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HELLP Syndrome as a Separate Entity Dr. Mohammed Abdalla EGYPT, Domiat G. HospitalHELLP Syndrome May it be a separate entity? yes HELLP, a syndrome characterized by hemolysis, elevated liver enzyme levels and a low platelet count, is an obstetric complication that is frequently misdiagnosed at initial presentation. Many investigators consider the syndrome to be a variant of preeclampsia, but it may be a separate entity.
HELLP Syndrome as a Separate Entity
Dr. Mohammed Abdalla
EGYPT, Domiat G. Hospital
May it be a separate entity?
HELLP, a syndrome characterized by hemolysis, elevated liver enzyme levels and a low platelet count, is an obstetric complication that is frequently misdiagnosed at initial presentation. Many investigators consider the syndrome to be a variant of preeclampsia, but it may be a separate entity.
In some cases, HELLP symptoms are the first warning of preeclampsia and the condition is misdiagnosed as hepatitis, idiopathic thrombocytopenic purpura, gallbladder disease, or thrombotic thrombocytopenic purpura.
Epidemiology and Risk Factors
• HELLP syndrome 0.2 to 0.6% of all pregnancies.
• Preeclampsia 5 - 7% of all pregnancies.
• Superimposed HELLP syndrome develops in 4 to 12 percent of women with preeclampsia or eclampsia.
• Maternal mortality has been estimated to be as high as 2 - 24%
• Perinatal mortality is equally high, ranging from 9 - 39%
Wolf JL. Liver disease in pregnancy. Med Clin North Am 1996.
Etiology and Pathogenesis
• The hemolysis in HELLP syndrome is a microangiopathic hemolytic anemia. Red blood cells become fragmented as they pass through small blood vessels with endothelial damage and fibrin deposits.
• The peripheral smear may reveal spherocytes, schistocytes, triangular cells and burr cells.
• Increase in Bilirubin and lactic dehydrogenase levels.
Etiology and Pathogenesis
The elevated liver enzyme levels in the syndrome are thought to be secondary to obstruction of hepatic blood flow by fibrin deposits in the sinusoids. This obstruction leads to periportal necrosis and, in severe cases, intrahepatic hemorrhage, subcapsular hematoma formation or hepatic rupture.
Etiology and Pathogenesis
The thrombocytopenia has been attributed to increased consumption and/or destruction of platelets.
With platelet activation, thromboxane A and serotonin are released, causing vasospasm, platelet agglutination and aggregation, and further endothelial damage.
• 90% of patients present with generalized malaise,
• 65% with epigastric pain,
• 30% with nausea and vomiting,
• 31% with headache.
All are nonspecific symptoms
Because of the variable nature of the clinical presentation, the diagnosis of HELLP syndrome is generally delayed for an average of eight days.
Usually presented by complications
In one retrospective chart review of patients with HELLP syndrome, only two of 14 patients entered the hospital with the correct diagnosis.
Because early diagnosis of this syndrome is critical, any pregnant woman who presents with malaise or a viral-type illness in the third trimester should be evaluated with a complete blood cell count and liver function tests.
The physical examination may be normal in patients with HELLP syndrome.
1) Right upper quadrant tenderness 90%
2) Edema is not a useful marker
3) Hypertension and proteinuria may be absent or mild.
There is agreement among most of the authors that, the diagnosis requires the concurrence of hemolysis, elevated liver enzymes, and low platelet count. However, there is obviously still a lack of consensus on the laboratory parameters and their cutoff values used to diagnose.
Martin JN Jr, Rinehart BK, May WL, Magann EF, Terrone DA, Blake PG
Laboratory Diagnostic Criteria for HELLP syndrome
Abnormal peripheral smear: spherocytes, schistocytes, triangular cells and burr cells
Total Bilirubin level > 1.2 mg/dL
Lactate dehydrogenase level > 600U/L
Elevated liver function test result
Serum aspartate amino transferase level > 70 U/L
Lactate dehydrogenase level > 600 U/L
Low platelet count
Platelet count < 150 000/mm3
Platelet count appears to be the most reliable indicator of the presence of HELLP syndrome
Clinical utility of strict diagnostic criteria for the HELLP
The use of strict diagnostic criteria in the definition of the HELLP syndrome allows for greater prediction of complication rates. and define the cases that are eligible to conservative management.
Based on the number of abnormalities.
Classification on the basis of platelet count
class I, less than 50,000 per 3mm
class II, 50,000 to less than 100,000 per mm3
class III, 100,000 to 150,000 per mm3
The treatment approach should be based on the estimated gestational age and the condition of the mother and fetus.
Prolongation of pregnancy, in theory, may be favourable for the foetus whereas it remains controversial whether maternal condition is further deteriorated by expectant management.
Visser W, Wallenburg HC. Temporising management of severe pre-eclampsia with and
without the HELLP syndrome. Br J Obstet Gynaecol 1995;102:111-7
Eligibility to conservative management
• Hypertension is controlled at less than
• Oliguria responds to fluid management.
• Elevated liver function values are not associated with right upper quadrant or epigastric pain.
• Class II – III (platelet count) > 50000
The Cochrane Library holds two protocols which when complete may summarize evidence to date on the use of corticosteroids for HELLP syndrome and interventionist versus expectant management of severe pre-eclampsia before term.
The antenatal administration of dexamethasone (Decadron) in a high dosage of 10 mg intravenously every 12 hours has been shown to markedly improve the laboratory abnormalities associated with HELLP syndrome.
Steroids given antenatally do not prevent the typical worsening of laboratory abnormalities after delivery. However, laboratory abnormalities resolve more quickly in patients who continue to receive steroids postpartum.
Magann EF, Bass D, Chauhan SP, Sullivan DL, Martin RW, Martin JN Jr. Am J Obstet Gynecol 1994;171:1148-53.
Corticosteroid therapy should be instituted in patients with HELLP syndrome who have a platelet count of less than 100,000 per mm3 and should be continued until liver function abnormalities are resolving and the platelet count is greater than 100,000 per mm3.
Magann EF, Perry KG Jr, Meydrech EF, Harris RL, Chauhan SP, Martin JN Jr. Am J Obstet Gynecol 1994;171:1154-8.
Intravenously administered dexamethasone appears to be more effective than intramuscularly administered betamethasone for the antepartum treatment of mothers with HELLP syndrome.
(Am J Obstet Gynecol 2001;184:1332-9.)
Administration of glucocorticoids increases the use of regional anesthesia in women with antepartum HELLP syndrome who have thrombocytopenia.
(Am J Obstet Gynecol 2002;186:475-9.)
Patients treated with dexamethasone exhibit longer time to delivery. This facilitates maternal transfer to a tertiary care center and postnatal maturity of fetal lungs.
(Am J Obstet Gynecol 2002;186:475-9.)
Patients with HELLP syndrome should be treated prophylactically with magnesium sulfate to prevent seizures, whether hypertension is present or not.
Antihypertensive therapy should be initiated if blood pressure is consistently greater than 160/110 mm hg despite the use of magnesium sulfate. The goal is to maintain diastolic blood pressure between 90 and 100 mm hg.
The most commonly used antihypertensive agent has been:
Between 38 -93 % of patients with HELLP syndrome receive some form of blood product.
Patients with a platelet count greater than 40,000 per mm3 are unlikely to bleed.
• • Patients who undergo cesarean section should be transfused if their platelet count is less than 50,000 per mm3.
• Prophylactic transfusion of platelets at delivery does not reduce the incidence of postpartum hemorrhage or hasten normalization of the platelet count.
• Patients with DIC should be given fresh frozen plasma and packed red blood cells.
Pain relief with intravenous narcotics and local anesthesia is acceptable but certainly not optimal for pain control.
Epidural anesthesia has been controversial but it is the technique of choice when it can be accomplished safely. Insertion of an epidural catheter is generally safe in patients with a platelet count greater than 100,000 per mm3.
General anesthesia can be used when regional anesthesia is considered unsafe.
Portis R, Jacobs MA, Skerman JH, Skerman EB. HELLP syndrome (hemolysis, elevated
liver enzymes, and low platelets) pathophysiology and anesthetic considerations.
AANA J 1997; 65:37-47.
• The mortality rate for women with HELLP syndrome is approximately 1.1 %
• From 1 to 25 % of affected women develop serious complications such as DIC, placental abruption, adult respiratory distress syndrome, hepatorenal failure, pulmonary edema, subcapsular hematoma and hepatic rupture.
• A significant percentage of patients receive blood products.
Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal
morbidity and mortality in 442 pregnancies with hemolysis, elevated liver
enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol 1993;169:1000-6
• Infant morbidity and mortality rates range from 10 to 60 %, depending on the severity of maternal disease.
• Infants affected by HELLP syndrome are more likely to experience intrauterine growth retardation and respiratory distress syndrome.
Dotsch J, Hohmann M, Kuhl PG. Neonatal morbidity and mortality associated with
maternal haemolysis, elevated liver enzymes and low platelets syndrome. Eur J
Once the diagnosis of HELLP syndrome has been established, the best markers to follow are the
maternal lactate dehydrogenase level and the maternal platelet count
The laboratory abnormalities in HELLP syndrome typically worsen after delivery and then begin to resolve by three to four days postpartum.
Martin JN Jr, Blake PG, Perry KG Jr, McCaul JF, Hess LW, Martin RW. The natural history of HELLP syndrome: patterns of disease progression and regression. Am J Obstet Gynecol 1991;164(6 pt 1):1500-9.
The incidence of hemorrhagic complications is higher when platelet counts are < 40,000 per mm3.
Patients with HELLP syndrome who complain of severe right upper quadrant pain, neck pain or shoulder pain should be considered for hepatic imaging regardless of the severity of the laboratory abnormalities, to assess for subcapsular haematoma or rupture.
Patients with HELLP syndrome should be routinely treated with corticosteroids.