The average gestational age of presentation of HELLP syndrome is 34 weeks. The majority of women with HELLP syndrome have hypertension and proteinuria prior to diagnosis. Patients are usually multiparous and over the age of 35 years old. Patients are often overweight and have edema in 50% of the cases. Many present with right upper quadrant or epigastric colicky pain with nausea and vomiting. This is usually preceded by malaise 1 to 2 days earlier. 30% to 60% also have a headache, and 20% have a visual disturbance. The condition exacerbates during the night. The symptoms continuously progress.[5][6]

Apart from the clinical signs and symptoms, laboratory investigations are required to diagnose HELLP syndrome. Two classifications are used to diagnose HELLP syndrome: Tennessee and Mississippi.

Main diagnostic criteria of the HELLP syndrome

The Tennessee classification system diagnostic criteria for HELLP are:

• Hemolysis
• Increased LDH (> or =600 IU/L)
• Increased AST (>or =70 IU/L)
• Low platelets (< 100 x 10(9)/L)

The HELLP syndrome may be complete or incomplete.

Mississippi classification measures the severity of the syndrome using the lowest observed platelet count along with the other two main clinical criteria (LDH and AST). Class I is the more severe, with a relatively high risk of morbidity and mortality, compared to the other two classes.

• Class I HELLP syndrome is characterized by a platelet count below 50,000/microL. (LDH> or=600 IU/L, AST> or= 70 IU/L)
• Class II HELLP syndrome is characterized by a platelet count of 50,000 to 100,000/microL. (LDH> or=600 IU/L, AST> or=70 IU/L)
• Class III HELLP syndrome is characterized by a platelet count of 100,000 to 150,000/microL. (LDH>OR=600 IU/L, AST> or= 40 IU/L)[5][6]

Given the lack of clinical trials for the management of HELLP syndrome based on the gestational age of presentation, many treatments are experimental in nature, and there has been a little significance in the improvement of perinatal outcomes between expectant management versus delivery before 34 weeks.[7] That being said, the course of true HELLP syndrome has the potential to quickly turn life-threatening for both mother and fetus. Therefore the recommendation is always to hospitalize patients for strict monitoring of laboratory values. During hospitalization, patients should be treated as severely pre-eclamptic and should receive magnesium sulfate for seizure prophylaxis along with blood pressure control with hydralazine, labetalol, or nifedipine in usual recommended fashion.

Maternal-fetal monitoring should be performed throughout each step of management since typically, immediate delivery is recommended for true HELLP patients except those with stable maternal-fetal conditions between 24 to 34 weeks gestation. For this group of patients, a recommendation is to give corticosteroids (betamethasone 12mg intramuscular every 12 hours for 2 doses or dexamethasone 12mg intravenously every 12 hours for 4 doses) then deliver 24 hours after the last dose. Steroid administration is not only beneficial to the fetus for lung maturity but also for improvement of laboratory values in patients, particularly in elevating platelet counts. Some patients may benefit from transfusions of red cells, platelets, and plasma. A study performed to assess the rate of epidural anesthesia in patients with HELLP saw an increase in rates of neuro-axial anesthesia in those who achieved a latency period after the administration of steroids.[8]

HELLP syndrome should be differentiated from other disorders of pregnancy with similar features:

1. Pre-eclampsia: It has normal liver enzymes and platelet count. Schistocytes are also absent.
2. Acute fatty liver of pregnancy (AFL): Hypoglycemia is present in AFL but absent in HELLP syndrome.
3. Thrombotic thrombocytopenic purpura (TTP): It usually manifests in 2nd or 3rd trimester, and liver abnormalities are not as elevated as in HELLP syndrome. Patients are typically normotensive and have undetectable ADAMTS 13 activity.
4. Hemolytic-uremic syndrome (HUS): It has the same findings as TTP except that its incidence is higher in the post-partum period, and patients have signs of renal failure.
5. Lupus flare: Liver pathology is absent in lupus.
6. Antiphospholipid syndrome (APS): Dominant features of APS are arterial/venous thrombosis and repeated pregnancy loss. Lupus anticoagulant, cardiolipin antibodies, beta-glycoprotein antibodies, prothrombin time (PT), and an activated partial thromboplastin time (aPTT) should be checked to confirm the diagnosis.[9]
7. Other: Viral hepatitis, cholecystitis, cholangitis, gastritis, gastric ulcer, acute pancreatitis, upper UTI.

HELLP syndrome is a life-threatening condition. The mortality rate of women with HELLP syndrome is 0%-24%, with a perinatal death rate of up to 37%.[2] Maternal death occurs due to disseminated intravascular coagulation (DIC), placental abruption, postpartum hemorrhage, or acute renal failure. DIC occurs in 15% to 62.5% of the cases. Placental abruption occurs in 11% to 25% of women with HELLP syndrome. Postpartum hemorrhage occurs in 12.5% to 40% and acute renal failure in 36% to 50% of the cases. Poor perinatal prognosis is because of placental abruption, intrauterine hypoxia and asphyxia, prematurity, and low birth weight.[10]

Patients with HELLP syndrome have a 19%-27% risk of developing HELLP syndrome in subsequent pregnancies. Class 1 HELLP syndrome has the highest recurrence rate. Recurrent cases occur in the latter part of the gestation period and are less severe after two episodes.[11]

Early diagnosis and treatment, along with maternal and neonatal intensive care, can help to reduce the mortality in HELLP syndrome.

HELLP syndrome is a life-threatening condition with high maternal and infant mortality rates. Maternal complications include:

1. Eclampsia
2. Placental abruption
3. Cesarean section
4. DIC
5. Recurrent thrombosis
6. Liver rupture
7. Cerebral infarction
8. Cerebral hemorrhage
9. Pulmonary/cerebral edema
10. Cardiovascular instability
11. Acute renal failure
12. Infection/sepsis
13. Maternal death[12]

Fetal complications include:

1. Perinatal death
2. Intrauterine growth restriction (IUGR)
3. Preterm delivery
4. Neonatal thrombocytopenia
5. Respiratory distress syndrome[5]

• Intensive care
• Anesthesiology
• Pediatrics/neonatology
• Maternal-fetal medicine

Patients with HELLP syndrome should be given information about the course of the disease. The risk of maternal and perinatal complications and mortality should be explained to the patient and their family. The risk of developing HELLP can only be decreased by maintaining a healthy lifestyle to prevent diseases such as hypertension and diabetes. Regular exercise should be followed. The patient should also be counseled about the risk of HELLP syndrome in subsequent pregnancies. Regular prenatal care and laboratory testing have to be done in the subsequent pregnancies.

The management of HELLP syndrome and its complications requires an interprofessional team approach. Pre-eclampsia, mainly managed by an obstetrician, may benefit from help from other specialties when HELLP syndrome develops. The patient's blood pressure and diabetes can be managed with the help of an internal medicine or family medicine clinician. Complications such as cesarean section and pulmonary edema require help from anesthesiology and a hospitalist, respectively. Continuous nursing and intensive care are required by the patient if they are admitted to an ICU. A neonatologist is needed to care for the neonate and its complications.