Disseminated intravascular coagulation complicating HELLP

syndrome: perioperative management

Rakesh Garg,1,2 M P Nath,1 A P Bhalla,1 and Ashwani Kumar1

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Abstract
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BACKGROUND
HELLP syndrome, a variant of severe preeclampsia, was first described in 1954 by Pritchard et
al and identified as a distinct clinical entity by Louis Weinstein in 1982.1,2 Waterstone et
al redefined HELLP syndrome as haemolysis (abnormal peripheral smear or raised total bilirubin
>20.5 μmol/l), raised liver enzyme activity (elevated aspartate aminotransferase >70 IU/l or
raised glutamyltransferase >70 IU/l) and low platelets (<100 000/ml). Sometimes HELLP
syndrome leads to disseminated intravascular coagulation (DIC), which can make emergency
surgery a serious challenge. We present the case of a pregnanat woman who was scheduled for
emergency caserean section with DIC superimposed on HELLP syndrome and preeclampsia.
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CASE PRESENTATION

Case report
A 29-year-old female weighing 50 kg was referred to our institute with haematuria and epistaxis.
She was gravida 1, para 1, 37 weeks of gestation and had not had any regular antenatal check
ups. She had had an episode of haematuria 10 days previously. On examination in a peripheral
hospital, she had elevated blood pressure and was started on α-methyldopa. She developed
profuse epistaxis which was controlled after bilateral nasal packing. Her coagulation profile was
deranged (prothrombin time (PT) raised 1 min above control).
There was no history of blurring of vision or epigastric pain. The patient denied a history of prior
abnormal bleeding episodes or ingestion of any medication except α-methyldopa. Examination
revealed pallor and pedal oedema. Blood pressure was 200/160 mm Hg and was controlled with
three 5 mg boluses of intravenous labetalol. Investigation revealed haemoglobin 5.8 g/dl,
platelets 109×109/l and lactate dehydrogenase 1196 U/l. Liver function tests showed increased
liver enzymes (SGPT 144 IU, SGOT 88 IU), bilirubin 3.2 mg/dl and serum albumin 2.8 g/dl.
Coagulation profile was suggestive of consumption coagulopathy (PT 63 s above control,
activated partial thromboplastin time (aPTT) 80 s above control, INR (international normalised
ratio) – 6.58 and D-dimer level >200 ng/ml). Urine examination showed proteinuria and
haematuria. Removal of the 4-day old nasal pack in the operating room resulted in resurgence of
profuse epistaxis. Anterior/posterior nasal packing controlled the bleeding but the postnasal
bleed continued. The trachea was electively intubated under midazolam sedation for protection
of the airway and the patient was placed on spontaneous ventilation with oxygen
supplementation at 4 l/min via a T-piece. The baby was monitored using cardiotocography and
Doppler ultrasonography.
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DIFFERENTIAL DIAGNOSIS
A diagnosis of DIC complicating HELLP in preeclampsia was made and an emergency
caesarean section was planned.
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TREATMENT
Urine output after catheterisation was >1 ml/kg/h. The patient was transfused with 15 ml/kg of
fresh frozen plasma (FFP) and PT improved to 7 s above control. After a loading dose of 4 g,
infusion of intravenous magnesium sulphate at 1 g/h was commenced. Intravenous
dexamethasone 12 mg was administered.
In the operating room, blood pressure was 154/92 mm Hg. Two 16-G intravenous cannula, the
left radial artery with a 20-G cannula and the right brachial vein with a peripherally inserted
central catheter were cannulated after local infiltration. The cardiotocographic examination of the
fetus was normal. General anaesthesia was induced with thiopentone 4 mg/kg and neuromuscular
blockade by vecuronium 0.1 mg/kg and maintained with isoflurane (0.5%) in oxygen and nitrous
oxide (50:50). After delivery of the baby, 2 μg/kg fentanyl was administered intravenously. The
baby weighed 2.42 kg and had Apgar scores of 5, 7 and 8 at 1, 5 and 10 min, respectively, and
was transferred to the nursery for observation. Intraoperative blood loss (1250 ml) was replaced
with 15 ml/kg FFP, three units of packed red blood cells (RBC), 1 litre hexastarch and 1 litre
crystalloids. The patient maintained a urine output of 2 ml/kg/h. At the end, anaesthesia was
discontinued and the patient was moved to the intensive care unit and put on pressure control
ventilation. Blood pressure was controlled with a titrated infusion of trinitroglycerine (2–6
μg/kg/min). Fentanyl (50–100 μg/h) and midazolam (1–2 mg/h) infusion was titrated according
to the patient’s response (analgesia and sedation). Postoperatively, the patient continued to bleed
vaginally. Haemoglobin was 7.3 g/dl, platelet count 41×109/l and PT prolonged by 20 s above
control. The patient developed profuse malena. Two units of RBC, six units of platelets and 10
ml/kg of FFP were transfused. The patient was started on intravenous tranexamic acid,
butryphase and oxytocin infusion at 5 units/h. Dexamethasone was continued 12 hourly. Further
platelets and FFP were transfused guided by platelet count, coagulation profile and evidence of
active bleeding. Blood pressure, central venous pressure and urine output were monitored.
Thromboprophylaxis was provided with pneumatic lower limb tourniquets.
Subsequently, the patient’s coagulation profile normalised and platelet count increased to
158×109/l on the second postoperative day. Magnesium sulphate, oxytocin and trinitroglycerine
infusions were discontinued on the first, second and third postoperative days, respectively.
Ventilatory support was weaned to oxygen via a T-piece at 4 l/min and nasal packing was
removed on the third postoperative day. No epistaxis ensued and there was no bleeding from any
site. Antifibrinolytic measures were continued until the fifth postoperative day. Subsequently, the
trachea was extubated and the patient remained comfortable on oxygen via a face mask.
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OUTCOME AND FOLLOW-UP

The patient remained haemodynamically stable. She was shifted to a high dependency ward on
the fifth postoperative day and was discharged on the 10th postoperative day.
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DISCUSSION
HELLP syndrome complicates 0.2–0.6% of all pregnancies, 4–12% of cases with severe
preeclampsia and 30–50% of eclamptic gravidas.1 Maternal and neonatal mortality is 2–24% and
3–39%, respectively.2–4HELLP syndrome may progress to DIC in 15–38% of patients.3,5 Patients
with HELLP syndrome are at increased risk of abruptio placentae, pulmonary oedema, ARDS,
ruptured liver haematoma, acute renal failure, cerebrovascular accident and multiorgan failure.3,6–
8

Our patient presented with haematuria, epistaxis, hypertension and generalised malaise together
with deranged liver function and coagulation parameters. At presentation we suspected
hypertensive bleeding, DIC or idiopathic epistaxis. Other differential diagnoses included acute
fatty liver of pregnancy, thrombotic thrombocytopenic purpura (TTP) and haemolytic uremic
syndrome (HUS).
TTP and HUS were ruled out in our patient because of the abnormal coagulation studies and
adequate urine output. Absence of severe liver damage and normal blood sugar (113 mg/dl) ruled
out acute fatty liver of pregnancy, although ammonia which is raised in this condition could not
be checked in our case. Thrombocytopenia and microangiopathic haemolytic anaemia are more
severe in HELLP syndrome than in preeclampsia and acute fatty liver of pregnancy.
Investigation confirmed DIC with jaundice along with HELLP syndrome and preeclampsia in
our patient. Hypertension usually precedes DIC associated with HELLP syndrome as was also
seen in our patient.
The PT, aPTT and serum fibrinogen levels are normal in HELLP syndrome but prolonged in
DIC. Evaluation of more sensitive markers of DIC, such as antithrombin III, α-2 antiplasmin,
plasminogens, fibrin monomer and D-dimers, differentiates DIC from HELLP
syndrome.1,5,6 Sibai et al defined DIC as the presence of thrombocytopenia, low fibrinogen levels
(ie, a plasma fibrinogen level <300 mg/dl) and fibrin degradation products >40 mg/ml.9 A
positive D-dimer test in the setting of preeclampsia is predictive of patients who will develop
HELLP syndrome.10
Thrombocytopenia is the major and early cause of alteration of coagulation in HELLP syndrome.
When the platelet count decreases to <50×109/l, an association with DIC can be considered, with
a worse prognosis. Fibrin degradation products are non-specific unless >40 mg/l but 15% of
patients with DIC have concentrations of <40 mg/l. Fibrin degradation products also have a long
half-life of 5–72 h and so do not always reflect current coagulation status.1
Aggressive treatment is indicated and delivery should be expedited, by caesarean section if
necessary although vaginal delivery is not contraindicated, along with control of blood pressure
and coagulation abnormality. DIC is treated with FFP to replenish the coagulation proteins, and
the anaemia may require blood transfusion. Patients with HELLP syndrome should be treated
prophylactically with magnesium sulfate to prevent seizures, whether hypertension is present or
not. Diuretics may compromise placental perfusion and are not preferred to control blood
pressure.1,2 α-Methyldopa, labetalol, nifedipine, sodium nitroprusside, nitroglycerine or a
combination of these drugs is required to control severe hypertension and to maintain diastolic
blood pressure at 90–100 mm Hg.3 Our patient required nitroglycerine infusion along with
labatalol, α-methyldopa and magnesium during the perioperative period.
In HELLP syndrome, platelet consumption is increased and the transfused platelets are likely to
be consumed as quickly as native ones. If DIC is also present, this process is further accelerated.
There appears to be little reason to delay surgery to provide time for platelet transfusions in
patients with HELLP syndrome. In fact, platelets may be transfused while preparing the patient
for surgery; the availability of cross-matched blood, platelet concentrates and FFP should be
checked before taking the patient with DIC and HELLP syndrome to the operating room.
Corticosteroid therapy is instituted in patients with a platelet count <100×109/l and should be
continued until liver function abnormalities are resolving and the platelet count is >100×109/l.11
Epidural anaesthesia is controversial but is generally safe in patients with a platelet count
>100×109/l, and normal coagulation studies and bleeding time. General anaesthesia can be used
when regional anaesthesia is considered unsafe. Our patient presented with signs and symptoms
and deranged coagulation suggestive of DIC, HELLP syndrome and preeclampsia. Such patients
who present at short notice for emergency caesarean section, pose considerable challenges to the
attending anaesthesiologist.
HELLP syndrome, by itself, is not an indication for invasive monitoring; the severity of the
coexisting state determines the need. We inserted an arterial line to ascertain accurate blood
pressure and titrate vasodilators for optimal control of blood pressure and thus maintain
uteroplacental perfusion. Preeclamptic women have smaller plasma volumes than normal
pregnant women and require adequate fluid management for optimal urine output and perfusion
of vital organs, thus mandating a central venous catheter. We preferred a peripherally inserted
central venous catheter in view of the deranged coagulation profile. The roles of the
anaesthesiologist, intensivist, obstetrician and haematologist in following up and managing such
cases is crucial.
In HELLP syndrome, pregnancies may be complicated by intrauterine growth retardation,
premature delivery, placental abruption, respiratory distress syndrome and cerebral haemorrhage
in the neonate. The presence of a neonatalogist for neonatal care and to prepare the equipment
and drugs required to resuscitate the neonate are mandatory.
LEARNING POINTS

 Patients with disseminated intravascular coagulation (DIC) complicating HELLP

syndrome and preeclampsia require great vigilance and multimodal management in the
perioperative period for uneventful outcome.
  DIC complicating HELLP syndrome and preeclampsia also requires a multidisciplinary
approach involving the anaesthetist, obstetrician, haematologist and head and neck
surgeon.

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Footnotes
Competing interests: none.

Patient consent: Patient/guardian consent was obtained for publication.

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