Journal of Obstetrics and Gynaecology, May 2013; 33: 331337
2013 Informa UK, Ltd.
ISSN 0144-3615 print/ISSN 1364-6893 online
DOI: 10.3109/01443615.2013.775231
REVIEW
HELLP syndrome: Understanding and management of a
pregnancy-specic disease
S. Aloizos, C. Seretis, N. Liakos, P. Aravosita, C. Mystakelli, E. Kanna & S. Gourgiotis
Intensive Care Unit, Mitera Obstetric and Gynecological Hospital of Athens, Greece
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HELLP, a syndrome characterised by haemolysis, elevated
liver enzymes and low platelets, a variant of pre-eclampsia/
eclampsia, is a multisystemic disorder with an incidence of up
to 0.9% in all pregnancies and occurs in about one-quarter of
pre-eclamptic patients. Most obstetricians presume that HELLP
derives from an autoimmune reaction, leading to a materno
fetal imbalance, with accompanying aggregation of platelets,
endothelial malfunction along with inborn errors of fatty acid
oxidative metabolism. HELLP is characterised by high mortal-
ity and morbidity rates, leading to possibly life-threatening
complications regarding both the mother and the fetus. Delivery
is indicated if HELLP syndrome occurs after 34 gestational weeks
or the fetal and/or maternal conditions deteriorate. Vaginal
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delivery is preferable. Standard corticosteroid treatment is of
uncertain clinical value in maternal HELLP syndrome. Future
observations and research results may shed more light on
improving our understanding of the aspects of HELLP syndrome.
Keywords: Eclampsia, HELLP syndrome, liver, management,
pregnancy
Introduction
Haemolysis, elevated liver enzymes and low platelets (HELLP)
syndrome, was first defined by Weinstein in 1982. It is a multi-
systemic disorder which remains an important cause of maternal
and perinatal mortality and morbidity.
Many researchers and clinicians consider this particular syn-
drome as a variant of another peculiar disease of pregnancy, pre-
eclampsia (Habli et al. 2009), whereas other physicians consider
it to be a totally different expression of the rejection of the fetus
in the late trimester of pregnancy, due to maternofetal immune
imbalance (Tung et al. 2001; Fang et al. 2008b).
HELLP syndrome has an incidence of 0.170.85% of all preg-
nancies, while the risk of recurrence in a subsequent pregnancy
is about 1927% (Isler et al. 2003). It is a multisystem disease
which is characterised by abnormal vascular tone, vasospasm
and coagulation defects. Although, its pathophysiology remains
partially unknown, activation of endothelial cells may lead to
release of von Willebrand factor multimers, which are highly
reactive with platelets. The syndrome seems to be the final
manifestation of some insult that leads to microvascular endothe-
lial damage and intravascular platelet activation. With platelet
activation, thromboxane A and serotonin are released, causing
vasospasm, platelet agglutination and aggregations and further
Correspondence: S. Gourgiotis, Intensive Care Unit, Mitera Obstetric and Gynecological Hospital of Athens, Erythrou Stavrou 6 and Kissias str PO Code
151 23, Maroussi, Athens, Greece. E-mail: drsgourgiotis@tiscali.co.uk
endothelial damage (Hulstein et al. 2003). Recently, fetal disor-
ders of mitochondrial fatty acid oxidation have been associated
with obstetric complications including HELLP syndrome; their
action is not yet understood (Tyni et al. 1998).
The aim of this paper is to summarise currently available
data regarding epidemiology, aetiology, pathology, clinical pre-
sentation, classification, management and prognosis of HELLP
syndrome and to discuss diagnostic problems and management
doubts that affect clinicians and obstetricians.
Methods
A systematic literature search for clinical reports and reviews
on HELLP syndrome published between 1989 and 2011 were
identified using PubMed and the Cochrane databases, and addi-
tional cited works not detected in the initial search were obtained.
Search words were HELLP syndrome, and HELLP syndrome
combined with diagnosis, clinical symptoms, complications,
morbidity, mortality, management, treatment, prognosis
and recurrence. Publications were selected for review based
on original research, highly regarded earlier publications and
comprehensive reviews, while those publications considered as
relevant were used at the authors discretion.
Pathophysiology
Haemolysis
The rapid decrease of the erythrocyte number during the full
presentation of HELLP syndrome is believed to result from
the cellular damage due to fibrin deposition, leading to fragmen-
tation of the red blood cells (Doshi and Zucker 2003; Moake
2009). This particular finding is consistent with microangio-
pathic haemolytic anaemia, with the distorted red cells seen in
the microscope (Burr cells) along with schistocytes, as well as
polychromasia on peripheral blood smear (Baxter and Weinstein
2004). In recent studies, many similarities between haemolytic
uraemic syndrome and HELLP syndrome have been reported
in the field of molecular mechanisms between blood cells,
which interact with one another, proposing a quite similar defec-
tive regulation between these two different pathological entities
(Fang et al. 2008a,b).
Widely accessible markers of haemolysis can be the levels
of lactic dehydrogenase and indirect (unconjugated) bilirubin.
Moreover, the presence of reticulocytes can be explained as an
effort of the organism to withstand this condition and release
the immature cells as a compensatory reflect (Haram et al.
 332 S. Aloizos et al.
2009). According to recent findings, a more sensitive and precise
indicator of haemolysis is the unusually low level of haptoglobin
(van Runnard Heimel et al. 2005); nevertheless, its assessment
is rarely included in the routine laboratory screening of the
patients.
Liver enzymes elevation
The liver involvement in HELLP syndrome is similar to the
alterations presented in pre-eclampsia. Typical histological
findings of liver biopsies of patients include fibrin deposition
intravascularly, which can lead to obstruction of the sinusoids
due to hyaline deposits of fibrin-like material, blood congestion
and elevated intrahepatic pressure, resulting in focal and/or
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periportal hepatocyte necrosis (Tsokos et al. 2002; Baxter and
Weinstein 2004; OBrien and Barton 2005). Moreover, Glissons
capsule may be engorged and distended, producing symptoms
such as mid-epigastric pain and tenderness (Suarez et al. 2002).
Elevation of liver enzymes may reflect the haemolytic
process as well as liver involvement. Haemolysis contributes sub-
stantially to the elevated levels of lactate dehydrogenase (LDH),
whereas enhanced aspartate aminotransferase (AST) and alanine
aminotransferase (ALT) levels are mostly due to liver injury.
Plasma glutathione S-transferase-a1 (GST-a1) may provide a
more sensitive indicator for acute liver damage than AST and
ALT, and allow earlier recognition (Prakash et al. 2010). How-
ever, measurement of GST-a1 is not widely available, and has
not yet found its place in the routine diagnostic procedure
(Molvarec et al. 2009).
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With respect to the elevation of liver enzymes, AST and
ALT may elevate up to 700 U/ml or more, indicating the degree
of liver malfunction; in addition, elevation of LDH levels can
not only suggest liver malfunction at cellular level, but may
also serve a marker of erythrocyte damage. Recently, the mea-
surement of GST-a1 is proposed as being a more sensitive marker
of liver damage, but has not yet found its place in the clinical
procedure (Knapen et al. 1999). However, up to the present
time, neither significant correlation between the histological
findings, biopsy results, clinical presentation and the laboratory
findings nor with the maternal/fetal outcome, have been solidly
founded (Doshi and Zucker 2003; Baxter and Weinstein 2004;
Martin et al. 2006).
The significantly increased levels of tissue plasminogen
activator and plasminogen activator inhibitor-1 (PAI-1) in the
context of HELLP syndrome compared with normal pregnancy,
suggests that platelet activation and the alteration of plasmino-
gen activation are involved in the pathogenesis of this syndrome
(Zhou et al. 2002).
Therefore, other theories have been proposed, suggesting
that HELLP syndrome might be an acute inflammatory condi-
tion; an assumption which might explain the liver cell apoptosis
(Mihu et al. 2007). Research results of CD95 antigen-blockage
(CD95 is produced in the placenta) in mothers presenting the
syndrome are in favour of this particular opinion (Mihu et al.
2007). Lastly, an inborn error of fatty acid oxidative metabolism
in fetuses born in the context of HELLP syndrome supports
the approach that the theory of maternofetal imbalance can
be preserved as a viable explanation of the pathophysiology of
the syndrome. A mutation allows a long chain fatty acid, i.e. a
3-hydroxyacyl metabolite that is produced by the fetus or the
placenta, to accumulate at maternal level due to a long chain
3-hydroxyacyl coenzyme A dehydrogenase deficiency (LCHAD),
which results in an insufficient mitochondrial oxidation of fatty
acids required for ketogenesis, once the liver glycogen source
has been exhausted (Holub et al. 2005).
Low platelet count
Thrombocytopenia (platelets 150 109/l) in pregnancy may be
caused by gestational thrombocytopenia (59%), immune throm-
bocytopenic purpura (11%), pre-eclampsia (10%) or HELLP syn-
drome (12%) (Thangaratinam et al. 2011). Platelets 100 109/l
are relatively rare in pre-eclampsia and gestational thrombocy-
topenia, frequent in immune thrombocytopenic purpura and
obligatory in the HELLP syndrome (according to the Sibai
definition) (Sibai 2004b). Decreased platelets count in the HELLP
syndrome is due to their increased consumption. Platelets are
activated, and adhere to damaged vascular endothelial cells,
resulting in increased platelet turnover with shorter lifespan
(Baxter and Weinstein 2004).
As previously mentioned, HELLP syndrome is a disease
with multisystemic expression. Most of the findings are attrib-
uted to the irregular vascular tone, the extensive vasospasm and
moreover, to the coagulation deficiency (Bick 2000; Franchini
2006). There seems to be an insult which progressively leads to
microvascular damage and intravascular platelet aggregation,
triggering a chain-reaction cascade, with the initial precipitating
factors remaining a matter of debate.
The activation of platelets, co-existing with the release of
thromboxane A and serotonin, leads to agglutination of the
platelets and, as supposed, to a decrease in their number; a fact
which stands for a major diagnostic element of the syndrome
(Baxter and Weinstein 2004). Due to the platelet activation,
the homeostatic mechanisms result in bone marrow and lead to
an increased number of released megakaryocytes circulating in
the vessels, resulting in circulation of platelets with a shorter mean
lifespan, since the newborn cells are adhered to the exposed col-
lagen of the traumatised endothelium. This condition explains the
thrombocytopenia present in patients with HELLP syndrome.
Many scientists believe that there is an alteration in the
maternofetal balance, provided that the existence of the syn-
drome is an acute rejection of the fetus by the immune system
of the mother (Steinborn 2001; Bonney 2007). In accordance
with this perception, studies have demonstrated the presence
of increased tissue plasminogen activator levels in addition to
plasminogen activator inhibitor-1 production, elevated sVEGR-1
and C5a multimers of the complement, increased circulating
amount of von Willebrand factor multimers and high fetally-
derived soluble HLA antigens levels (sHLA-DR) in the maternal
plasma (Hulstein et al. 2003; Fang et al. 2008b; Molvarec et al.
2009; Steinborn et al. 2000).
Classification
Nowadays, there are two classification systems used to categorise
the patients with possible HELLP syndrome. They were proposed
by researchers in the universities of Mississippi and Tennessee,
in the mid-1980s, to enable professionals to identify and classify
patients, to guide the therapeutic interventions and to provide
a solid platform for comparison between clinics worldwide
(Table I) (Sibai 1990, 2004a; Martin et al. 2006).
The Tennessee Classification categorises patients in complete
and partial HELLP syndrome, according to the presence of
the following criteria: (1) thrombocytopenia; (2) liver dysfunc-
tion; (3) haemolysis. There might be an expression of complete
HELLP syndrome (all of the three parameters) or partial HELLP
syndrome (only one or two parameters).
The Mississippi Triple Class System divides patients into
three categories, by categorising them according to the thrombo-
cytes counted with the laboratory screening: class I, class II and
class III. There shall be a distinct elevation of LDH ( 600 IU/l).
Moreover, there is a specific detail in the present classification,

Table I. Main diagnostic criteria of the HELLP syndrome.
HELLP
class Tennessee classication Mississippi classication
1 PLTs 100 109/l PLTs 50 109/l
AST 70 IU/l AST or ALT 70 IU/l
LDH 600 IU/l LDH 600 IU/l
2 PLTs 100 109/l and 50 109/l
AST or ALT 70 IU/l
LDH 600 IU/l
3 PLTs 150 109/l and 100 109/l
AST or ALT 40 IU/l
LDH 600 IU/l
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PLTs, platelets; AST, aspartate aminotransferase; LDH, lactate dehydrogenase; ALT,
alanine aminotransferase.
since there is no difference when taking into account the levels
of AST and ALT. Both count as equal, only one elevated is needed
in order to classify the patient.
The introduction and clinical application of these classifi-
cation systems enabled the standardisation of the diagnosis of
HELLP syndrome. Nevertheless, the complex pathogenesis of the
syndrome may require additional and more specific laboratory
examinations, for instance assessment of circulating autoanti-
bodies or special clotting tests.
Diagnosis
The diagnosis of HELLP syndrome is suspected in pregnant
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women with signs of pre-eclampsia or even eclampsia, com-
bined with the following triad of laboratory findings: haemo-
lysis, elevated liver function tests and low platelet count (Nunes
et al. 2005; van Runnard Heimel et al. 2005; Haram et al. 2009).
However, these findings can be encountered both in antepar-
tum and postpartum patients (Baxter and Weinstein 2004). As
expected, the more extended the laboratory abnormalities, the
greater the risk for the patient.
Thrombocytopenia varies among the patients and could be
the first indicator to the physician that there is an emerging
situation. A platelet count 100,000/l gives more confidence
to the physician to safely diagnose the underlying cause of
the abnormal blood test results, in accordance to the clinical mani-
festations of thrombocytopenia, which may have been evident
by the time of laboratory confirmation (Martin et al. 1999).
Moreover, the disturbance of liver function, indicated by
the laboratory results of elevated LDH and liver aminotrans-
ferases, significantly increases clinical suspicion. Also, the
severe anaemia presented as a low haematocrit and abnormal
peripheral blood smear lead to the diagnosis. It must be pointed
out that the abnormal coagulation tests due to consumptive
coagulopathy, the elevated serum creatinine levels and lastly,
the proteinuria in urine, are some other indicators that ring the
bell of the physician (Prakash et al. 2010). In 30% of cases, a mod-
erate increase in gamma-glutamyl transpeptidase (GT), alkaline
phosphatase and serum bilirubin is found (ChNg et al. 2002).
Patients may present with nonspecific symptoms, mostly
nausea, vomiting, malaise/fatigue and viral-like symptoms, as
well as more specific ones, as mid-epigastric/right upper quad-
rant discomfort, blurred vision, altered consciousness, clonus,
bleeding diathesis, and pulmonary oedema, abdominal disten-
sion and hypertension or hypovolaemic shock (Thangaratinam
et al. 2011). Many patients report a history of malaise some days
before presentation (Sibai 2004a). Up to 3060% of women have
headache and about 20% visual symptoms (Sibai 2004b). The
symptoms usually continuously progress and their intensity often
HELLP syndrome 333
changes spontaneously. The HELLP syndrome is characterised
by exacerbation during the night and recovery during the day
(Koenen et al. 2006).
As previously mentioned, HELLP syndrome may appear
only with mild symptoms, which can misguide the physicians
to other pathological conditions, especially to conditions
which present as acute abdomen (gastroenteritis, cholecystitis,
pancreatitis, upper urinary tract infection). Hepatitis, thrombotic
thrombocytopenic purpura of haemolytic uraemic syndrome,
as well as pregnancy-related diseases (benign thrombocytopenia,
acute fatty liver disease) can easily mislead to a wrong diagnosis.
It should not be forgotten that thrombocytopenia of immuno-
logical causes can mimic the HELLP syndrome (lupus erythe-
matosus, antiphospholipid syndrome), highlighting the need
for accurate differential diagnosis of the syndrome.
Thus, it is evident an immediate response is profound in this
situation. A chemistry panel including blood nitrogen urea,
creatinine, liver function enzymes, LDH, glucose, total bilirubin
and uric acid is the first step of the approach to the syndrome.
A complete evaluation of the blood parameters is also essential,
indicating the abnormalities in the erythrocyte count, the periph-
eral smear, PT and aPTT prolongation, and fibrinogen levels
(Poo and Gngora 2006).
Liver imaging is important for the evaluation of subcapsular
or intraparenchymal haemorrhage and hepatic rupture.
Diagnostic tools include ultrasound (U/S), computed tomog-
raphy (CT) and magnetic resonance imaging (MRI) (Martin
et al. 1999; Gilboa et al. 2006). In pregnant women, U/S and MRI
are preferred due to the absence of ionising radiation. CT is the
preferred method of imaging in the postpartum period (Martin
et al. 1999; Maher et al. 2004). Transabdominal U/S evidences
intrahepatic haematomas as hypoechogenic structures. CT or
MRI can detect hemoperitoneum, intrahepatic haematoma,
and an irregular interface between the normal hepatic paren-
chyma and intrahepatic haematoma corresponding to the cap-
sule rupture site (Maher et al. 2004). Hepatic arteriography, an
invasive procedure, can establish the site of haemorrhage and is
only performed before arterial embolisation (Maher et al. 2004).
Liver biopsy has a risk of haemorrhage and hepatic rup-
ture. Periportal haemorrhage, focal parenchymatous necrosis
and macrovesicular steatosis can be observed in one-third of
patients. Fibrin deposits and hyaline deposits are shown by immu-
nofluorescence at the level of liver sinusoids. Liver specimens
show a positive reaction with IL-1, IL-8, TNF- and neutrophil
elastase antibodies (Jurez-Azpilcueta et al. 2003).
Differential diagnosis
The HELLP syndrome may be misdiagnosed as viral hepatitis,
cholangitis and other acute diseases (Table II) (Magann and
Martin 1999; Haram et al. 2009). Other less common, but seri-
ous conditions that may mimic HELLP, include immunologic
thrombocytopenia, acute fatty liver of pregnancy, haemolytic
uraemic syndrome, thrombotic thrombocytopenic purpura,
and systemic lupus erythematosus (Baxter and Weinstein 2004;
Sibai 2004b). These conditions are associated with high maternal
mortality and may cause long-term sequelae (Sibai 2004b). They
may be mistaken for HELLP syndrome and a careful diagnostic
evaluation is required, as their therapy is quite different.
Complications, morbidity, and mortality
HELLP syndrome may result in the manifestation of various
life-threatening complications, such as placental abruption, pul-
monary oedema followed by acute respiratory distress, dissemi-
nated intravascular coagulation (DIC), cerebral haemorrhage,
 334 S. Aloizos et al.
Table II. Dierential diagnosis of the HELLP syndrome.
1 Diseases related to pregnancy Benign thrombocytopenia of
pregnancy
Acute fatty liver of pregnancy
2 Diseases not related to Virus hepatitis
pregnancy Cholangitis
Cholecystitis
Acute pancreatitis
Gastritis
Gastric ulcer
Upper urinary tract infection
3 Thrombocytopenia Immunological thrombocytopenia
Folate deciency
Antiphospholipid syndrome
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Systemic lupus erythematosus
4 Rare diseases that mimic Haemolytic uraemic syndrome
HELLP syndrome Thrombotic thrombocytopenic
purpura
septic shock, acute renal failure and hepatic haemorrhage due
to hepatic rupture (Barton and Sibai 2009; Zeeman 2009).
As we noticed, differences in the outcome of the neonates
depend on the study publication, while a low gestational age at
delivery is the main problem rather than HELLP itself. Infants
born to mothers with the HELLP syndrome may develop throm-
bocytopenia and neurological complications. However, most
neonates born to women with HELLP have a normal long-term
development.
Nevertheless, up to the present time, although the severity of
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the clinical manifestations of the syndrome would be reason-
ably regarded as a prognostic factor of fetal mortality, the estab-
lishment of relative predictive scores still remains a matter of
scientific debate.
Mother
The most frequent maternal complication of HELLP syndrome
is DIC, present in about 40% of the patients (Garg et al. 2009),
being itself catastrophic, which may lead to the establishment
of multi-organ dysfunction syndrome. In a large retrospec-
tive cohort study comprising 442 pregnancies complicated by
the HELLP syndrome, the maternal mortality was 1.1% (Sibai
et al. 1993), which is in accordance with other reports (Martin
et al. 2003; Ycesoy et al. 2005; Sibai 2004a). However, higher
maternal mortality, up to 25%, has been reported (van Runnard
Heimel et al. 2005). The perinatal mortality and morbidity among
pregnant women with HELLP syndrome is primarily dependent
on the gestational age when the condition develops (Abramovici
et al. 1999; Aslan et al. 2004). Unexpected rapid death from
HELLP may require forensic expertise (Simic et al. 2005).
Haemorrhage or stroke have been reported to be the primary
cause of death in 26% and the most contributing factor in another
45% of the deaths (Isler et al. 1999).
Another possibly devastating complication is hepatic hae-
morrhage, which may result in hepatic rupture, a complication
that can significantly increase both maternal and fetal mor-
bidity-mortality (Pavlis et al. 2009; Zeirideen and Kadir 2009)
(maternal mortality rate in hepatic rupture ranges from 18%
to 86%) (Mihu et al. 2007). In the majority of these cases, the
right hepatic lobe is most frequently affected, up to a rate of 75%
(Poo and Gngora 2006). The source of haemorrhage may be
intrahepatic, due to hepatic infarction, leading to the formation
of haematomas which may accumulate in retroperitoneum;
nevertheless, in cases of self-restrained hepatic rupture, may
present as a subcapsular or perihepatic haematoma. Although
pre-eclampsia is known to occur more often in primigravidas,
spontaneous liver rupture usually occurs in multigravidas
(Neerhof et al. 1989). However, this condition may also occur in
the first pregnancy. Most liver-related complications of HELLP
syndrome occur during the 3rd trimester or late in the 2nd
trimester, while few cases have been reported during the immedi-
ate postpartum period (Nunes et al. 2005).
Uncontrolled bleeding after hepatic rupture is a critical
clinical condition and is associated with increased mortality for
both the pregnant mother and the fetus. Being one of the most
severe consequences of HELLP syndrome, hepatic rupture is
reported in about 200 cases in the English language literature
(Poo and Gngora 2006). Most of the time it occurs during
the late 2nd or the 3rd trimester, but it has also been reported
during the immediate postpartum period (Reck et al. 2001). In
this case, and if failure of conservative or minimally-invasive
treatment occurs, emergency surgery is likely to be the only
therapeutic option. It is very important to remember that the
risk of hepatic rupture in patients with HELLP syndrome is
not reduced after labour or the emergency extraction of the
fetus, but it may present 2448 h after labour and/or complete
regression of the syndrome.
Neonate
Regarding the fetus-related complications of HELLP syndrome,
the most common ones are the necessity of pre-term delivery;
severe neonatal hypoglycaemia; thrombocytopenia; respira-
tory distress syndrome; low birth weight; hyperbilirubinaemia;
bronchopulmonary dysplasia; intraventricular bleeding; necro-
tising enterocolitis and even neonatal death (intrauterine
or postpartum) (Aliefendiolu et al. 2000; Aslan et al. 2004;
Gul et al. 2005). The clinical condition of the neonate in the
vast majority of the cases requires hospitalisation in neonatal
intensive care units, underlining the urgency of an effective
multidisciplinary medical approach. However, it does not
appear that HELLP syndrome increases neonatal mortality,
irrespective of gestational age, and data regarding neonatal
outcomes such as respiratory distress syndrome, intraventricu-
lar haemorrhage, necrotising enterocolitis and sepsis, are con-
flicting (Haram et al. 2009).
Neonates delivered before 32 completed weeks gestation,
have the highest risk of perinatal death (Abramovici et al.
1999; Aslan et al. 2004). Gul et al. (2005) reported a perinatal
mortality of 34% before 32 weeks gestation, and 8% after the
32nd gestational week. Prematurity, placental insufficiency,
with or without intrauterine growth restriction and abruption
placentae, are the leading causes of neonatal death (Magann
and Martin 1999; Baxter and Weinstein 2004). Hepatic rupture
has a perinatal mortality that can reach 80% (Mihu et al. 2007).
Neonatal thrombocytopenia occurs in between 15% and 38%
of cases (Harms et al. 1995; Ertan et al. 2002) and is a significant
risk factor for both intraventricular haemorrhage and long-
term neurological complications (Ertan et al. 2002; Singhal
et al. 2004).
Other authors inform that infants born to mothers with the
HELLP syndrome are not at increased risk of morbidity com-
pared with otherwise healthy infants of the same gestational
age (Gortner et al. 1992; Harms et al. 1995; Abramovici et al.
1999) and that gestational age at delivery and birth weight
primarily affect perinatal mortality, rather than the severity
of the hypertensive disease (Osmanagaoglu et al. 2004).
Abramovici et al. (1999) indicated that neonatal morbidity or
death is directly related to gestational age at delivery and showed
that prenatal management in HELLP syndrome improved
 HELLP syndrome 335

neonatal outcome in cases with advanced gestational age.
Kandler et al. (1998) reported that in the time span between
6 and 72 months (median 24 months) after delivery, 90% of
children born from mothers with HELLP showed normal devel-
opment or only minor disabilities.
Treatment
The management of patients with pre-eclampsia and HELLP syn-
drome is controversial. Most therapeutic modalities are similar to
those applied for severe pre-eclampsia. Patients should be imme-
diately referred to a tertiary care centre (OBrien and Barton 2005).
Treatment should be performed in intensive care units (ICUs)
with dialysis and ventilatory support in severe cases, and consist of
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tions, which may lead to systematic dysfunction (OBrien and
Barton 2005; Mihu et al. 2007).
When one or more of the numerous complications are present,
an aggressive approach and treatment will minimise the risks of
both mother and the fetus. A combination of plasma exchanges
and fresh frozen plasma units is able to sustain the platelet count
and the fluid restriction will comprehend to avoid the cerebral
oedema (Bayraktarolu et al. 2006; Myers 2010). In cases of con-
comitant renal failure, haemodialysis is the gold standard thera-
peutic approach. For patients with a platelet count 70,000/l,
or with coagulation parameters far from the normal ranges, the
spinal or epidural anaesthesia is not suggested, due to possible
bleeding. When needed, transfusion of packed erythrocytes

is obligatory for the patient, in order to sustain a preferable

plasma expanders, antithrombotic agents, heparin, antithrombin,
count of erythrocytes. Finally, recent studies propose the use of
aspirin in low doses, prostacyclin, immunosuppressive agents,
activated recombinant factor VII, as a measure to minimise the
steroids, fresh frozen plasma, dialysis. Whether the treatment
risk of uncontrolled haemorrhage or haematoma formation
approach should be conservative or aggressive remains controver-
(Dart et al. 2004; Merchant et al. 2004).
sial (Magann and Martin 1999; OBrien and Barton 2005). What is
In case hepatic complications occur, the continuous
widely believed is that the wait-and-see status should not exceed a
monitoring of the patient is the first step to the successful con-
certain length of time and a prominent approach to the syndrome
frontation of a possible surgical emergency. In case the patient
should include an enforced delivery or caesarean section.
is admitted for caesarean section, the surgeon should always
For pregnant women who have completed 34 weeks
examine the liver; if a rather small liver haematoma is detected,
gestation and where there is evidence of fetal lung maturity,
with Glissons capsule being intact, its evacuation is not required
delivery is the only solution proposed; most of the time it is the
(Mihu et al. 2007). In any other case, evacuation is recom-
definitive treatment of the syndrome (Doshi and Zucker 2003;
mended, so the extension or the subsequent hepatic rupture
Mihu et al. 2007). If there is no obstetric complication present,
can be prevented. In general, when hepatic complications are
vaginal delivery is preferred (Mihu et al. 2007). However, expect-
to be treated by surgical procedures, the surgeons may choose
ant management before completed 34 weeks gestation may be an
between the so-called conservative approaches, such as liver
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acceptable option in selected cases, if it is performed in tertiary

tamponade or packing (with the lowest mortality rate, about
care units under close maternal and fetal surveillance (Paruk
2030%); fibrin gluing of the area; suture ligation or placement
and Moodley 2000; Haddad and Sibai 2005). Possible advan-
of polypropylene meshes. Moreover, there are more aggressive
tages due to limited prolongation of pregnancy should be care-
approaches, such as partial resection; hepatic lobectomy and,
fully weighed against the increased risks for maternal and fetal
in specific cases, orthotopic liver transplantation (Reck et al.
complications (Haram et al. 2009). If the maternal condition
2001; Poo and Gngora 2006; Miguelote et al. 2009).
worsens, immediate caesarean section is inevitable (Van Dam
New studies show that treatments under radiological guid-
et al. 1989; Haddad and Sibai 2005). Conservative treatment is
ance also offer a plausible solution. Hepatic artery ligation,
contraindicated in women with DIC (Haddad and Sibai 2005).
embolisation and the use of argon beam coagulation are possible
For patients less than 34 weeks gestation, the use of corticos-
therapeutic weapons in the arsenal of the surgeons (Poo and
teroids is recommended in order to accelerate fetal pulmonary
Gngora 2006).
maturity and reduce the perinatal complications of the fetus
(Martin et al. 2006). The improvement of thrombocytopenia has
been more frequently observed for the low doses compared with Conclusions
the high doses of corticoids (Isler et al. 2001; Martin et al. 2003).
Despite all recent advances in its early diagnosis and treatment,
In an analysis performed on 170 patients with HELLP syndrome,
HELLP syndrome is still a serious, life-threatening complication
the maternal advantage of corticotherapy was an extension of
of the 3rd trimester of pregnancy. With its pathogenesis remain-
the time period between hospital admission and the induction
ing not fully elucidated, HELLP syndrome remains a complex
of delivery, and the fetal advantage was an increase in weight
diagnostic and therapeutic challenge. It appears that the success-
at birth. The authors concluded that steroid therapy should only
ful confrontation lies upon the increased clinical suspicion of
be administered to very well selected cases, since it does not
the obstetrician and the aggressive treatment, with conservative,
improve prognosis (Vidaeff and Yeomans 2007; Matchaba and
minimally invasive or even surgical approach. Finally, although it
Moodley 2009).
is essential that high-risk pregnant women should be closely moni-
Plasmapheresis with fresh frozen plasma has been proposed
tored in order to prevent the development of the syndrome, it is of
as a therapeutic method for patients who show a progres-
vital importance to assess the need of patient counselling before
sive increase in bilirubinaemia, serum creatinine, have severe
pregnancy, especially in cases with a positive history of develop-
thrombocytopenia and for patients in whom HELLP syndrome
ment of pre-eclampsia/eclampsia in previous pregnancies.
persists for more than 72 h postpartum, but it has no favourable
results in patients with fulminant haemolysis (Eser et al. 2005;
Declaration of interest: The authors report no conflicts of inter-
Bayraktarolu et al. 2006).
est. The authors alone are responsible for the content and writing
The administration of magnesium sulphate intravenously
of the paper.
as a measure of prophylaxis against seizures is considered to be
essential and if needed (Peral et al. 2006), the patient should
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