Keywords

Pregnancy-induced hypertension, pre-eclampsia, HELLP syndrome

About the Authors

Associate ProfessorSenior ResidentDept. of Obstetrics and GynecologySenior Resident Dept. of MedicineESI-PGIMSR, MGM Hospital, Parel, Mumbai, MaharashtraAddress for correspondenceDr Jaya Kundan GedamL-1, RH-2 Ground Floor, L-1, RH-2, Sector-6, Vashi, Navi Mumbai, Maharashtra E-mail: jayagedam@gmail.com

Introduction

HELLP syndrome is a life-threatening complication of pregnancy characterized by hemolysis, elevated liver enzymes and low platelets. It is usually considered to be a variant or complication of pre-eclampsia. However, in 20% of cases it may occur without pre-eclampsia during antenatal or intrapartum period. HELLP is a multisystemic disorder, leading to generalized vasospasm, microthrombi formation and coagulation defects. Clinical features include severe headache, malaise, nausea and vomiting, pain around upper abdomen and pedal edema. In about 20% of all women, disseminated intravascular coagulation (DIC) is also seen with HELLP syndrome.

Case Report

A 35-year-old pregnant woman, primigravida, was admitted in emergency to our hospital in the 36th week of gestation, with mild pregnancy- induced hypertension (PIH). Her antenatal check- ups were infrequent but normal. On admission, her blood pressure was 140/90 mmHg (after 2 hours; 170/100 mmHg). Urine albumin was trace. Capsule nifedipine 5 mg was given orally. Prophylactic injection magnesium sulfate (MgSO4) was started. Her cervix was 2 cm dilated, 25% effaced. Augmentation of labor was done with oxytocin. All laboratory findings including hematological, biochemical and coagulation profile, were within their normal limits (Table 1). Three hours after her admission, cervical dilatation was 3-4 cm and 50-60% effaced, bleeding per vagina started with passage of clots. Artificial rupture of membrane was done to rule out abruptio placenta, liquor was clear. In view of excessive bleeding per vaginum, cesarean section was done under spinal anesthesia.

Peroperative findings: A live born, 2800 g, female fetus with a 9 Apgar at 5th minute was delivered. As uterus was flabby, intravenous (IV) oxytocin infusion 30 units was started and injection carboprost 0.25 mg intramuscular (IM) given. Uterus became well- contracted, but excessive oozing from raw areas around uterine incision, muscle, skin continued. So, injection tranexamic acid IV was given. Patient became severely pale. Frank hematuria was observed. Though patient’s vitals were maintained but due to excessive bleeding and with probable diagnosis of DIC central line was inserted in operation theater.

Patient was shifted to intensive care unit (ICU) postoperatively for further management. All necessary blood and urine investigations were sent. Injection vitamin K, IV antibiotics and injection tranexamic acids were given along with IV fluids.

Postoperative hematological parameters were: hemoglobin - 7.80 g/dL and platelet count - 71,000/uL after 2 hours of operation. Hematological and biochemical parameters, including serum glutamic oxaloacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT) and lactate dehydrogenase (LDH), were detected to be higher than their respective reference intervals; 170.4 IU/L, 111 IU/L and 1,538 IU/L, respectively.

Peripheral blood smear-Schistocytes - occasional, spherocytes - present, urine albumin was trace, red blood cell (RBC) - 75-100/hpf, coagulation profile - prothrombin time (PT) - 15 sec, mean PT/control value 13.3, fibrinogen - 243 mg/dL, D-dimer - 1.40 mg/L, plasma fibrin degradation products (FDP) level - 2.91 µg/L. Diagnosis of HELLP syndrome was made. Transfusion of 4 platelets, 2 units of fresh frozen plasma, 2 units of packed cells were done.

After 8 hours, the laboratory findings were consistent with those of HELLP syndrome, which included hemolysis (hemoglobin - 7.2 g/dL, LDH - 1,618 IU/L),elevated liver enzymes (SGOT - 122.4 IU/L, SGPT - 75.8 IU/L) and low platelet counts (platelet: 50,000/uL) (Table 1). Coagulation profile and other biochemical parameters such as electrolyte values were in normal limitation of references.

An ultrasound examination showed a normal liver structure and an empty uterine cavity. MgSO4 infusion (2 g/hour; 24 hours) and steroids were administered (dexamethasone; total 30 mg for 48 hours), with supportive therapy such as: IV fluids, fresh frozen plasma and packed red blood cells.

The laboratory findings improved dramatically after the treatment and at the 88th hour (4th day) of postpartum period, all of them completely regressed to normal value. Foley’s catheter was removed on post-op Day 5. Patient was shifted to ward on post-op Day 7.

On Day 8, suture removal was done. Wound was healthy. Patient was discharged on iron and calcium supplements. Contraceptive counseling was done. She was discharged on the 10th day without any sequelae.

Discussion

Pregnancy-induced hypertensive disorders constitute 12-22% of all pregnancies and 17.6% maternal mortality.1 The HELLP syndrome is usually considered to be a variant or complication of pre-eclampsia. However, in 20% of cases it may occur without pre- eclampsia during antenatal or intrapartum period. It is difficult for clinicians to predict the development of pre-eclampsia due to absence of obvious signs.2,3

HELLP is a multisystemic disorder, leading to generalized vasospasm, microthrombi formation and coagulation defects. It is the final manifestation of insult that leads to microvascular endothelial damage and intravascular platelet aggregation.4 There is an important role of coagulopathy in causation of HELLP syndrome suggested by clinically evident DIC as a secondary pathophysiological phenomenon to the primary process is seen in 4-38% of the patients. If not treated timely may lead to renal failure.5

HELLP syndrome is a dreaded obstetric emergency usually associated with pre-eclampsia. Its incidence is 0.5-0.9% of all pregnancies. It is related with severe pre-eclampsia (10-20%). Both conditions occur after 20 weeks of gestation and may occur after 5-12 weeks of childbirth.

Clinical features include severe headache (30%), malaise (90%), nausea and vomiting (30%), pain around upper abdomen (65%) and pedal edema. In about 20% of all women, DIC is also seen with HELLP syndrome.6 Our patient presented only with excessive bleeding per vaginum during first stage of labor. She had no other complaints.

Pregnant women who present with HELLP syndrome can be misdiagnosed in the early stages, thereby increasing the risk of maternal morbidity. In a patient with PIH and suspected to have HELLP, following blood tests should be performed: full blood count, liver function tests including enzymes, renal function tests, coagulogram, serum electrolytes and D-dimer. We conducted all the above investigations in our patient and we diagnosed our patient was suffering from HELLP syndrome as D-dimer was positive. A positive D-dimer test in a patient of pre-eclampsia is predictive of HELLP syndrome.7

D-dimer is a more sensitive indicator of subclinical coagulopathy and may be positive before other coagulation studies become abnormal. According to Mississippi classification3,8 and Tennessee classification, the disease can be classified as mild, moderate and severe. Our case was a severe variety of HELLP syndrome. Sibai has proposed strict criteria for true or complete HELLP syndrome platelet - <1,00,000, AST - >70 IU/L, LDH - >600 IU/L.9 The definitive treatment of HELLP syndrome is immediate delivery of baby either by cesarean section or normal vaginal route.9

As our patient was hemodynamically stable, she was operated under spinal anesthesia. Corticosteroids can reduce the severity of intravascular endothelial injury and improve blood flow. They also decrease hepatocyte and platelet consumption in HELLP syndrome.10

Conclusion

In conclusion, when patients present with mild-PIH presents with excessive bleeding per vagina during labor, it must be kept in mind that it can be one of the signs of HELLP syndrome and if we are aware of this, we can significantly reduce the maternal morbidity and prevent the development of maternal mortality.

References

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