nifedipine) may be sufficient. Intravenous fluids are generally required.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

HELLP syndrome was identified as a distinct clinical entity (as opposed to severe preeclampsia) by Dr Louis Weinstein in 1982.^[1]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

The platelet count has been found to be moderately predictive of the severity of HELLP syndrome. This system is termed the Mississippi classification.^[1]

Severe: < 50 K

Moderately severe: Between 50 and 100 K

Mild: > 100 K

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

The exact cause of HELLP is unknown, but general activation of the coagulation cascade is considered the main underlying problem. Fibrin forms crosslinked networks in the small blood vessels. This leads to a microangiopathic hemolytic anemia: the mesh causes destruction of red blood cells as if they were being forced through a strainer. Additionally, platelets are consumed. As the liver appears to be the main site of this process, downstream liver cells suffer ischemia, leading to periportal necrosis. Other organs can be similarly affected. HELLP syndrome leads to a variant form of disseminated intravascular coagulation (DIC), leading to paradoxical bleeding, which can make emergency surgery a serious challenge.

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

• 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

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

Rarely, post caesarean patients with HELLP may be in shock, which mimics either a pulmonary embolism or hemorrhage.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

The incidence of HELLP is reported to be 0.2-0.6% of all pregnancies. Of women with preeclampsia, 4-12% also develop signs of a “superimposed” HELLP syndrome. HELLP usually begins during the third trimester, and usually in caucasian women over the age of 25. Rarely, cases have been reported as early as 23 weeks gestation.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

Often, a patient who develops HELLP syndrome has already been followed up for pregnancy-induced hypertension (gestational hypertension), or is suspected to develop pre-eclampsia (high blood pressure and proteinuria). Up to 8% of all cases present after delivery.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

• 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.

• Mortality is 7-35% and perinatal mortality of the child may be up to 40%.
• 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.
• HELLP syndrome may return in up to 1 out of 4 future pregnancies.

Template:WH Template:WS