HELLP syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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
Historical Perspective
HELLP syndrome was identified as a distinct clinical entity (as opposed to severe preeclampsia) by Dr Louis Weinstein in 1982.[1]
Pathophysiology
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.
Differentiating HELLP Syndrome from other Diseases
Rarely, post caesarean patients with HELLP may be in shock, which mimics either a pulmonary embolism or hemorrhage.
Epidemiology and Demographics
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.
Risk Factors
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.
Diagnosis
History and Symptoms
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.
CT
A CT scan may show bleeding into the liver.
Treatment
Medical Therapy
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.
References
- ↑ 1.0 1.1 Weinstein L (1982). "Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy". Am. J. Obstet. Gynecol. 142 (2): 159–67. PMID 7055180.
- ↑ Padden MO (1999). "HELLP syndrome: recognition and perinatal management". American family physician. 60 (3): 829–36, 839. PMID 10498110.