HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Official reprint from UpToDate®

www.uptodate.com © 2021 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

HELLP syndrome (hemolysis, elevated liver enzymes,

and low platelets)
Author: Baha M Sibai, MD
Section Editors: Charles J Lockwood, MD, MHCM, Keith D Lindor, MD
Deputy Editor: Vanessa A Barss, MD, FACOG

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Jun 2021. | This topic last updated: Aug 25, 2020.

INTRODUCTION

HELLP is an acronym that refers to a syndrome in pregnant and postpartum women

characterized by hemolysis with a microangiopathic blood smear, elevated liver enzymes,
and a low platelet count [1]. It probably represents a severe form of preeclampsia (
table 1A-B), but the relationship between the two disorders remains controversial. HELLP
may be a separate disorder from preeclampsia because as many as 15 to 20 percent of
patients with HELLP syndrome do not have antecedent hypertension or proteinuria [2-4].

Delivery eventually leads to resolution of signs and symptoms. Maternal complications are
primarily related to bleeding, which can include hepatic hemorrhage. Neonatal complications
are primarily related to the gestational age at delivery, which is commonly preterm.

This topic will focus on the clinical presentation, diagnosis, differential diagnosis, and
management of HELLP syndrome. Preeclampsia is reviewed in detail separately.

● (See "Preeclampsia: Clinical features and diagnosis".)

● (See "Preeclampsia: Management and prognosis".)
● (See "Preeclampsia with severe features: Expectant management remote from term".)

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 1 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

PREVALENCE

HELLP develops in 0.1 to 1.0 percent of pregnant women overall. Among women with severe
preeclampsia/eclampsia, 1 to 2 percent have microangiopathic hemolysis and thus can be
considered to have HELLP.

RISK FACTORS

A previous history of preeclampsia or HELLP is a risk factor for HELLP syndrome. (See
'Recurrence in subsequent pregnancies' below.)

A variety of genetic variants associated with an increased risk for HELLP syndrome has been
reported, but they have no role in clinical management [5]. (See 'Pathogenesis' below.)

In contrast to preeclampsia, nulliparity is not a risk factor for HELLP syndrome [6]. Half or
more of affected patients are multiparous.

PATHOGENESIS

The pathogenesis of HELLP syndrome is unclear. If it is a severe form of preeclampsia, it

likely has the same origin (see "Preeclampsia: Pathogenesis"). If it is a separate entity, it can
still have a similar origin, but it then diverges along a different pathway for unknown reasons
such that there is greater hepatic inflammation and greater activation of the coagulation
system than in preeclampsia [5,7,8].

A subset of HELLP syndrome may be related to thrombotic microangiopathy caused by

complement dysregulation, which may be treatable without prompt delivery of the fetus. In a
case report of a woman with severe early HELLP syndrome, treatment with eculizumab, a
targeted inhibitor of complement protein C5, was associated with marked clinical
improvement and complete normalization of laboratory parameters for 16 days, after which
HELLP recurred [9]. The authors chose this intervention based on the hypothesis that severe
preeclampsia/HELLP is a systemic inflammatory disorder and the complement cascade is a
key mediator, and the observation that women with mutations in complement regulatory

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 2 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

proteins appear to be at increased risk of severe preeclampsia [10]. Further research of

possible benefits and harms is warranted [11].

In less than 2 percent of patients with HELLP, the underlying etiology appears to be related to
fetal long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency [12,13]. In one case
series, all six pregnancies with fetal LCHAD deficiency developed severe maternal liver
disease (HELLP or acute fatty liver of pregnancy [AFLP]) [14]. These complications probably
were not due to chance or maternal heterozygosity for LCHAD deficiency alone because
three other pregnancies with unaffected fetuses among these mothers were uncomplicated.
In another case series in which 19 fetuses had LCHAD deficiency, 15 mothers (79 percent)
developed AFLP or the HELLP syndrome during their pregnancies [15]. Although these
findings inform theories about the pathogenesis of HELLP, evaluation for genetic variants
associated with LCHAD deficiency has no role in clinical management of women with HELLP.
(See "Acute fatty liver of pregnancy", section on 'Fetal long-chain 3-hydroxyacyl CoA
dehydrogenase (LCHAD) deficiency'.)

PATHOPHYSIOLOGY

Microangiopathy and activation of intravascular coagulation can account for all of the
laboratory findings in HELLP syndrome. Hepatic histology may show microvascular fibrin
deposition, neutrophilic infiltrate, fatty infiltration, lobular necrosis, and periportal
hemorrhage ( picture 1) [16]. Although renal dysfunction is not an essential diagnostic
criterion, microvascular dysfunction may also occur in the kidney and may increase its
vulnerability to an ischemic insult [17].

PATIENT PRESENTATION

Signs and symptoms — HELLP syndrome has a variable presentation ( table 2) [18]. The
onset of symptoms is usually rapid onset, and they progressively worsen.

Abdominal pain, which may be colicky, is the most common symptom and is present in most
patients. It may be localized to the midepigastrium, right upper quadrant, or below the
sternum [19]. The area may be tender on physical examination. Many patients also have

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 3 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

nausea, vomiting, and generalized malaise, which may be mistaken for a nonspecific viral
illness or viral hepatitis, particularly if the serum aspartate aminotransferase and lactate
dehydrogenase levels are markedly elevated. Less common symptoms include headache,
visual changes, jaundice, and ascites.

On physical examination, hypertension (defined as blood pressure ≥140/90 mmHg) and

proteinuria are present in approximately 85 percent of cases, but it is important to note that
either or both may be absent in women with otherwise severe HELLP syndrome [18].

Serious maternal morbidity may be present at initial presentation or develop shortly

thereafter. This includes disseminated intravascular coagulation, abruptio placentae, acute
kidney injury, pulmonary edema, subcapsular or intraparenchymal liver hematoma, and
retinal detachment [19]. (See 'Maternal outcome' below.)

Thrombocytopenia-related bleeding (mucosal, hematuria, petechial hemorrhages,

ecchymosis) is an unusual presentation [18].

Gestational age at onset — Symptoms typically develop between 28 and 37 weeks of

gestation, but onset in the late second trimester or at term/postpartum is also common. In a
large series including over 440 pregnancies complicated by the HELLP syndrome, 70 percent
occurred before delivery, approximately 80 percent of these cases occurred before <37
weeks, and fewer than 3 percent occurred between 17 and 20 weeks [19].

In the 30 percent of cases that occurred postpartum, most were diagnosed within 48 hours
of delivery, but occasionally as long as seven days after birth; 80 percent had evidence of
preeclampsia before delivery. Why some cases of HELLP and preeclampsia develop
postpartum is unknown and confusing since delivering the placenta initiates resolution of
the disease in most patients.

DIAGNOSTIC EVALUATION

In pregnant women with characteristic symptoms of HELLP (eg, right upper

quadrant/midepigastric pain, nausea, vomiting, generalized malaise) and/or new onset
hypertension in the second half of pregnancy or first postpartum week, we obtain the

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 4 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

laboratory tests that establish/exclude the diagnosis of HELLP. Because pain may precede
laboratory abnormalities by several hours, repeating the laboratory tests in four to six hours
can be helpful unless another cause for pain has been determined [20].

Laboratory work-up should include [18]:

● Complete blood count

● Peripheral smear
● Aspartate aminotransferase, alanine aminotransferase, bilirubin
● Creatinine

In patients with elevated liver chemistries, the author also obtains haptoglobin and lactate
dehydrogenase levels and coagulation studies (fibrinogen, prothrombin time, activated
partial thromboplastin time).

DIAGNOSIS

The diagnosis of HELLP syndrome is based upon the presence of all of the laboratory
abnormalities comprising its name (hemolysis with a microangiopathic blood smear
[fragmented red blood cells; ie, schistocytes, burr cells], elevated liver enzymes, and low
platelet count) in a pregnant/postpartum woman.

Pregnant/postpartum women who have some of the typical laboratory abnormalities but do
not meet all of the laboratory criteria described below are considered to have partial HELLP
syndrome [6]. These patients may progress and meet all criteria.

Laboratory criteria for diagnosis — We require the presence of the following criteria to
diagnose HELLP (Tennessee classification) [21]:

● Hemolysis, established by at least two of the following:

• Peripheral smear with schistocytes and burr cells ( picture 2)

• Serum bilirubin ≥1.2 mg/dL (20.52 micromol/L)
• Low serum haptoglobin (≤25 mg/dL) or lactate dehydrogenase (LDH) ≥2 times the
upper level of normal (based on laboratory-specific reference ranges)

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 5 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

• Severe anemia, unrelated to blood loss

● Elevated liver enzymes:

• Aspartate aminotransferase (AST) or alanine aminotransferase (ALT) ≥2 times the

upper level of normal (based on laboratory-specific reference ranges)

● Low platelets: <100,000 cells/microL

The use of twice the upper limit of normal threshold was chosen, in part, to avoid problems
related to differences in assays, which may result in an elevated absolute value in one
hospital that is considered near normal in another.

In HELLP, an elevated LDH level is a nonspecific marker that can be associated with severe
hemolysis, acute hepatocellular injury, or both. The total bilirubin level is increased as a
result of an increase in the indirect (unconjugated) fraction from hemolysis. Haptoglobin
level is a specific marker of hemolysis: 25 mg/dL provides the best cutoff between hemolytic
and non-hemolytic disorders. (See "Diagnosis of hemolytic anemia in adults", section on
'High LDH and bilirubin; low haptoglobin'.)

Severe anemia in pregnancy can be defined as hemoglobin level <8 to 10 g/dL, depending on
the trimester. (See "Anemia in pregnancy", section on 'Definition of anemia'.)

The American College of Obstetricians and Gynecologists suggests the following diagnostic
criteria and acknowledges the absence of clinical consensus among experts [22]:

● LDH ≥600 IU/L, and

● AST and ALT elevated more than twice the upper limit of normal, and
● Platelet count <100,000 cells/microL

Subclassification — Although not commonly used, some clinicians subclassify HELLP

based on severity of thrombocytopenia (Mississippi classification) [23]:

● Class 1 – Platelet count ≤50,000 cells/microL plus LDH >600 IU/L and AST or ALT ≥70 IU/L

● Class 2 – Platelet count >50,000 but ≤100,000 cells/microL plus LDH >600 IU/L and AST or
ALT ≥70 IU/L

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 6 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

● Class 3 – Platelet count >100,000 but ≤150,000 cells/microL plus LDH >600 IU/L and AST
or ALT ≥40 IU/L

DIFFERENTIAL DIAGNOSIS

HELLP syndrome may occasionally be confused with other diseases complicating pregnancy.
The four major disorders in differential diagnosis are acute fatty liver of pregnancy,
thrombotic thrombocytopenic purpura, pregnancy-related hemolytic-uremic syndrome, and
systemic lupus erythematosus ( table 3A-B). There is also overlap with preeclampsia with
severe features, which may not be a separate disease. In HELLP, angiopathy and liver
dysfunction are marked, and the magnitude of hypertension is not highly correlated with the
level of angiopathy and liver dysfunction. By contrast, most cases of severe preeclampsia
have severe hypertension; thrombocytopenia and liver dysfunction, although present, are
not as markedly abnormal as in HELLP. However, the clinical and histologic features are so
similar that establishing the correct diagnosis may not be possible; furthermore, HELLP can
occur concurrently with these disorders. (See "Hypertensive disorders in pregnancy:
Approach to differential diagnosis".)

MANAGEMENT IN PATIENTS PRESENTING BEFORE DELIVERY

The initial steps in management are to assess the mother as described above, stabilize
women who are unstable, and assess fetal status (nonstress test and ultrasound examination
for biophysical profile and fetal presentation).

Because of the potential for severe maternal complications, which can develop rapidly,
women with HELLP should be managed at a tertiary care center with appropriate levels of
maternal and neonatal intensive care.

Patients requiring urgent assessment or intervention

● Women with severe hypertension should receive antihypertensive therapy (eg,

intravenous labetalol ( table 4)) promptly to reduce the risk of stroke. The approach to
antihypertensive therapy is the same as that for preeclampsia. (See "Treatment of

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 7 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

hypertension in pregnant and postpartum women", section on 'Acute therapy of severe

hypertension'.)

● Women with an abnormal fetal heart rate tracing or low biophysical profile score
should be managed according to usual clinical standards. (See "Intrapartum category I,
II, and III fetal heart rate tracings: Management" and "Biophysical profile test for
antepartum fetal assessment".)

● Women with severe right upper quadrant/epigastric pain may have hepatic bleeding
or hepatic swelling portending liver rupture. The pain may be associated with
hypotension and tachycardia; shoulder, back, or neck pain; dyspnea or pain on
inspiration; nausea/vomiting; and/or abdominal distention beyond that expected for the
pregnant state [24,25]. In a series of 33 HELLP patients complaining of severe right
upper quadrant abdominal pain and either shoulder pain, neck pain, or relapsing
hypotension, imaging studies revealed an abnormal liver in 45 percent, most commonly
subcapsular hematoma and intraparenchymal hemorrhage ( image 1 and image 2)
[24]. In the overall population of patients with HELLP, however, the incidence of
subcapsular hematoma is much less, estimated to be 0.9 to 1.6 percent [19,26].

The aminotransferases in women with hepatic bleeding are usually modestly elevated,
but values of 4000 to 5000 IU/L can occasionally be seen. Because the correlation
between the magnitude of laboratory abnormalities and hepatic histology is poor [16],
women with severe symptoms should undergo an appropriate imaging study
expeditiously to look for hepatic bleeding, even if liver enzymes are not severalfold
above the normal range [21,24,25]. Bedside ultrasound screening (focused assessment
with sonography for trauma) is a good initial study, followed by formal ultrasound
examination and computed tomography (CT) or magnetic resonance imaging (MRI),
when needed for clinical decision making. Imaging using CT or MRI is more dependable
than ultrasonography for detecting hepatic hematoma and rupture ( image 3 and
image 4) but may not be as readily available and CT exposes the fetus to ionizing
radiation. (See "Diagnostic imaging in pregnant and nursing patients".)

The hematoma may remain contained, or rupture, with resulting hemorrhage into the
peritoneal cavity. Rupture is a life-threatening complication for both the mother and

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 8 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

fetus, especially if diagnosis and treatment are delayed. The management of a

hematoma is to support the patient with volume replacement and transfusion of blood
and blood products, as needed. Prompt delivery is indicated once she is
hemodynamically stable and severe anemia and coagulopathy, if present, have been
corrected. We stabilize the mother before delivery, even in cases with nonreassuring
fetal heart rate patterns or a low biophysical profile score.

A team experienced in liver trauma surgery should be consulted during maternal

stabilization and prior to delivery [27]. A stable contained hematoma may be managed
conservatively. Operative management of an expanding or ruptured hematoma includes
packing, drainage, hepatic artery ligation, and/or resection of affected areas of the liver.
For patients with intractable hemorrhage despite these interventions, administration of
recombinant factor VIIa [28] and liver transplantation [29-32] have been successful in
case reports.

Repeat ultrasound evaluation of the liver is performed 48 hours after delivery. If stable,
repeat testing is performed again in one week and at six weeks postpartum. In general,
most patients will be discharged by one week postpartum if the images are stable. If
laboratory abnormalities are resolving after delivery, the patient may be discharged
home with outpatient follow-up. It may take months for a hematoma to resolve
completely [21,24].

Surgical intervention in patients who develop a hematoma after delivery is indicated in

those with hemodynamic instability, persistent bleeding, increasing pain, or continued
expansion of the hematoma on serial ultrasound examinations [33]. Percutaneous
embolization of the hepatic arteries is a reasonable first-line therapy in women who are
hemodynamically stable [34,35].

There are no ongoing hepatic sequelae following recovery.

● Women with disseminated intravascular coagulation (DIC), pulmonary edema, or

acute kidney injury should be stabilized and delivered.

• (See "Disseminated intravascular coagulation (DIC) during pregnancy: Clinical

findings, etiology, and diagnosis".)

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 9 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

• (See "Acute respiratory failure during pregnancy and the peripartum period", section
on 'Pulmonary edema'.)
• (See "Acute kidney injury in pregnancy".)

Our approach — Our approach is illustrated in the algorithm ( algorithm 1).

Intravenous fluids are administered as in patients with preeclampsia. (See "Preeclampsia:

Management and prognosis", section on 'Fluids'.)

Candidates for prompt delivery — The cornerstone of therapy for HELLP occurring
during pregnancy is delivery, which is the only effective treatment. There is consensus
among experts that prompt delivery is indicated after maternal stabilization for any of the
following [18,36]:

● Pregnancies ≥34 weeks of gestation.

● Pregnancies that have not reached a stage of fetal maturity that ensures a reasonable
chance of extrauterine survival. (See "Periviable birth (limit of viability)".)

● Fetal demise.

● Abruptio placentae.

In the absence of any of these three scenarios or the urgent clinical scenarios described
above (hepatic bleeding, DIC, pulmonary edema, acute kidney injury, abnormal fetal heart
rate pattern), delivery may be delayed until a course of corticosteroids has been
administered and completed (eg, 48 hours after the first injection of betamethasone). (See
'Management of candidates for 48 hour delay before delivery' below.)

Magnesium sulfate is given intravenously to patients on the labor and delivery unit to
prevent convulsions and for fetal/neonatal neuroprotection in pregnancies between 24 and
32 weeks of gestation with a live fetus. (See "Preeclampsia: Management and prognosis",
section on 'Regimen' and "Neuroprotective effects of in utero exposure to magnesium
sulfate".)

We do not manage patients with HELLP syndrome expectantly at any gestational age and

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 10 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

consider conservative management for more than 48 hours investigational. There are few
studies on the outcome of expectant management of HELLP syndrome. In these studies, the
laboratory abnormalities of HELLP syndrome reversed in a subset of patients managed
expectantly, and serious maternal complications were uncommon with careful maternal
monitoring and timely intervention. However, the aim of expectant management is to
improve neonatal morbidity and mortality. There is no evidence demonstrating improvement
in overall perinatal outcome with expectant management compared with pregnancies
delivered after a course of corticosteroids and no maternal benefits from expectant
management. The following studies support our approach:

● In a study that treated 128 women with HELLP <34 weeks of gestation with volume
expansion and pharmacologic vasodilation under invasive hemodynamic monitoring,
delivery was necessitated in 22/128 (17 percent) of patients within 48 hours; the
remaining patients had a median prolongation of pregnancy of 15 days [37]. Although
there was no maternal mortality or serious maternal morbidity and more than one-half
(55/102) of the women had complete reversal of their laboratory abnormalities with
expectant management, 11 fetal and 7 neonatal deaths occurred.

● In another series, 41 women with HELLP <35 weeks of gestation were managed
expectantly [38]. Delivery was required within 48 hours in 14/41 (34 percent), the
remaining patients had a median prolongation of pregnancy of three days, and more
than one-half (15/27) had compete reversal of their laboratory abnormalities [38].
However, there were 10 fetal deaths.

Management of candidates for 48 hour delay before delivery

● Betamethasone administration to promote fetal pulmonary maturity – When both the

maternal and fetal status are reassuring and the gestational age is above the lower limit
of viability and <34 weeks of gestation, we administer a course of betamethasone before
delivering pregnancies complicated by HELLP syndrome [18,36]. Although a short delay
in delivery for betamethasone administration does not appear to increase maternal or
fetal morbidity or mortality [39], we advise not attempting delaying delivery beyond 48
hours because disease progression usually occurs, sometimes with rapid maternal
deterioration.

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 11 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

We do not give betamethasone for fetal lung maturity in pregnancies with gestational
age ≥34 weeks since no patients with HELLP were enrolled in randomized trials of the
efficacy of steroids after 34 weeks. During administration of betamethasone, all women
are kept in labor and delivery with continuous fetal monitoring. (See "Antenatal
corticosteroid therapy for reduction of neonatal respiratory morbidity and mortality from
preterm delivery", section on 'Long-term harms'.)

● Magnesium sulfate is initiated at the time of admission and continued through delivery
and the postpartum period to prevent maternal seizures and for fetal/neonatal
neuroprotection. (See "Neuroprotective effects of in utero exposure to magnesium
sulfate" and "Preeclampsia: Management and prognosis", section on 'Seizure
prophylaxis'.)

● Antihypertensive medication is administered to control severe hypertension, if present.

(See "Treatment of hypertension in pregnant and postpartum women".)

● The author repeats the complete blood count and platelet count at 24 and 48 hours after
administering steroids and more often if clinical deterioration is suspected. The
American College of Obstetricians and Gynecologists recommends laboratory testing at
least at 12 hour intervals until delivery and in the postpartum period [22].

This information is useful when considering whether to administer red blood cell
transfusions, whether neuraxial anesthesia can be performed safely (see "Adverse
effects of neuraxial analgesia and anesthesia for obstetrics", section on 'Neuraxial
analgesia and low platelets'), and whether platelet transfusion is indicated. (See
'Indications for platelet transfusion' below.)

Indications for red cell transfusion — We transfuse red blood cells if the hemoglobin is <7
g/dL and/or if the patient has ecchymosis, severe hematuria, or suspected abruption.

Indications for platelet transfusion — Actively bleeding patients with thrombocytopenia

should be transfused with platelets. Platelet transfusion may be indicated to prevent
excessive bleeding during delivery if the platelet count is less than 20,000 cells/microL, but
the threshold for prophylactic platelet transfusion in this setting is controversial. The decision
depends on patient-specific factors; consultation with the hematology service may be

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 12 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

helpful. It is also useful to notify the blood bank that platelet transfusions may be required.

If cesarean delivery is planned, platelet transfusion may be required. Some experts

recommend platelet transfusion to achieve a preoperative platelet count greater than 40,000
to 50,000 cells/microL [18], but the minimum count before a neuraxial procedure is
controversial and depends on factors in addition to platelet concentration. (See "Clinical and
laboratory aspects of platelet transfusion therapy", section on 'Preparation for an invasive
procedure' and "Adverse effects of neuraxial analgesia and anesthesia for obstetrics", section
on 'Neuraxial analgesia and low platelets'.)

Route of delivery — Vaginal delivery is desirable in the absence of standard indications for
cesarean delivery (eg, breech, nonreassuring fetal status). We induce women regardless of
gestational age when the cervix is favorable. When the cervix is unfavorable, we believe
cesarean delivery is probably preferable to induction in pregnancies less than 30 to 32 weeks
of gestation, especially if there are signs of fetal compromise (growth restriction,
oligohydramnios). Induction of these pregnancies, even with use of cervical ripening agents,
generally has a high failure rate and is often prolonged, thereby potentially exposing the
mother and fetus to a higher risk of complications from severe HELLP syndrome [18]. (See
"Induction of labor with oxytocin" and "Induction of labor: Techniques for preinduction
cervical ripening".)

Anesthesia/analgesia — Thrombocytopenia and coagulation abnormalities may preclude

use of neuraxial anesthesia for labor and delivery. The minimum platelet count necessary to
safely perform neuraxial anesthesia is unknown, and practice varies. Use of neuraxial and
general anesthesia for these patients is reviewed separately. (See "Anesthesia for the patient
with preeclampsia", section on 'Coagulation'.)

Opioids administered intravenously provide some pain relief without risk of maternal
bleeding, which may occur with intramuscular administration or with placement of neuraxial
anesthesia, removal of a neuraxial catheter, or placement of a pudendal nerve block.
However, there is no contraindication to perineal infiltration of an anesthetic for performing
an episiotomy or repairing the perineum. (See "Pharmacologic management of pain during
labor and delivery".)

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 13 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Cesarean delivery — In patients with severe laboratory abnormalities that are suggestive of
liver hematoma, we perform a midline skin incision. After delivery of the fetus, if
preoperative imaging was not performed, the liver may be palpated very gently to assess for
the presence of an unruptured hematoma.

Because of the increased risk of subfascial and wound hematoma in women with
thrombocytopenia who undergo cesarean delivery, the author places a subfascial drain and
leaves the skin incision open for the first 48 postoperative hours [4]. Some surgeons place a
subfascial and/or suprafascial drain and close the incision with staples, so it is easy to open
partially if a hematoma develops. The management of the abdominal wall incision after
delivery should be individualized, depending on the surgeon's assessment of risk of
hematoma/seroma development.

Is there a role for dexamethasone in treatment of HELLP? — We do not treat patients

with HELLP syndrome with dexamethasone. The two largest, randomized, double-blind,
placebo-controlled trials evaluating the use of dexamethasone to improve maternal outcome
in patients with HELLP syndrome did not establish a benefit [40,41], in contrast to initial
observational studies and small randomized trials that suggested more rapid improvement
in maternal laboratory and clinical parameters [42-45].

In a meta-analysis of 11 trials (n = 550 women) comparing corticosteroid treatment with

placebo/no treatment in women with HELLP syndrome, steroid administration did not lead to
a convincing reduction in maternal death (risk ratio [RR] 0.95, 95% CI 0.28-3.21), maternal
death or severe maternal morbidity (RR 0.27, 95% CI 0.03-2.12), or perinatal/infant death (RR
0.64, 95% CI 0.21-1.97), but the standardized mean difference in platelet count favored the
steroid group (0.67, 95% CI 0.26-1.10) [46].

The use of dexamethasone rather than betamethasone to promote fetal pulmonary maturity
is a separate issue. (See "Antenatal corticosteroid therapy for reduction of neonatal
respiratory morbidity and mortality from preterm delivery", section on 'Betamethasone or
dexamethasone?'.)

POSTPARTUM COURSE

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 14 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Laboratory values may initially worsen in the 48 hours following delivery (eg, platelet count
usually decreases by 40 percent/day, hematocrit falls, and liver enzymes increase) [47], which
is the reason that the American College of Obstetricians and Gynecologists recommends
laboratory testing at least at 12 hour intervals in the postpartum period [22]. We stop
checking laboratory values once they are clearly beginning to return to normal. Although
liver enzymes return to normal postpartum, in one report, total bilirubin levels were elevated
in 11 (20 percent) of the women who had liver function tests checked 3 to 101 months after
delivery [48].

An upward trend in platelet count and a downward trend in lactate dehydrogenase (LDH)
concentration are usually seen by the fourth postpartum day in the absence of
complications. In a series of 158 women with HELLP syndrome, platelet counts decreased
until 24 to 48 hours after delivery, while serum LDH concentration usually peaked at this time
[47]. In all patients who recovered, a platelet count greater than 100,000 cells/microL was
achieved with supportive care alone by the sixth postpartum day or within 72 hours of the
platelet nadir. Others have reported similar findings [49]. The platelet count may rebound;
one group reported values of 413,000 to 871,000 cells/microL [50].

If the platelet count continues to fall and LDH continues to rise after the fourth postpartum
day, then diagnoses other than HELLP syndrome (eg, primary thrombotic
microangiopathy) should be considered [22]. However, recovery can be delayed in women
with particularly severe HELLP, such as those with disseminated intravascular coagulation
(DIC), platelet count less than 20,000 cells/microL, renal dysfunction, or ascites [18,51]. These
women are at risk of developing pulmonary edema and acute kidney injury.

Magnesium sulfate is usually continued for 24 to 48 hours postpartum. (See "Preeclampsia:

Management and prognosis", section on 'Duration of therapy'.)

Women who are critically ill or at substantial risk for developing serious complications can
benefit from transfer to an intensive care setting, rather than a postpartum unit. Potential
indications for intensive monitoring include threatened or actual liver rupture or fulminant
liver failure, DIC, acute kidney injury, massive transfusion with concern about protecting the
airway, transfusion-related acute lung injury, and cardiac ischemia or cardiomyopathy.
Supportive care may involve oxygenation and ventilation (ie, supplemental oxygen or

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 15 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

mechanical ventilation), sedation, pain control, hemodynamic support (ie, vasopressors),

monitoring, volume management (ie, intravenous fluids or diuretics), nutritional support,
stress ulcer prophylaxis, and venous thromboembolism prophylaxis. (See "Critical illness
during pregnancy and the peripartum period".)

OUTCOME AND PROGNOSIS

Maternal outcome

Complications — The outcome for mothers with HELLP is generally good; however,
serious complications are relatively common. In the author's series of 437 women with
HELLP syndrome at a tertiary care facility, the following complications were observed [19]:

● Bleeding – 55 percent required transfusions with blood or blood products; 2 percent

required laparotomies for major intraabdominal bleeding
● Disseminated intravascular coagulation (DIC) – 21 percent
● Abruptio placentae – 16 percent
● Acute renal failure – 8 percent
● Pulmonary edema – 6 percent
● Subcapsular liver hematoma (or hepatic rupture) – 1 percent
● Retinal detachment – 1 percent
● Death – 1 percent

Many of these complications are interdependent (eg, abruptio placentae is a common

obstetric etiology of DIC, which, in turn, may induce acute kidney injury, which may lead to
pulmonary edema).

Additional complications that have been reported in other series include adult respiratory
distress syndrome, sepsis, stroke, cerebral hemorrhage and edema, and hepatic infarction
(in patients with antiphospholipid syndrome) [6,52,53]. Wound complications secondary to
bleeding and hematomas are common in women with thrombocytopenia.

The risk of serious morbidity correlates with increasing severity of maternal symptoms and
laboratory abnormalities [23,54]. In a report of four patients with aspartate aminotransferase

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 16 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

levels >2000 IU/L and lactate dehydrogenase levels >3000 IU/L, all had disordered mental
status, jaundice, intense hemolysis, and severe hypertension; one had multiorgan failure;
and two died [54].

HELLP syndrome with or without acute kidney injury does not affect long-term renal function
[55,56].

Recurrence in subsequent pregnancies — In a 2015 meta-analysis of individual patient

data from 512 women with HELLP who became pregnant again, 7 percent developed HELLP
in a subsequent pregnancy [57]. In addition, 18 percent developed preeclampsia and 18
percent gestational hypertension. In a subsequent Norwegian registry-based study of 577
women with HELLP in their first pregnancy and then a second pregnancy, 24 percent
developed hypertensive disorders of pregnancy that included either HELLP syndrome,
preeclampsia, pregnancy-induced hypertension, or eclampsia in the second pregnancy
compared with 3.6 percent of women with no HELLP in their first pregnancy [58]. The study
did not provide results for recurrent HELLP syndrome alone. The risk of recurrence of
hypertensive disorders of pregnancy was higher in women with preterm versus term HELLP
in the first pregnancy (30.3 versus 16.5 percent).

Prevention — There is no evidence that any therapy prevents recurrent HELLP syndrome,
but data are limited. The author considers HELLP syndrome a form of severe preeclampsia
and prescribes low-dose aspirin in future pregnancies to reduce the risk of preeclampsia. Use
of low-dose aspirin for prevention of preeclampsia is discussed separately. (See
"Preeclampsia: Prevention", section on 'Low-dose aspirin'.)

Fetal/neonatal outcome — Maternal laboratory parameters do not predict risk for fetal
demise. The neonatal and long-term prognoses are most strongly associated with
gestational age at delivery and birth weight [59-67].

The overall perinatal mortality rate is 7 to 20 percent; complications of preterm delivery,

intrauterine growth restriction, and abruptio placentae are the leading causes of perinatal
death [18,60].

Preterm delivery is common (70 percent; with 15 percent of births before 27 weeks) [59].
Leukopenia, neutropenia, and thrombocytopenia may be observed in the neonate but

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 17 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

appear to be related to intrauterine growth restriction, prematurity, and maternal

hypertension rather than HELLP [61]. Maternal HELLP does not affect fetal/neonatal liver
function.

MANAGEMENT OF PATIENTS PRESENTING AFTER DELIVERY

All of the signs and symptoms of HELLP, including subcapsular hematoma and liver rupture,
can initially appear in the postpartum period [68]. Management is similar to that of HELLP
diagnosed before delivery, except fetal status no longer needs to be considered.

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Hypertensive
disorders of pregnancy".)

INFORMATION FOR PATIENTS

UpToDate offers two types of patient education materials, "The Basics" and "Beyond the
Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th
grade reading level, and they answer the four or five key questions a patient might have
about a given condition. These articles are best for patients who want a general overview
and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are
longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th
grade reading level and are best for patients who want in-depth information and are
comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to
print or e-mail these topics to your patients. (You can also locate patient education articles on
a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

● Basics topic (see "Patient education: HELLP syndrome (The Basics)" and "Patient

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 18 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

education: High blood pressure and pregnancy (The Basics)")

SUMMARY AND RECOMMENDATIONS

● HELLP syndrome (hemolysis with a microangiopathic blood smear, elevated liver

enzymes, and low platelet count) develops in 0.1 to 1 percent of pregnancies. (See
'Prevalence' above.)

● The most common clinical presentation is abdominal pain and tenderness in the
midepigastrium, right upper quadrant, or below the sternum. Many patients also have
nausea, vomiting, and malaise, which may be mistaken for a viral illness. Hypertension
and proteinuria are present in approximately 85 percent of cases. Most cases of HELLP
are diagnosed between 28 and 36 weeks of gestation, but symptoms may present up to
7 days postpartum. (See 'Patient presentation' above.)

● The diagnosis of HELLP is based on the presence of all of the following criteria
(Tennessee classification) (see 'Diagnosis' above):

• Hemolysis, established by at least two of the following:

- Peripheral smear with schistocytes and burr cells ( picture 2)

- Serum bilirubin ≥1.2 mg/dL (20.52 micromol)
- Low serum haptoglobin or lactate dehydrogenase (LDH) ≥2 times the upper
level of normal (based on laboratory-specific reference ranges)
- Severe anemia, unrelated to blood loss

• Elevated liver enzymes:

- Aspartate aminotransferase (AST) or alanine aminotransferase (ALT) ≥2 times

the upper level of normal (based on laboratory-specific reference ranges)

• Low platelets: <100,000 cells/microL

● The four major disorders in differential diagnosis are acute fatty liver of pregnancy,
thrombotic thrombocytopenic purpura, pregnancy-related hemolytic-uremic syndrome,

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 19 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

and systemic lupus erythematosus. All have features that overlap with HELLP (
table 3A-B). (See 'Differential diagnosis' above.)

● The outcome for mothers with HELLP syndrome is generally good, but serious
complications such as abruptio placentae, acute kidney injury, subcapsular liver
hematoma or hepatic rupture, pulmonary edema, and retinal detachment may occur.
(See 'Maternal outcome' above.)

● The short-term and long-term prognoses for the infant are primarily related to
gestational age at delivery and birth weight: Preterm delivery and low birth weight are
common. Maternal HELLP does not affect fetal/neonatal liver function. (See
'Fetal/neonatal outcome' above.)

● Future pregnancies are at increased risk of developing HELLP, preeclampsia, and

gestational hypertension. (See 'Recurrence in subsequent pregnancies' above.)

Management recommendations — Our general approach is illustrated in the algorithm (

algorithm 1).

● The initial steps in management are to assess the mother, stabilize women who are
unstable, and assess gestational age and fetal status (nonstress test, ultrasound
examination for biophysical profile and fetal presentation). Because of the potential for
severe maternal complications, which can develop rapidly, women with HELLP should be
managed at a tertiary care center with appropriate levels of maternal and neonatal
intensive care. (See 'Management in patients presenting before delivery' above.)

● Women with severe hypertension should receive antihypertensive therapy (eg,

intravenous labetalol ( table 4)) promptly to reduce the risk of stroke. (See 'Patients
requiring urgent assessment or intervention' above.)

● Although uncommon, severe right upper quadrant/epigastric pain may be due to

hepatic bleeding, which may remain contained or rupture the liver capsule. The
management of a hematoma is to support the patient with volume replacement and
transfusion of blood and blood products, as needed. Prompt delivery is indicated once
she is hemodynamically stable and severe anemia and coagulopathy, if present, have

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 20 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

been corrected. A team experienced in liver trauma surgery should be consulted during
maternal stabilization and prior to delivery. (See 'Patients requiring urgent assessment
or intervention' above.)

● Women with disseminated intravascular coagulation, pulmonary edema, or renal failure

should be stabilized and delivered. (See 'Patients requiring urgent assessment or
intervention' above.)

● For pregnancies ≥34 weeks of gestation in which maternal and fetal status are
reassuring, we recommend delivery rather than expectant management (Grade 1C). In
this population, the potential risks of preterm birth are outweighed by the risk of
developing serious complication associated with HELLP syndrome. We also deliver
pregnancies below the limit of viability because expectant management is associated
with a high risk of developing maternal complications without significant improvement
in perinatal prognosis. (See 'Our approach' above.)

● For pregnancies above the limit of viability and <34 weeks of gestation in which maternal
and fetal status are reassuring, we suggest delivery after a course of betamethasone to
accelerate fetal pulmonary maturity rather than expectant management or prompt
delivery (Grade 2C). Although the laboratory abnormalities of HELLP syndrome will
reverse in a subgroup of patients managed expectantly and serious maternal
complications are uncommon with careful maternal monitoring, overall perinatal
outcome is not improved with expectant management. (See 'Management of candidates
for 48 hour delay before delivery' above.)

● For pregnancies less than 30 to 32 weeks with an unfavorable cervix, we suggest

cesarean delivery (Grade 2C). These patients are likely to have a prolonged induction if
vaginal delivery is attempted. (See 'Route of delivery' above.)

● We recommend not administering dexamethasone for treatment of HELLP syndrome

(Grade 1B). Dexamethasone does not accelerate resolution of laboratory abnormalities
or reduce the risk of maternal complications. (See 'Is there a role for dexamethasone in
treatment of HELLP?' above.)

● Magnesium sulfate is given intravenously to patients on the labor and delivery unit to

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 21 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

prevent convulsions and for fetal/neonatal neuroprotection in pregnancies between 24

and 32 weeks of gestation with a live fetus. (See "Preeclampsia: Management and
prognosis", section on 'Regimen' and "Neuroprotective effects of in utero exposure to
magnesium sulfate".)

Use of UpToDate is subject to the Subscription and License Agreement.

REFERENCES

1. Stone JH. HELLP syndrome: hemolysis, elevated liver enzymes, and low platelets. JAMA
1998; 280:559.

2. Sibai BM, Taslimi MM, el-Nazer A, et al. Maternal-perinatal outcome associated with the
syndrome of hemolysis, elevated liver enzymes, and low platelets in severe
preeclampsia-eclampsia. Am J Obstet Gynecol 1986; 155:501.

3. Reubinoff BE, Schenker JG. HELLP syndrome--a syndrome of hemolysis, elevated liver
enzymes and low platelet count--complicating preeclampsia-eclampsia. Int J Gynaecol
Obstet 1991; 36:95.
4. Sibai BM. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets):
much ado about nothing? Am J Obstet Gynecol 1990; 162:311.

5. Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis, elevated liver

enzymes, and low platelet count (HELLP): a review. Eur J Obstet Gynecol Reprod Biol
2013; 166:117.

6. Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical utility of strict diagnostic criteria
for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J
Obstet Gynecol 1996; 175:460.

7. Benedetto C, Marozio L, Tancredi A, et al. Biochemistry of HELLP syndrome. Adv Clin

Chem 2011; 53:85.

8. Jebbink J, Wolters A, Fernando F, et al. Molecular genetics of preeclampsia and HELLP

syndrome - a review. Biochim Biophys Acta 2012; 1822:1960.

9. Burwick RM, Feinberg BB. Eculizumab for the treatment of preeclampsia/HELLP

syndrome. Placenta 2013; 34:201.

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 22 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

10. Salmon JE, Heuser C, Triebwasser M, et al. Mutations in complement regulatory proteins
predispose to preeclampsia: a genetic analysis of the PROMISSE cohort. PLoS Med 2011;
8:e1001013.
11. Burwick RM, Burwick NR, Feinberg BB. Eculizumab fails to inhibit generation of C5a in
vivo. Blood 2014; 124:3502.

12. Yang Z, Yamada J, Zhao Y, et al. Prospective screening for pediatric mitochondrial
trifunctional protein defects in pregnancies complicated by liver disease. JAMA 2002;
288:2163.

13. Yang Z, Zhao Y, Bennett MJ, et al. Fetal genotypes and pregnancy outcomes in 35
families with mitochondrial trifunctional protein mutations. Am J Obstet Gynecol 2002;
187:715.

14. Wilcken B, Leung KC, Hammond J, et al. Pregnancy and fetal long-chain 3-hydroxyacyl
coenzyme A dehydrogenase deficiency. Lancet 1993; 341:407.

15. Ibdah JA, Bennett MJ, Rinaldo P, et al. A fetal fatty-acid oxidation disorder as a cause of
liver disease in pregnant women. N Engl J Med 1999; 340:1723.

16. Barton JR, Riely CA, Adamec TA, et al. Hepatic histopathologic condition does not
correlate with laboratory abnormalities in HELLP syndrome (hemolysis, elevated liver
enzymes, and low platelet count). Am J Obstet Gynecol 1992; 167:1538.

17. Ye W, Shu H, Yu Y, et al. Acute kidney injury in patients with HELLP syndrome. Int Urol
Nephrol 2019; 51:1199.

18. Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis,
elevated liver enzymes, and low platelet count. Obstet Gynecol 2004; 103:981.

19. Sibai BM, Ramadan MK, Usta I, et al. Maternal morbidity and mortality in 442
pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP
syndrome). Am J Obstet Gynecol 1993; 169:1000.

20. O'Brien JM, Barton JR. Controversies with the diagnosis and management of HELLP
syndrome. Clin Obstet Gynecol 2005; 48:460.

21. Ditisheim A, Sibai BM. Diagnosis and Management of HELLP Syndrome Complicated by
Liver Hematoma. Clin Obstet Gynecol 2017; 60:190.

22. Gestational Hypertension and Preeclampsia: ACOG Practice Bulletin, Number 222.

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 23 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Obstet Gynecol 2020; 135:e237.

23. Martin JN Jr, Rose CH, Briery CM. Understanding and managing HELLP syndrome: the
integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol
2006; 195:914.

24. Barton JR, Sibai BM. Hepatic imaging in HELLP syndrome (hemolysis, elevated liver
enzymes, and low platelet count). Am J Obstet Gynecol 1996; 174:1820.

25. Nunes JO, Turner MA, Fulcher AS. Abdominal imaging features of HELLP syndrome: a 10-
year retrospective review. AJR Am J Roentgenol 2005; 185:1205.

26. Haddad B, Barton JR, Livingston JC, et al. Risk factors for adverse maternal outcomes
among women with HELLP (hemolysis, elevated liver enzymes, and low platelet count)
syndrome. Am J Obstet Gynecol 2000; 183:444.

27. Stevenson JT, Graham DJ. Hepatic hemorrhage and the HELLP syndrome: a surgeon's
perspective. Am Surg 1995; 61:756.

28. Merchant SH, Mathew P, Vanderjagt TJ, et al. Recombinant factor VIIa in management of
spontaneous subcapsular liver hematoma associated with pregnancy. Obstet Gynecol
2004; 103:1055.

29. Erhard J, Lange R, Niebel W, et al. Acute liver necrosis in the HELLP syndrome: successful
outcome after orthotopic liver transplantation. A case report. Transpl Int 1993; 6:179.

30. Hunter SK, Martin M, Benda JA, Zlatnik FJ. Liver transplant after massive spontaneous
hepatic rupture in pregnancy complicated by preeclampsia. Obstet Gynecol 1995;
85:819.
31. Araujo AC, Leao MD, Nobrega MH, et al. Characteristics and treatment of hepatic rupture
caused by HELLP syndrome. Am J Obstet Gynecol 2006; 195:129.

32. Zarrinpar A, Farmer DG, Ghobrial RM, et al. Liver transplantation for HELLP syndrome.
Am Surg 2007; 73:1013.

33. Wilson RH, Marshall BM. Postpartum rupture of a subcapsular hematoma of the liver.
Acta Obstet Gynecol Scand 1992; 71:394.

34. Rinehart BK, Terrone DA, Magann EF, et al. Preeclampsia-associated hepatic hemorrhage
and rupture: mode of management related to maternal and perinatal outcome. Obstet
Gynecol Surv 1999; 54:196.

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 24 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

35. Grand'Maison S, Sauvé N, Weber F, et al. Hepatic rupture in hemolysis, elevated liver
enzymes, low platelets syndrome. Obstet Gynecol 2012; 119:617.
36. American College of Obstetricians and Gynecologists, Task Force on Hypertension in
Pregnancy. Hypertension in pregnancy. Report of the American College of Obstetricians
and Gynecologists’ Task Force on Hypertension in Pregnancy. Obstet Gynecol 2013;
122:1122.
37. Visser W, Wallenburg HC. Temporising management of severe pre-eclampsia with and
without the HELLP syndrome. Br J Obstet Gynaecol 1995; 102:111.
38. van Pampus MG, Wolf H, Westenberg SM, et al. Maternal and perinatal outcome after
expectant management of the HELLP syndrome compared with pre-eclampsia without
HELLP syndrome. Eur J Obstet Gynecol Reprod Biol 1998; 76:31.

39. Fitzpatrick KE, Hinshaw K, Kurinczuk JJ, Knight M. Risk factors, management, and
outcomes of hemolysis, elevated liver enzymes, and low platelets syndrome and
elevated liver enzymes, low platelets syndrome. Obstet Gynecol 2014; 123:618.

40. Fonseca JE, Méndez F, Cataño C, Arias F. Dexamethasone treatment does not improve
the outcome of women with HELLP syndrome: a double-blind, placebo-controlled,
randomized clinical trial. Am J Obstet Gynecol 2005; 193:1591.
41. Katz L, de Amorim MM, Figueiroa JN, Pinto e Silva JL. Postpartum dexamethasone for
women with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome: a
double-blind, placebo-controlled, randomized clinical trial. Am J Obstet Gynecol 2008;
198:283.e1.
42. O'Brien JM, Shumate SA, Satchwell SL, et al. Maternal benefit of corticosteroid therapy in
patients with HELLP (hemolysis, elevated liver enzymes, and low platelet count)
syndrome: impact on the rate of regional anesthesia. Am J Obstet Gynecol 2002;
186:475.
43. Matchaba P, Moodley J. Corticosteroids for HELLP syndrome in pregnancy. Cochrane
Database Syst Rev 2004; :CD002076.
44. Isler CM, Barrilleaux PS, Magann EF, et al. A prospective, randomized trial comparing the
efficacy of dexamethasone and betamethasone for the treatment of antepartum HELLP
(hemolysis, elevated liver enzymes, and low platelet count) syndrome. Am J Obstet

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 25 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Gynecol 2001; 184:1332.

45. Martin JN Jr, Thigpen BD, Rose CH, et al. Maternal benefit of high-dose intravenous
corticosteroid therapy for HELLP syndrome. Am J Obstet Gynecol 2003; 189:830.
46. Woudstra DM, Chandra S, Hofmeyr GJ, Dowswell T. Corticosteroids for HELLP (hemolysis,
elevated liver enzymes, low platelets) syndrome in pregnancy. Cochrane Database Syst
Rev 2010; :CD008148.

47. Martin JN Jr, Blake PG, Perry KG Jr, et al. The natural history of HELLP syndrome: patterns
of disease progression and regression. Am J Obstet Gynecol 1991; 164:1500.
48. Knapen MF, van Altena AM, Peters WH, et al. Liver function following pregnancy
complicated by the HELLP syndrome. Br J Obstet Gynaecol 1998; 105:1208.
49. Hupuczi P, Nagy B, Sziller I, et al. Characteristic laboratory changes in pregnancies
complicated by HELLP syndrome. Hypertens Pregnancy 2007; 26:389.

50. Neiger R, Contag SA, Coustan DR. The resolution of preeclampsia-related

thrombocytopenia. Obstet Gynecol 1991; 77:692.
51. Martin JN Jr, Blake PG, Lowry SL, et al. Pregnancy complicated by preeclampsia-
eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet
count: how rapid is postpartum recovery? Obstet Gynecol 1990; 76:737.
52. Martin JN Jr, Rinehart BK, May WL, et al. The spectrum of severe preeclampsia:
comparative analysis by HELLP (hemolysis, elevated liver enzyme levels, and low platelet
count) syndrome classification. Am J Obstet Gynecol 1999; 180:1373.

53. Pauzner R, Dulitzky M, Carp H, et al. Hepatic infarctions during pregnancy are associated
with the antiphospholipid syndrome and in addition with complete or incomplete HELLP
syndrome. J Thromb Haemost 2003; 1:1758.

54. Catanzarite VA, Steinberg SM, Mosley CA, et al. Severe preeclampsia with fulminant and
extreme elevation of aspartate aminotransferase and lactate dehydrogenase levels: high
risk for maternal death. Am J Perinatol 1995; 12:310.
55. Jacquemyn Y, Jochems L, Duiker E, et al. Long-term renal function after HELLP syndrome.
Gynecol Obstet Invest 2004; 57:117.
56. Drakeley AJ, Le Roux PA, Anthony J, Penny J. Acute renal failure complicating severe
preeclampsia requiring admission to an obstetric intensive care unit. Am J Obstet

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 26 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Gynecol 2002; 186:253.

57. van Oostwaard MF, Langenveld J, Schuit E, et al. Recurrence of hypertensive disorders of
pregnancy: an individual patient data metaanalysis. Am J Obstet Gynecol 2015;
212:624.e1.

58. Malmström O, Håberg SE, Morken NH. Probability and outcomes of second pregnancy
after HELLP syndrome in the first: A population-based registry study. Acta Obstet
Gynecol Scand 2020; 99:1206.
59. Abramovici D, Friedman SA, Mercer BM, et al. Neonatal outcome in severe preeclampsia
at 24 to 36 weeks' gestation: does the HELLP (hemolysis, elevated liver enzymes, and low
platelet count) syndrome matter? Am J Obstet Gynecol 1999; 180:221.
60. Sibai BM, Spinnato JA, Watson DL, et al. Pregnancy outcome in 303 cases with severe
preeclampsia. Obstet Gynecol 1984; 64:319.

61. Harms K, Rath W, Herting E, Kuhn W. Maternal hemolysis, elevated liver enzymes, low
platelet count, and neonatal outcome. Am J Perinatol 1995; 12:1.
62. Singhal N, Amin HJ, Pollard JK, et al. Maternal haemolysis, elevated liver enzymes and
low platelets syndrome: perinatal and neurodevelopmental neonatal outcomes for
infants weighing less than 1250 g. J Paediatr Child Health 2004; 40:121.
63. Dötsch J, Hohmann M, Kühl PG. Neonatal morbidity and mortality associated with
maternal haemolysis elevated liver enzymes and low platelets syndrome. Eur J Pediatr
1997; 156:389.
64. Gortner L, Pohlandt F, Bartmann P, et al. Short-term outcome in infants with birth
weights less than 1750 g born to mothers with HELLP syndrome. J Perinat Med 1992;
20:25.

65. Kändler C, Kevekordes B, Zenker M, et al. Prognosis of children born to mothers with
HELLP-syndrome. J Perinat Med 1998; 26:486.
66. Murray D, O'Riordan M, Geary M, et al. The HELLP syndrome: maternal and perinatal
outcome. Ir Med J 2001; 94:16.
67. Guzel AI, Kuyumcuoglu U, Celik Y. Are maternal and fetal parameters related to perinatal
mortality in HELLP syndrome? Arch Gynecol Obstet 2011; 283:1227.

68. Gilboa Y, Bardin R, Feldberg D, Bachar GN. Postpartum hepatic rupture and

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 27 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

retroperitoneal hematoma associated with HELLP syndrome. Isr Med Assoc J 2006;
8:219.
Topic 6778 Version 59.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 28 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

GRAPHICS

Criteria for the diagnosis of preeclampsia

Systolic blood pressure ≥140 mmHg or diastolic blood pressure ≥90 mmHg on at least 2 occasions at least 4 hours
apart after 20 weeks of gestation in a previously normotensive patient AND the new onset of 1 or more of the
following*:

Proteinuria ≥0.3 g in a 24-hour urine specimen or protein/creatinine ratio ≥0.3 (mg/mg) (30 mg/mmol) in a random
urine specimen or dipstick ≥2+ if a quantitative measurement is unavailable

Platelet count <100,000/microL

Serum creatinine >1.1 mg/dL (97.2 micromol/L) or doubling of the creatinine concentration in the absence of other
renal disease

Liver transaminases at least twice the upper limit of the normal concentrations for the local laboratory

Pulmonary edema

New-onset and persistent headache not accounted for by alternative diagnoses and not responding to usual doses of
analgesics ¶

Visual symptoms (eg, blurred vision, flashing lights or sparks, scotomata)

Preeclampsia is considered superimposed when it occurs in a woman with chronic hypertension. It is characterized by
worsening or resistant hypertension (especially acutely), the new onset of proteinuria or a sudden increase in
proteinuria, and/or significant new end-organ dysfunction after 20 weeks of gestation in a woman with chronic
hypertension.

* If systolic blood pressure is ≥160 mmHg or diastolic blood pressure is ≥110 mmHg, confirmation within minutes is sufficient.
​ ¶ Response to analgesia does not exclude the possibility of preeclampsia.

Adapted from: American College of Obstetricians and Gynecologists (ACOG) Practice Bulletin No. 222: Gestational
Hypertension and Preeclampsia. Obstet Gynecol 2020; 135:e237.

Graphic 79977 Version 36.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 29 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

In a patient with preeclampsia, the presence of one or more of the following indicates a
diagnosis of "preeclampsia with severe features"

Severe blood pressure elevation:

Systolic blood pressure ≥160 mmHg or diastolic blood pressure ≥110 mmHg on 2 occasions at least 4 hours apart while
the patient is on bedrest; however, antihypertensive therapy generally should be initiated upon confirmation of severe
hypertension, in which case criteria for severe blood pressure elevation can be satisfied without waiting until 4 hours
have elapsed.

Symptoms of central nervous system dysfunction:

New-onset cerebral or visual disturbance, such as:
Photopsia, scotomata, cortical blindness, retinal vasospasm
Severe headache (ie, incapacitating, "the worst headache I've ever had") or headache that persists and progresses
despite analgesic therapy and not accounted for by alternative diagnoses

Hepatic abnormality:
Impaired liver function not accounted for by another diagnosis and characterized by serum transaminase concentration
>2 times the upper limit of the normal range or severe persistent right upper quadrant or epigastric pain unresponsive
to medication and not accounted for by an alternative diagnosis

Thrombocytopenia:
<100,000 platelets/microL

Renal abnormality:
Renal insufficiency (serum creatinine >1.1 mg/dL [97.2 micromol/L] or a doubling of the serum creatinine concentration
in the absence of other renal disease)

Pulmonary edema

Reference:
1. American College of Obstetricians and Gynecologists (ACOG) Practice Bulletin No. 222: Gestational Hypertension and
Preeclampsia. Obstet Gynecol 2020; 135:e237.

Graphic 76975 Version 26.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 30 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

HELLP syndrome

Liver biopsy from a patient with HELLP syndrome. The zones immediately adjacent
to the portal triads show collections of red blood cells, without inflammation or
necrosis of hepatocytes.

HELLP: hemolysis, elevated liver enzymes, and low platelets.

Courtesy of Caroline A Riely, MD.

Graphic 69691 Version 2.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 31 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Reported frequency of signs and symptoms of HELLP syndrome

Sign/symptom Frequency, percent

Proteinuria 86 to 100

Hypertension 82 to 88

Right upper quadrant/epigastric pain 40 to 90

Nausea, vomiting 29 to 84

Headache 33 to 61

Visual changes 10 to 20

Jaundice 5

HELLP: hemolysis, elevated liver enzymes, and low platelets.

Graphic 64665 Version 3.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 32 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Peripheral smear in microangiopathic hemolytic anemia showing

presence of schistocytes

Peripheral blood smear from a patient with a microangiopathic hemolytic anemia

with marked red cell fragmentation. The smear shows multiple helmet cells
(arrows) and other fragmented red cells (small arrowhead); microspherocytes are
also seen (large arrowheads). The platelet number is reduced; the large platelet in
the center (dashed arrow) suggests that the thrombocytopenia is due to enhanced
destruction.

Courtesy of Carola von Kapff, SH (ASCP).

Graphic 70851 Version 8.0

Normal peripheral blood smear

High-power view of a normal peripheral blood smear. Several platelets

(arrowheads) and a normal lymphocyte (arrow) can also be seen. The red cells
are of relatively uniform size and shape. The diameter of the normal red cell
should approximate that of the nucleus of the small lymphocyte; central pallor

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 33 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

(dashed arrow) should equal one-third of its diameter.

Courtesy of Carola von Kapff, SH (ASCP).

Graphic 59683 Version 5.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 34 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Frequency of various signs and symptoms among imitators of preeclampsia

Signs and HELLP syndrome, AFLP, TTP, HUS, Exacerbation of SLE,

symptoms percent percent percent percent percent

Hypertension 85 50 20 to 75 80 to 90 80 with APA, nephritis

Proteinuria 90 to 95 30 to 50 With 80 to 90 100 with nephritis

hematuria

Fever Absent 25 to 32 20 to 50 NR Common during flare

Jaundice 5 to 10 40 to 90 Rare Rare Absent

Nausea and 40 50 to 80 Common Common Only with APA

vomiting

Abdominal pain 60 to 80 35 to 50 Common Common Only with APA

Central nervous 40 to 60 30 to 40 60 to 70 NR 50 with APA

system

HELLP: hemolysis, elevated liver enzymes, low platelets; AFLP: acute fatty liver of pregnancy; TTP: thrombotic thrombocytopenic
purpura; HUS: hemolytic uremic syndrome; SLE: systemic lupus erythematosus; APA: antiphospholipid antibodies with or without
catastrophic antiphospholipid syndrome; NR: values not reported; common: reported as the most common presentation.

Reproduced with permission from: Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol 2007; 109:956. Copyright © 2007
Lippincott Williams & Wilkins.

Graphic 64296 Version 11.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 35 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Frequency and severity of laboratory findings among imitators of preeclampsia

HELLP
Laboratory findings AFLP TTP HUS Exacerbation of SLE
syndrome

Thrombocytopenia (less More than More than 20,000 or More than More than 50,000
than 100,000/mm 3) 20,000 50,000 less 20,000

Hemolysis (%) 50 to 100 15 to 20 100 100 14 to 23 in patients with APA

Anemia (%) Less than 50 Absent 100 100 14 to 23 in patients with APA

DIC (%) Less than 20 50 to 100 Rare Rare Rare

Hypoglycemia (%) Absent 50 to 100 Absent Absent Absent

VW factor multimers (%) Absent Absent 80 to 90 80 Less than 10

ADAMTS13 less than 5% (%) Absent Absent 33 to 100 Rare Rare

Impaired renal function (%) 50 90 to 100 30 100 40 to 80

LDH (international units/L) 600 or more Variable More More than May be elevated in patients with APA
than 1000 and liver involvement
1000

Elevated ammonia (%) Rare 50 Absent Absent Absent

Elevated bilirubin (%) 50 to 60 100 100 NA Less than 10

Elevated transaminases (%) 100 100 Usually Usually In patients with APA and liver
mild* mild* involvement

HELLP: hemolysis, elevated liver enzymes, low platelets; AFLP: acute fatty liver of pregnancy; TTP: thrombotic thrombocytopenic
purpura; HUS: hemolytic uremic syndrome; SLE: systemic lupus erythematosus; APA: antiphospholipid antibodies with or without
catastrophic antiphospholipid syndrome; DIC: disseminated intravascular coagulopathy; VW: von Willebrand; ADAMTS13: von
Willebrand factor-cleaving metalloprotease; LDH: lactic dehydrogenase; NR: values not reported.
* Levels less than 100 international units/L.

Reproduced with permission from: Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol 2007; 109:956. Copyright © 2007
Lippincott Williams & Wilkins.

Graphic 65674 Version 13.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 36 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Antihypertensive agents used for urgent blood pressure control in pregnancy

Drug Initial dose Follow-up

Labetalol 20 mg IV gradually over 2 minutes. Repeat BP measurement at 10-minute

intervals:
If BP remains above target level at 10
minutes, give 40 mg IV over 2 minutes.
If BP remains above target level at 20
minutes, give 80 mg IV over 2 minutes.
If BP remains above target level at 30
minutes, give 80 mg IV over 2 minutes.
If BP remains above target level at 40
minutes, give 80 mg IV over 2 minutes.
Cumulative maximum dose is 300 mg. If target
BP is not achieved, switch to another class of
agent.

A continuous IV infusion of 1 to 2 mg/minute Adjust dose within this range to achieve target
can be used instead of intermittent therapy or blood pressure.
started after 20 mg IV dose. Cumulative maximum dose is 300 mg. If target
Requires use of programmable infusion pump BP is not achieved, switch to another class of
and continuous noninvasive monitoring of agent.
blood pressure and heart rate.

Hydralazine 5 mg IV gradually over 1 to 2 minutes.* Repeat BP measurement at 20-minute

Adequate reduction of blood pressure is less intervals:
predictable than with IV labetalol. If BP remains above target level at 20
minutes, give 5 or 10 mg IV over 2
minutes, depending on the initial
response.
If BP remains above target level at 40
minutes, give 10 mg IV over 2 minutes,
depending on the previous response.
Cumulative maximum dose is 30 mg. If target
BP is not achieved, switch to another class of
agent.

Nifedipine extended 30 mg orally. If target BP is not achieved in 1 to 2 hours,

release another dose can be administered.
If target BP is not achieved, switch to another
class of agent.

Nicardipine The initial dose is 5 mg/hour IV by infusion Adjust dose within this range to achieve target
(parenteral) pump and can be increased to a maximum of BP.
15 mg/hour.
Onset of action is delayed by 5 to 15 minutes; in
general, rapid titration is avoided to minimize
risk of overshooting dose.
Requires use of a programmable infusion
pump and continuous noninvasive monitoring
of blood pressure and heart rate.

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 37 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Nifedipine immediate 10 mg orally. Repeat BP measurement at 20-minute

release* May be associated with precipitous drops in BP intervals:
in some women, with associated FHR If BP remains above target at 20 minutes,
decelerations for which emergency cesarean give 10 or 20 mg orally, depending on the
delivery may be indicated. As such, this initial response.
regimen is not typically used as a first-line If BP remains above target at 40 minutes,
option and is usually reserved only for women give 10 or 20 mg orally, depending on the
without IV access. If used, FHR should be previous response.
monitored while administering short-acting Cumulative maximum dose is 180 mg.
nifedipine.
If target BP is not achieved, switch to another
class of agent.

Labetalol and hydralazine are the preferred drugs.

IV: intravenous; BP: blood pressure; FHR: fetal heart rate.

* We caution against use of immediate-release oral nifedipine, although some obstetric guidelines have endorsed its use as a
first-line option for emergency treatment of acute, severe hypertension in pregnancy or postpartum (other options were labetalol
and hydralazine), particularly when IV access is not in place. In most cases, use of immediate-release oral nifedipine will be safe
and well tolerated; however, there is a risk of an acute, precipitous fall in blood pressure, which may result in a reduction in
uteroplacental perfusion. The immediate-release preparations are also associated with a higher incidence of headache and
tachycardia. In nonpregnant adults, the package insert states that "nifedipine capsules should not be used for the acute reduction
of blood pressure."

Adapted from: ​
1. American College of Obstetricians and Gynecologists. Gestational hyertension and preeclampsia. Practice Bulletin, Number
222. Obstet Gynecol 2020; 135:e237.
2. Bernstein PS, Martin JN Jr, Barton JR, et al. National Partnership for Maternal Safety: Consensus Bundle on Severe Hypertension
During Pregnancy and the Postpartum Period. Obstet Gynecol 2017; 130:347.

Graphic 110261 Version 9.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 38 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Liver infarction subcapsular hematoma HELLP syndrome on CT scan

(A) An axial CT scan through the upper abdomen shows a large subcapsular hematoma compressing the liver
(arrow).
(B) This image shows a large and irregular perfusion defect involving the right lobe and part of the left lobe of
the liver (arrows).

CT: computed tomography; HELLP: hemolysis, elevated liver enzymes, and low platelets.

Courtesy of Jonathan B Kruskal, MD, PhD.

Graphic 89900 Version 2.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 39 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Liver infarction HELLP syndrome on CT scan

An axial CT scan through the upper abdomen shows multiple perfusion defects (arrowheads) in the
posterior aspect of the right lobe of the liver. A subcapsular hematoma is present (arrow). The
spleen is enlarged (dashed arrow).

CT: computed tomography; HELLP: hemolysis, elevated liver enzymes, and low platelets.

Courtesy of Jonathan B Kruskal, MD, PhD.

Graphic 89901 Version 2.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 40 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Subcapsular liver hematoma on MR

These MRIs are from a patient with HELLP syndrome and a subcapsular liver hematoma.
(A) The T2-weighted image shows a 9 mm hyperintense focus (arrow) that could represent a
small laceration or a hepatic cyst.
(B) The T1-weighted image shows an adjacent subcapsular hematoma (arrowhead) with both
hyperintense and hypointense components reflecting a complex solid thrombus. The
subcapsular hematoma was estimated as occupying less than 10 percent of the liver surface.

MRI: magnetic resonance imaging; HELLP: hemolysis, elevated liver enzymes, and low platelets.

Graphic 86843 Version 3.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 41 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Hepatic hematoma with rupture

Magnetic resonance image from a pregnant woman with hepatic hematoma with
rupture. This cut shows collected blood under the hepatic capsule running from the
dome of the liver down along the right side, pushing the remaining normal
parenchyma toward the midline.

Reproduced with permission from Barton JR, Sibai BM, Am J Obstet Gynecol 1996; 174:1820.

Graphic 68521 Version 3.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 42 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Management of patients with HELLP syndrome

HELLP: hemolysis, elevated liver enzymes, and low platelets; LDH: lactate dehydrogenase;
AST: aspartate transaminase; ALT: alanine transaminase.
* Criteria for HELLP: ​
Hemolysis, established by at least 2 of the following: ​
Peripheral smear with schistocytes and burr cells
Serum bilirubin ≥1.2 mg/dL (20.52 micromol)
Low serum haptoglobin (≤25 mg/dL) or LDH ≥2 times the upper level of normal
(in the local laboratory)
Severe anemia, unrelated to blood loss
Elevated liver enzymes: ​
AST or ALT ≥2 times the upper level of normal (in the local laboratory)
Low platelets: <100,000 cells/microL
¶ Patients with severe epigastric or right upper quadrant pain should undergo an
appropriate imaging study expeditiously to evaluate for hepatic bleeding, even if liver
enzymes are not severalfold above the normal range.
Δ The mother and fetus should be closely monitored during this period. Refer to UpToDate
content on HELLP syndrome.

Graphic 121602 Version 1.0

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 43 de 44
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) - UpToDate 09/07/21 12:14

Contributor Disclosures
Baha M Sibai, MD Nothing to disclose Charles J Lockwood, MD, MHCM Nothing to disclose Keith D
Lindor, MD Grant/Research/Clinical Trial Support: National Institute of Health [Primary sclerosing
cholangitis]. Vanessa A Barss, MD, FACOG Nothing to disclose

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.

Conflict of interest policy

https://www.uptodate.com/contents/hellp-syndrome-hemolysis-elevated-liver-enzymes-and-low-platelets/print Página 44 de 44