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HELLP syndrome is a life-threatening complication of pre-eclampsia. Both conditions occur during the latter stages of pregnancy, or sometimes after childbirth.
HELLP is an abbreviation of the main findings:
- Hemolytic anemia
- Elevated Liver enzymesand
- Low Platelet count
- 1 Signs and symptoms
- 2 Diagnosis
- 3 Classification
- 4 Pathophysiology
- 5 Treatment
- 6 Epidemiology
- 7 History
- 8 References
Signs and symptoms
Often, a patient who develops HELLP syndrome has already been followed up for gestational hypertension, or is suspected to develop pre-eclampsia(high blood pressure and proteinuria). Up to 8% of all cases present after delivery.
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. Oedemamay occur but its absence does not exclude HELLP syndrome. Arterial hypertensionis a diagnostic requirement, but may be mild. Rupture of the liver capsule and a resultant hematomamay occur. If the patient gets a seizureor coma, the condition has progressed into full-blown eclampsia.
In a patient with possible HELLP syndrome, a batch of blood testsis performed: a full blood count, liver enzymes, renal functionand electrolytesand coagulationstudies. Often, fibrindegradation products (FDPs) are determined, which can be elevated. Lactate dehydrogenaseis a marker of hemolysis and is elevated (>600 U/liter). Proteinuriais present but can be mild.
The plateletcount has been found to be moderately predictive of severity: under 50 million/L is class I (severe), between 50 and 100 is class II (moderately severe) and >100 is class III (mild). This system is termed the Mississippi classification (Martin et al 1990).
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 cellsas if they were being forced through a strainer. Additionally, plateletsare consumed. As the liverappears 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.
The only effective treatment is delivery of the baby, preferably by cesarean section. Several medications have been investigated for the treatment of HELLP syndrome, but evidence is conflicting as to whether magnesium sulfatedecreases the risk of seizures and progress to eclampsia. The DIC is treated with fresh frozen plasmato replenish the coagulation proteins, and the anemiamay require blood transfusion. In mild cases, corticosteroidsand antihypertensives(labetolol, hydralazine, nifedipine) may be sufficient. Intravenous fluids are generally required.
Its incidence is reported as 0.2-0.6% of all pregnancies. Of women with (pre)eclampsia, 4-12% also develop signs of a "superimposed" HELLP syndrome. Mortality is 7-35% and perinatal mortality of the child may be up to 40%.
HELLP syndrome was identified as a distinct clinical entity (as opposed to severe preeclampsia) by Dr Louis Weinstein in 1982.
- Martin JN Jr, Blake PG, Lowry SL, Perry KG Jr, Files JC, Morrison JC. Pregnancy complicated by preeclampsia-eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet count: how rapid is postpartum recovery? Obstet Gynecol 1990;76:737-41. PMID 2216215.
- Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982;142:159-67. PMID 7055180.de:HELLP-Syndrom
Categories: Obstetrics| Medical emergencies
This article is licensed under the GNU Free Documentation License.
It uses material from the Wikipedia article HELLP syndrome.