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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 endothelial damage (Hulstein et al. 2003). Recently, fetal disor-
liver enzymes and low platelets, a variant of pre-eclampsia/ ders of mitochondrial fatty acid oxidation have been associated
eclampsia, is a multisystemic disorder with an incidence of up with obstetric complications including HELLP syndrome; their
to 0.9% in all pregnancies and occurs in about one-quarter of action is not yet understood (Tyni et al. 1998).
pre-eclamptic patients. Most obstetricians presume that HELLP The aim of this paper is to summarise currently available
derives from an autoimmune reaction, leading to a materno data regarding epidemiology, aetiology, pathology, clinical pre-
fetal imbalance, with accompanying aggregation of platelets, sentation, classification, management and prognosis of HELLP
endothelial malfunction along with inborn errors of fatty acid syndrome and to discuss diagnostic problems and management
oxidative metabolism. HELLP is characterised by high mortal- doubts that affect clinicians and obstetricians.
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 Methods
or the fetal and/or maternal conditions deteriorate. Vaginal A systematic literature search for clinical reports and reviews
For personal use only.

delivery is preferable. Standard corticosteroid treatment is of on HELLP syndrome published between 1989 and 2011 were
uncertain clinical value in maternal HELLP syndrome. Future identified using PubMed and the Cochrane databases, and addi-
observations and research results may shed more light on tional cited works not detected in the initial search were obtained.
improving our understanding of the aspects of HELLP syndrome. Search words were HELLP syndrome, and HELLP syndrome
combined with diagnosis, clinical symptoms, complications,
Keywords: Eclampsia, HELLP syndrome, liver, management,
morbidity, mortality, management, treatment, prognosis
pregnancy
and recurrence. Publications were selected for review based
on original research, highly regarded earlier publications and
comprehensive reviews, while those publications considered as
Introduction relevant were used at the authors discretion.
Haemolysis, elevated liver enzymes and low platelets (HELLP)
syndrome, was first defined by Weinstein in 1982. It is a multi- Pathophysiology
systemic disorder which remains an important cause of maternal Haemolysis
and perinatal mortality and morbidity. The rapid decrease of the erythrocyte number during the full
Many researchers and clinicians consider this particular syn- presentation of HELLP syndrome is believed to result from
drome as a variant of another peculiar disease of pregnancy, pre- the cellular damage due to fibrin deposition, leading to fragmen-
eclampsia (Habli et al. 2009), whereas other physicians consider tation of the red blood cells (Doshi and Zucker 2003; Moake
it to be a totally different expression of the rejection of the fetus 2009). This particular finding is consistent with microangio-
in the late trimester of pregnancy, due to maternofetal immune pathic haemolytic anaemia, with the distorted red cells seen in
imbalance (Tung et al. 2001; Fang et al. 2008b). the microscope (Burr cells) along with schistocytes, as well as
HELLP syndrome has an incidence of 0.170.85% of all preg- polychromasia on peripheral blood smear (Baxter and Weinstein
nancies, while the risk of recurrence in a subsequent pregnancy 2004). In recent studies, many similarities between haemolytic
is about 1927% (Isler et al. 2003). It is a multisystem disease uraemic syndrome and HELLP syndrome have been reported
which is characterised by abnormal vascular tone, vasospasm in the field of molecular mechanisms between blood cells,
and coagulation defects. Although, its pathophysiology remains which interact with one another, proposing a quite similar defec-
partially unknown, activation of endothelial cells may lead to tive regulation between these two different pathological entities
release of von Willebrand factor multimers, which are highly (Fang et al. 2008a,b).
reactive with platelets. The syndrome seems to be the final Widely accessible markers of haemolysis can be the levels
manifestation of some insult that leads to microvascular endothe- of lactic dehydrogenase and indirect (unconjugated) bilirubin.
lial damage and intravascular platelet activation. With platelet Moreover, the presence of reticulocytes can be explained as an
activation, thromboxane A and serotonin are released, causing effort of the organism to withstand this condition and release
vasospasm, platelet agglutination and aggregations and further the immature cells as a compensatory reflect (Haram et al.

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
332 S. Aloizos et al.

2009). According to recent findings, a more sensitive and precise Low platelet count
indicator of haemolysis is the unusually low level of haptoglobin Thrombocytopenia (platelets 150 109/l) in pregnancy may be
(van Runnard Heimel et al. 2005); nevertheless, its assessment caused by gestational thrombocytopenia (59%), immune throm-
is rarely included in the routine laboratory screening of the bocytopenic purpura (11%), pre-eclampsia (10%) or HELLP syn-
patients. 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
Liver enzymes elevation
obligatory in the HELLP syndrome (according to the Sibai
The liver involvement in HELLP syndrome is similar to the
definition) (Sibai 2004b). Decreased platelets count in the HELLP
alterations presented in pre-eclampsia. Typical histological
syndrome is due to their increased consumption. Platelets are
findings of liver biopsies of patients include fibrin deposition
activated, and adhere to damaged vascular endothelial cells,
intravascularly, which can lead to obstruction of the sinusoids
resulting in increased platelet turnover with shorter lifespan
due to hyaline deposits of fibrin-like material, blood congestion
(Baxter and Weinstein 2004).
and elevated intrahepatic pressure, resulting in focal and/or
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As previously mentioned, HELLP syndrome is a disease


periportal hepatocyte necrosis (Tsokos et al. 2002; Baxter and
with multisystemic expression. Most of the findings are attrib-
Weinstein 2004; OBrien and Barton 2005). Moreover, Glissons
uted to the irregular vascular tone, the extensive vasospasm and
capsule may be engorged and distended, producing symptoms
moreover, to the coagulation deficiency (Bick 2000; Franchini
such as mid-epigastric pain and tenderness (Suarez et al. 2002).
2006). There seems to be an insult which progressively leads to
Elevation of liver enzymes may reflect the haemolytic
microvascular damage and intravascular platelet aggregation,
process as well as liver involvement. Haemolysis contributes sub-
triggering a chain-reaction cascade, with the initial precipitating
stantially to the elevated levels of lactate dehydrogenase (LDH),
factors remaining a matter of debate.
whereas enhanced aspartate aminotransferase (AST) and alanine
The activation of platelets, co-existing with the release of
aminotransferase (ALT) levels are mostly due to liver injury.
thromboxane A and serotonin, leads to agglutination of the
Plasma glutathione S-transferase-a1 (GST-a1) may provide a
platelets and, as supposed, to a decrease in their number; a fact
more sensitive indicator for acute liver damage than AST and
which stands for a major diagnostic element of the syndrome
ALT, and allow earlier recognition (Prakash et al. 2010). How-
(Baxter and Weinstein 2004). Due to the platelet activation,
ever, measurement of GST-a1 is not widely available, and has
the homeostatic mechanisms result in bone marrow and lead to
not yet found its place in the routine diagnostic procedure
an increased number of released megakaryocytes circulating in
(Molvarec et al. 2009).
the vessels, resulting in circulation of platelets with a shorter mean
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With respect to the elevation of liver enzymes, AST and


lifespan, since the newborn cells are adhered to the exposed col-
ALT may elevate up to 700 U/ml or more, indicating the degree
lagen of the traumatised endothelium. This condition explains the
of liver malfunction; in addition, elevation of LDH levels can
thrombocytopenia present in patients with HELLP syndrome.
not only suggest liver malfunction at cellular level, but may
Many scientists believe that there is an alteration in the
also serve a marker of erythrocyte damage. Recently, the mea-
maternofetal balance, provided that the existence of the syn-
surement of GST-a1 is proposed as being a more sensitive marker
drome is an acute rejection of the fetus by the immune system
of liver damage, but has not yet found its place in the clinical
of the mother (Steinborn 2001; Bonney 2007). In accordance
procedure (Knapen et al. 1999). However, up to the present
with this perception, studies have demonstrated the presence
time, neither significant correlation between the histological
of increased tissue plasminogen activator levels in addition to
findings, biopsy results, clinical presentation and the laboratory
plasminogen activator inhibitor-1 production, elevated sVEGR-1
findings nor with the maternal/fetal outcome, have been solidly
and C5a multimers of the complement, increased circulating
founded (Doshi and Zucker 2003; Baxter and Weinstein 2004;
amount of von Willebrand factor multimers and high fetally-
Martin et al. 2006).
derived soluble HLA antigens levels (sHLA-DR) in the maternal
The significantly increased levels of tissue plasminogen
plasma (Hulstein et al. 2003; Fang et al. 2008b; Molvarec et al.
activator and plasminogen activator inhibitor-1 (PAI-1) in the
2009; Steinborn et al. 2000).
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 Classification
(Zhou et al. 2002). Nowadays, there are two classification systems used to categorise
Therefore, other theories have been proposed, suggesting the patients with possible HELLP syndrome. They were proposed
that HELLP syndrome might be an acute inflammatory condi- by researchers in the universities of Mississippi and Tennessee,
tion; an assumption which might explain the liver cell apoptosis in the mid-1980s, to enable professionals to identify and classify
(Mihu et al. 2007). Research results of CD95 antigen-blockage patients, to guide the therapeutic interventions and to provide
(CD95 is produced in the placenta) in mothers presenting the a solid platform for comparison between clinics worldwide
syndrome are in favour of this particular opinion (Mihu et al. (Table I) (Sibai 1990, 2004a; Martin et al. 2006).
2007). Lastly, an inborn error of fatty acid oxidative metabolism The Tennessee Classification categorises patients in complete
in fetuses born in the context of HELLP syndrome supports and partial HELLP syndrome, according to the presence of
the approach that the theory of maternofetal imbalance can the following criteria: (1) thrombocytopenia; (2) liver dysfunc-
be preserved as a viable explanation of the pathophysiology of tion; (3) haemolysis. There might be an expression of complete
the syndrome. A mutation allows a long chain fatty acid, i.e. a HELLP syndrome (all of the three parameters) or partial HELLP
3-hydroxyacyl metabolite that is produced by the fetus or the syndrome (only one or two parameters).
placenta, to accumulate at maternal level due to a long chain The Mississippi Triple Class System divides patients into
3-hydroxyacyl coenzyme A dehydrogenase deficiency (LCHAD), three categories, by categorising them according to the thrombo-
which results in an insufficient mitochondrial oxidation of fatty cytes counted with the laboratory screening: class I, class II and
acids required for ketogenesis, once the liver glycogen source class III. There shall be a distinct elevation of LDH ( 600 IU/l).
has been exhausted (Holub et al. 2005). Moreover, there is a specific detail in the present classification,
HELLP syndrome 333
Table I. Main diagnostic criteria of the HELLP syndrome. changes spontaneously. The HELLP syndrome is characterised
HELLP by exacerbation during the night and recovery during the day
class Tennessee classication Mississippi classication (Koenen et al. 2006).
As previously mentioned, HELLP syndrome may appear
1 PLTs 100 109/l PLTs 50 109/l only with mild symptoms, which can misguide the physicians
AST 70 IU/l AST or ALT 70 IU/l to other pathological conditions, especially to conditions
LDH 600 IU/l LDH 600 IU/l which present as acute abdomen (gastroenteritis, cholecystitis,
2 PLTs 100 109/l and 50 109/l pancreatitis, upper urinary tract infection). Hepatitis, thrombotic
AST or ALT 70 IU/l thrombocytopenic purpura of haemolytic uraemic syndrome,
LDH 600 IU/l
as well as pregnancy-related diseases (benign thrombocytopenia,
3 PLTs 150 109/l and 100 109/l
acute fatty liver disease) can easily mislead to a wrong diagnosis.
AST or ALT 40 IU/l
LDH 600 IU/l
It should not be forgotten that thrombocytopenia of immuno-
logical causes can mimic the HELLP syndrome (lupus erythe-
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PLTs, platelets; AST, aspartate aminotransferase; LDH, lactate dehydrogenase; ALT, matosus, antiphospholipid syndrome), highlighting the need
alanine aminotransferase.
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,
since there is no difference when taking into account the levels
creatinine, liver function enzymes, LDH, glucose, total bilirubin
of AST and ALT. Both count as equal, only one elevated is needed
and uric acid is the first step of the approach to the syndrome.
in order to classify the patient.
A complete evaluation of the blood parameters is also essential,
The introduction and clinical application of these classifi-
indicating the abnormalities in the erythrocyte count, the periph-
cation systems enabled the standardisation of the diagnosis of
eral smear, PT and aPTT prolongation, and fibrinogen levels
HELLP syndrome. Nevertheless, the complex pathogenesis of the
(Poo and Gngora 2006).
syndrome may require additional and more specific laboratory
Liver imaging is important for the evaluation of subcapsular
examinations, for instance assessment of circulating autoanti-
or intraparenchymal haemorrhage and hepatic rupture.
bodies or special clotting tests.
Diagnostic tools include ultrasound (U/S), computed tomog-
Diagnosis raphy (CT) and magnetic resonance imaging (MRI) (Martin
et al. 1999; Gilboa et al. 2006). In pregnant women, U/S and MRI
The diagnosis of HELLP syndrome is suspected in pregnant
are preferred due to the absence of ionising radiation. CT is the
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women with signs of pre-eclampsia or even eclampsia, com-


preferred method of imaging in the postpartum period (Martin
bined with the following triad of laboratory findings: haemo-
et al. 1999; Maher et al. 2004). Transabdominal U/S evidences
lysis, elevated liver function tests and low platelet count (Nunes
intrahepatic haematomas as hypoechogenic structures. CT or
et al. 2005; van Runnard Heimel et al. 2005; Haram et al. 2009).
MRI can detect hemoperitoneum, intrahepatic haematoma,
However, these findings can be encountered both in antepar-
and an irregular interface between the normal hepatic paren-
tum and postpartum patients (Baxter and Weinstein 2004). As
chyma and intrahepatic haematoma corresponding to the cap-
expected, the more extended the laboratory abnormalities, the
sule rupture site (Maher et al. 2004). Hepatic arteriography, an
greater the risk for the patient.
invasive procedure, can establish the site of haemorrhage and is
Thrombocytopenia varies among the patients and could be
only performed before arterial embolisation (Maher et al. 2004).
the first indicator to the physician that there is an emerging
Liver biopsy has a risk of haemorrhage and hepatic rup-
situation. A platelet count 100,000/l gives more confidence
ture. Periportal haemorrhage, focal parenchymatous necrosis
to the physician to safely diagnose the underlying cause of
and macrovesicular steatosis can be observed in one-third of
the abnormal blood test results, in accordance to the clinical mani-
patients. Fibrin deposits and hyaline deposits are shown by immu-
festations of thrombocytopenia, which may have been evident
nofluorescence at the level of liver sinusoids. Liver specimens
by the time of laboratory confirmation (Martin et al. 1999).
show a positive reaction with IL-1, IL-8, TNF- and neutrophil
Moreover, the disturbance of liver function, indicated by
elastase antibodies (Jurez-Azpilcueta et al. 2003).
the laboratory results of elevated LDH and liver aminotrans-
ferases, significantly increases clinical suspicion. Also, the
Differential diagnosis
severe anaemia presented as a low haematocrit and abnormal
peripheral blood smear lead to the diagnosis. It must be pointed The HELLP syndrome may be misdiagnosed as viral hepatitis,
out that the abnormal coagulation tests due to consumptive cholangitis and other acute diseases (Table II) (Magann and
coagulopathy, the elevated serum creatinine levels and lastly, Martin 1999; Haram et al. 2009). Other less common, but seri-
the proteinuria in urine, are some other indicators that ring the ous conditions that may mimic HELLP, include immunologic
bell of the physician (Prakash et al. 2010). In 30% of cases, a mod- thrombocytopenia, acute fatty liver of pregnancy, haemolytic
erate increase in gamma-glutamyl transpeptidase (GT), alkaline uraemic syndrome, thrombotic thrombocytopenic purpura,
phosphatase and serum bilirubin is found (ChNg et al. 2002). and systemic lupus erythematosus (Baxter and Weinstein 2004;
Patients may present with nonspecific symptoms, mostly Sibai 2004b). These conditions are associated with high maternal
nausea, vomiting, malaise/fatigue and viral-like symptoms, as mortality and may cause long-term sequelae (Sibai 2004b). They
well as more specific ones, as mid-epigastric/right upper quad- may be mistaken for HELLP syndrome and a careful diagnostic
rant discomfort, blurred vision, altered consciousness, clonus, evaluation is required, as their therapy is quite different.
bleeding diathesis, and pulmonary oedema, abdominal disten-
sion and hypertension or hypovolaemic shock (Thangaratinam Complications, morbidity, and mortality
et al. 2011). Many patients report a history of malaise some days HELLP syndrome may result in the manifestation of various
before presentation (Sibai 2004a). Up to 3060% of women have life-threatening complications, such as placental abruption, pul-
headache and about 20% visual symptoms (Sibai 2004b). The monary oedema followed by acute respiratory distress, dissemi-
symptoms usually continuously progress and their intensity often nated intravascular coagulation (DIC), cerebral haemorrhage,
334 S. Aloizos et al.
Table II. Dierential diagnosis of the HELLP syndrome. pre-eclampsia is known to occur more often in primigravidas,
spontaneous liver rupture usually occurs in multigravidas
1 Diseases related to pregnancy Benign thrombocytopenia of
pregnancy
(Neerhof et al. 1989). However, this condition may also occur in
Acute fatty liver of pregnancy the first pregnancy. Most liver-related complications of HELLP
2 Diseases not related to Virus hepatitis syndrome occur during the 3rd trimester or late in the 2nd
pregnancy Cholangitis trimester, while few cases have been reported during the immedi-
Cholecystitis ate postpartum period (Nunes et al. 2005).
Acute pancreatitis Uncontrolled bleeding after hepatic rupture is a critical
Gastritis clinical condition and is associated with increased mortality for
Gastric ulcer both the pregnant mother and the fetus. Being one of the most
Upper urinary tract infection
severe consequences of HELLP syndrome, hepatic rupture is
3 Thrombocytopenia Immunological thrombocytopenia
Folate deciency
reported in about 200 cases in the English language literature
Antiphospholipid syndrome (Poo and Gngora 2006). Most of the time it occurs during
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Systemic lupus erythematosus the late 2nd or the 3rd trimester, but it has also been reported
4 Rare diseases that mimic Haemolytic uraemic syndrome during the immediate postpartum period (Reck et al. 2001). In
HELLP syndrome Thrombotic thrombocytopenic this case, and if failure of conservative or minimally-invasive
purpura 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
septic shock, acute renal failure and hepatic haemorrhage due not reduced after labour or the emergency extraction of the
to hepatic rupture (Barton and Sibai 2009; Zeeman 2009). fetus, but it may present 2448 h after labour and/or complete
As we noticed, differences in the outcome of the neonates regression of the syndrome.
depend on the study publication, while a low gestational age at
delivery is the main problem rather than HELLP itself. Infants Neonate
born to mothers with the HELLP syndrome may develop throm- Regarding the fetus-related complications of HELLP syndrome,
bocytopenia and neurological complications. However, most the most common ones are the necessity of pre-term delivery;
neonates born to women with HELLP have a normal long-term severe neonatal hypoglycaemia; thrombocytopenia; respira-
development. tory distress syndrome; low birth weight; hyperbilirubinaemia;
Nevertheless, up to the present time, although the severity of
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bronchopulmonary dysplasia; intraventricular bleeding; necro-


the clinical manifestations of the syndrome would be reason- tising enterocolitis and even neonatal death (intrauterine
ably regarded as a prognostic factor of fetal mortality, the estab- or postpartum) (Aliefendiolu et al. 2000; Aslan et al. 2004;
lishment of relative predictive scores still remains a matter of Gul et al. 2005). The clinical condition of the neonate in the
scientific debate. vast majority of the cases requires hospitalisation in neonatal
intensive care units, underlining the urgency of an effective
Mother multidisciplinary medical approach. However, it does not
The most frequent maternal complication of HELLP syndrome appear that HELLP syndrome increases neonatal mortality,
is DIC, present in about 40% of the patients (Garg et al. 2009), irrespective of gestational age, and data regarding neonatal
being itself catastrophic, which may lead to the establishment outcomes such as respiratory distress syndrome, intraventricu-
of multi-organ dysfunction syndrome. In a large retrospec- lar haemorrhage, necrotising enterocolitis and sepsis, are con-
tive cohort study comprising 442 pregnancies complicated by flicting (Haram et al. 2009).
the HELLP syndrome, the maternal mortality was 1.1% (Sibai Neonates delivered before 32 completed weeks gestation,
et al. 1993), which is in accordance with other reports (Martin have the highest risk of perinatal death (Abramovici et al.
et al. 2003; Ycesoy et al. 2005; Sibai 2004a). However, higher 1999; Aslan et al. 2004). Gul et al. (2005) reported a perinatal
maternal mortality, up to 25%, has been reported (van Runnard mortality of 34% before 32 weeks gestation, and 8% after the
Heimel et al. 2005). The perinatal mortality and morbidity among 32nd gestational week. Prematurity, placental insufficiency,
pregnant women with HELLP syndrome is primarily dependent with or without intrauterine growth restriction and abruption
on the gestational age when the condition develops (Abramovici placentae, are the leading causes of neonatal death (Magann
et al. 1999; Aslan et al. 2004). Unexpected rapid death from and Martin 1999; Baxter and Weinstein 2004). Hepatic rupture
HELLP may require forensic expertise (Simic et al. 2005). has a perinatal mortality that can reach 80% (Mihu et al. 2007).
Haemorrhage or stroke have been reported to be the primary Neonatal thrombocytopenia occurs in between 15% and 38%
cause of death in 26% and the most contributing factor in another of cases (Harms et al. 1995; Ertan et al. 2002) and is a significant
45% of the deaths (Isler et al. 1999). risk factor for both intraventricular haemorrhage and long-
Another possibly devastating complication is hepatic hae- term neurological complications (Ertan et al. 2002; Singhal
morrhage, which may result in hepatic rupture, a complication et al. 2004).
that can significantly increase both maternal and fetal mor- Other authors inform that infants born to mothers with the
bidity-mortality (Pavlis et al. 2009; Zeirideen and Kadir 2009) HELLP syndrome are not at increased risk of morbidity com-
(maternal mortality rate in hepatic rupture ranges from 18% pared with otherwise healthy infants of the same gestational
to 86%) (Mihu et al. 2007). In the majority of these cases, the age (Gortner et al. 1992; Harms et al. 1995; Abramovici et al.
right hepatic lobe is most frequently affected, up to a rate of 75% 1999) and that gestational age at delivery and birth weight
(Poo and Gngora 2006). The source of haemorrhage may be primarily affect perinatal mortality, rather than the severity
intrahepatic, due to hepatic infarction, leading to the formation of the hypertensive disease (Osmanagaoglu et al. 2004).
of haematomas which may accumulate in retroperitoneum; Abramovici et al. (1999) indicated that neonatal morbidity or
nevertheless, in cases of self-restrained hepatic rupture, may death is directly related to gestational age at delivery and showed
present as a subcapsular or perihepatic haematoma. Although that prenatal management in HELLP syndrome improved
HELLP syndrome 335

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