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A Review of HELLP Syndrome

William M. Curtin, MD genesis, characteristics of affected patients, laboratory criteria for
Louis Weinstein, MD diagnosis, sources of maternal and perinatal morbidity, and mortality
and management of complications. The possible role for conservative
HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome
management and antenatal treatment with the intention of prolong-
is a variant of severe preeclampsia which is associated with substantial
ing pregnancy will be explored.
maternal and perinatal morbidity and mortality. As with preeclampsia,
the etiology and pathogenesis of HELLP syndrome is not completely Pathogenesis
understood. An increase in vascular thrombosis and activation of the The etiology of preeclampsia remains unclear with the pathogenesis
coagulation system may be important in the clinical presentation of this being incompletely understood. Theories on the causation center on
disorder. Laboratory criteria for the diagnosis of HELLP syndrome have abnormal placentation resulting in placental ischemia and the pro-
been classically described but lack uniformity among different duction of a circulating toxic factor causing endothelial injury.6 The
institutions. Aggressive management of HELLP syndrome with disturbance in endothelial cells is believed to cause vascular constric-
expeditious delivery appears to yield the lowest perinatal mortality rates. tion, increased capillary leakage, and platelet aggregation resulting in
Conservative or expectant management has been associated with higher the clinical picture of hypertension, proteinuria, edema, and throm-
stillbirth rates with antenatal corticosteroids not causing resolution of bocytopenia.
the laboratory abnormalities. Resolution of laboratory abnormalities in It is unclear why certain patients with severe preeclampsia de-
HELLP syndrome runs a protracted course over several days after delivery. velop HELLP syndrome. It is possible that these patients have more
Despite nearly two decades since HELLP syndrome has been defined as a endothelial injury with greater activation of the coagulation system.
clinical entity, treatment for the disorder still remains delivery of the Paternoster et al.7 showed evidence of a compensated disseminated
patient. intravascular coagulation (DIC) in patients with HELLP syndrome
when compared with normotensive control patients with preeclampsia
Although the laboratory abnormalities in HELLP syndrome were without HELLP. They found statistically significant increases in
described decades ago,1 it was in 1982 that Weinstein devised the plasma fibronectin and D-dimer levels and decreases in antithrom-
acronym HELLP (H 5 hemolysis, EL 5 elevated liver enzymes, bin-III levels when the patients with HELLP syndrome were compared
and LP 5 low platelets) to define a subset of severe preeclampsia to the patients with preeclampsia. The HELLP group and the pre-
seen in 29 patients.2 The need to recognize HELLP syndrome as a eclamptic group also showed significant decreases in protein C and S
unique form of severe preeclampsia was emphasized, because activity as compared to the control normotensive group, but no signif-
often patients were given a nonobstetric diagnosis, such as chole- icant differences in comparison to each other. Routine tests to detect
cystitis or hepatitis, and treatment was delayed. Aggressive treat- DIC (fibrinogen, prothrombin time, partial thromboplastin time),
ment with maternal stabilization and expeditious delivery was were not significantly different among the three groups with the ex-
advocated to prevent maternal and perinatal mortality. ception of the partial thromboplastin time between the control and
HELLP syndrome is reported to occur in 20% of women with preeclamptic group. The findings of these investigators suggest more
severe preeclampsia and in 10% of women with eclampsia.3 Ma- endothelial damage, consumption of coagulation inhibitors, and
gann and associates4 reported the incidence of HELLP syndrome to activation of fibrinolysis in patients with HELLP syndrome than in
be 0.11% among all live born deliveries over a 12-year period at patients with preeclampsia without HELLP and normotensive
the University of Mississippi Medical Center. Maternal mortality controls.7
ranges from 0% to 4%.2,3,5 Perinatal mortality appears to be pri- Further support for the role of an enhanced thrombotic tendency
marily related to the gestational age at delivery. in the pathogenesis of HELLP syndrome was provided by Dekker et al.8
The focus of this review on HELLP syndrome will be on patho- in a study of 101 patients with severe early-onset preeclampsia, 53 of
who had HELLP syndrome. They demonstrated by testing, at least 10
Department of Obstetrics and Gynecology (W. M. C., L. W.), Division of Maternal Fetal Medi- weeks postpartum, that greater than 50% of patients had an abnor-
cine (L. W.), Medical College of Ohio, Toledo, OH.
mality associated with an increased tendency toward thrombosis. The
Address correspondence and reprint requests to William M. Curtin, MD, Department of Ob-
stetrics & Gynecology, Medical College of Ohio, Ruppert Center, Suite 1500, 3120 Glendale
most common abnormalities identified were anticardiolipin antibod-
Avenue, Toledo, OH 43614-5809. ies, protein S deficiency, hyperhomocysteinemia, and activated pro-
Journal of Perinatology (1999) 19(2) 138 –143
© 1999 Stockton Press. All rights reserved. 0743– 8346/99 $12
138 http://www.stockton-press.co.uk
A Review of HELLP Syndrome Curtin and Weinstein

tein C resistance. The authors speculated that identification of these line) or absolute hypertension ($140/90 mm Hg) present in all
abnormalities may provide a basis for pharmacologic management in patients. Weinstein reported right upper quadrant tenderness to palpa-
subsequent pregnancies. tion in all patients and edema in 69%.2 Although HELLP syndrome
Immunologic factors may be the underlying initiator of pre- reportedly can occur in the absence of proteinuria, the few studies
eclampsia and HELLP syndrome. It is possible that a maternal cell- including data on proteinuria show that it is present in varying de-
mediated immune response to pregnancy with cytokine-mediated grees in all patients.2,16
endothelial damage is important in HELLP syndrome. Haeger et al.9
reported increased plasma levels of tumor necrosis factor-a at the Laboratory Diagnosis
time of delivery in patients with HELLP syndrome as compared to The laboratory criteria used for diagnosis of HELLP syndrome vary
normal pregnant controls. Tumor necrosis factor-a, by its effect on among institutions, and comparisons of patients between institutions
endothelial cells and coagulation, may be important in the patho- are complicated by the lack of uniform definitions of the three com-
physiology of preeclampsia and HELLP syndrome. ponents of the disorder. Hemolysis, the most difficult feature to detect
The histologic pattern of injury in the liver in preeclampsia is one and probably the most specific, is frequently not defined. Tests for liver
of fibrin deposition in the periportal sinusoids and hemorrhage into enzymes include several different methods for enzyme analysis with
the space of Disse with resultant hepatocellular necrosis.10 Blood can varied reference ranges. Thrombocytopenia has been classically de-
dissect through portal connective tissue and collect to form subcapsu- fined as ,100,000/ml. The following reviews diagnostic criteria used
lar liver hematoma. Aarnoudse et al.11 studied needle biopsies of the in HELLP syndrome.
liver in patients with HELLP syndrome and noted periportal lesions
consisting of neutrophilic infiltrates, necrosis of hepatocytes and Hemolysis
fibrin microthrombi, and fibrin deposits in the sinusoids. They con- Markers for hemolysis include elevated indirect bilirubin and low
cluded that the basic histopathologic changes in the liver are identical haptoglobin levels. Morphologic features on peripheral blood smear
to those previously described for preeclampsia. Barton and col- that are indicative of hemolysis include schistocytosis, polychromasia
leagues12 found periportal hemorrhage, fibrin deposition, and fatty (implying reticulocytosis), anisocytosis, and poikilocytosis. Schisto-
infiltration in 11 patients with HELLP syndrome who were delivered cytes (red cell fragments) and burr cells (irregularly contracted red
by cesarean section and underwent needle biopsy of the liver under cells with prominent spicules) are associated with small blood vessel
direct visualization. They found no correlation between the severity of disease or fibrin deposition in small blood vessels and thus are fea-
the histologic findings and the clinical and laboratory findings. tures of microangiopathic hemolytic anemia.17 Weinstein defined
HELLP syndrome shares clinical and laboratory features with hemolysis based on the presence of schistocytes and/or Burr cells on
acute fatty liver of pregnancy. Liver biopsy in acute fatty liver of preg- peripheral smear and believed that microangiopathic hemolytic ane-
nancy demonstrates microvesicular fatty infiltration of hepatocytes.13 mia was present to some degree in all patients with HELLP syndrome.2
Liver specimens examined for fat deposition from preeclamptic pa- Most patients with HELLP syndrome are not anemic on presentation
tients with and without liver dysfunction demonstrate significant but may develop a drop in hematocrit out of proportion to the
amounts of microvesicular fat.14 This has led to speculation that amount of blood loss at delivery.2 In a prospective study measuring
preeclampsia, HELLP syndrome, and acute fatty liver of pregnancy are laboratory markers of hemolysis in HELLP syndrome, Wilke and col-
all part of the same pathologic spectrum.14,15 leagues18 found an abnormal peripheral smear in only 11 of 25 pa-
tients. In patients with normal splenic function, abnormal red cells
Maternal Characteristics may be cleared rapidly from the circulation and may not be readily
apparent on peripheral blood smears.19 Sibai recommended that, in
HELLP syndrome occurs in women at all reproductive ages, with a
addition to an abnormal blood smear, increased bilirubin and lactate
mean age of approximately 24 years.2,3 It has been described in most
dehydrogenase (LDH) be required for the diagnosis of hemolysis.20
ethnic groups in the United States and occurs in both primigravida
Although these additional criteria may be useful in the diagnosis of
and multiparous patients. Approximately 19% have pre-existing
hemolysis, neither of these tests is specific. Only elevated indirect
chronic hypertension.2,3
bilirubin is indicative of hemolysis, and LDH is found not only in red
In Sibai and colleagues’ study3 of 442 pregnancies with HELLP
blood cells, but also in liver, skeletal muscle, cardiac muscle, and
syndrome, 70% were diagnosed antepartum and 30% postpartum.
kidney. Paternoster et al.7 demonstrated LDH elevations in some pa-
Mean gestational age at delivery is approximately 32 weeks and
tients with preeclampsia and found bilirubin elevations in only 25%
ranges from 24 to 39 weeks.2,5
of their patients with HELLP syndrome. Reductions in serum hapto-
globin, however, were found in all patients with HELLP syndrome. In
Clinical Presentation
Wilke’s study18 reduced haptoglobin levels were identified in all of
Presenting symptoms include malaise, right upper quadrant tender- their patients with HELLP syndrome. They also performed electro-
ness, nausea, and vomiting. Blood pressure elevation in HELLP syn- phoretic separation of LDH isoenzymes in five patients with HELLP
drome is variable in degree, with relative (.30/15 mm Hg over base- syndrome and found that the elevated plasma LDH levels in 4 of 5
Journal of Perinatology (1999) 19(2) 138 –143 139
Curtin and Weinstein A Review of HELLP Syndrome

patients were associated with a relative increase in the LDH5 isoen- and with a live fetus upon admission, van Pampus and colleagues24
zyme, which is of liver origin.18 In summary, the most sensitive and reported a perinatal mortality rate of 19.6%. There were 10 fetal
objective marker of hemolysis in the HELLP syndrome is a reduced deaths and 1 neonatal death.
serum haptoglobin level. Neonatal survival in infants born to mothers with HELLP syn-
drome is mainly dependent upon gestational age and birth weight at
Elevated Liver Enzymes delivery. It does not appear that HELLP syndrome, independent of
Hepatic injury in HELLP syndrome is manifest by elevation in aspar-
gestational age, increases neonatal mortality. Magann and associates4
tate aminotransferase (AST), formerly termed serum glutamate ox-
at the University of Mississippi showed that ultimate neonatal salvage
aloacetate transaminase (SGOT), and alanine aminotransferase
was primarily related to gestational age and birth weight at delivery
(ALT), formerly serum glutamate pyruvate transaminase (SGPT).
and not due to the severity of the HELLP syndrome.
LDH elevation also occurs in liver injury. Although Weinstein did not
Weinstein reported hematologic abnormalities (thrombocytope-
define the degree of liver enzyme elevation necessary for HELLP syn-
nia, leukopenia, and abnormal peripheral smears) in the neonates
drome diagnosis, Sibai made specific recommendations2,3,20; he de-
born to mothers with HELLP syndrome.2 He speculated that some
fined elevated liver enzymes by an AST (SGOT) value of 70 U/l. The
humoral substance crossing the placenta may result in these findings.
latter value correlated to three standard deviations above the mean in
Neonatal thrombocytopenia has been reported in up to 50% of preg-
their hospital laboratory. Other authors have used elevation of AST
nancies complicated by HELLP syndrome,5 but it is not unique to this
and ALT to two standard deviations above the mean to define the
entity. No correlation has been demonstrated between maternal and
elevated liver enzyme component of HELLP syndrome.7,21
neonatal platelet counts.22 Leukopenia and/or neutropenia were
Low Platelets present in up to 40% of neonates in HELLP syndrome2,22 but have also
In Weinstein’s study, 18 of 29 patients had thrombocytopenia, defined occurred in infants born to mothers with preeclampsia in the absence
as a platelet count of ,100,000/ml, on admission, and all 29 patients of HELLP.25 Weinstein noted that 92% of newborn infants born to
had a nadir platelet count of ,100,000/ml.2 Other authors have also mothers with HELLP syndrome had abnormal peripheral blood
defined thrombocytopenia as a platelet count of ,100,000/ml.3,7,22 smears with burr cells and/or schistocytes.2 Although these findings
are suggestive of hemolysis in the fetus, anemia at birth is uncom-
Maternal Mortality and Morbidity mon in infants from mothers with HELLP syndrome. Little data is
available on liver enzymes in infants born to mothers with HELLP
Maternal mortality in HELLP syndrome ranges from 0% to 4%.2,3,5 In
syndrome. Harms and colleagues22 evaluated liver enzymes in 13
Sibai’s series,3 maternal death in HELLP syndrome was from various
neonates born to mothers with HELLP syndrome and found slight
complications: one from ruptured liver hematoma, one from pulmo-
elevations in only two.
nary embolism, and three from diffuse hypoxic encephalopathy. Ra-
val5 reported one maternal death from DIC, and Weinstein2 reported
one maternal death in a patient with severe microangiopathic hemo- Timing of Delivery
lytic anemia, marked hyperbilirubinemia, and massive ascites. Because of the potential for maternal and perinatal mortality in
As HELLP syndrome frequently occurs before term with an unfa- HELLP syndrome, aggressive management with expeditious delivery
vorable cervix, cesarean section is a common mode of delivery, with has been advocated.2,20,22 This does not preclude vaginal delivery if the
rates ranging from 42% to 98%.2,3,22 Blood product transfusion, with cervix is sufficiently ripe for induction of labor. Sibai states that, in the
its associated risks, has been used frequently. One study noted transfu- absence of laboratory evidence of DIC and absent fetal lung maturity,
sion in 25% of patients.23 DIC has been described in up to 15% of antenatal corticosteroids to accelerate fetal lung maturity can be
patients, half of whom had placental abruption.23 Wound hematoma given with delivery 48 hours later.20 He emphasized the need for con-
or infection is a common complication after cesarean section and tinuous maternal and fetal monitoring during this period. Studies
presents in 7% to 14% of HELLP syndrome patients.16,23 Acute renal that advocate aggressive management and delivery regardless of ges-
failure has been reported as a complication in 8%, the majority sec- tational age demonstrate the lowest perinatal mortality rates.2,22
ondary to acute tubular necrosis.3 Ecclampsia is seen in 4% to 9% of Harms and colleagues22 managed 80 pregnancies with HELLP syn-
HELLP patients.3,23 Other complications reported include severe ascites drome with immediate cesarean section, regardless of gestational age,
(8%), pleural effusion (6%), pulmonary edema (6%), and subcapsu- and reported an overall perinatal mortality rate of 5.6%.
lar liver hematoma requiring laparotomy (1%).3 In some cases, the laboratory abnormalities in HELLP syndrome
will resolve with conservative management. Van Pampus et al.24 re-
Perinatal Mortality and Morbidity
ported a series of 51 patients with HELLP syndrome who were man-
Perinatal mortality in HELLP syndrome ranges from 5% to nearly aged expectantly in order to prolong pregnancy; delivery was per-
20%.2,16,22,24 Studies that advocate expectant or temporizing manage- formed mainly for fetal indications. In 30% laboratory abnormalities
ment have higher perinatal mortality rates, mainly because of still- normalized. The median prolongation of pregnancy in their entire
births.16,24 In a study of 51 patients with HELLP syndrome not in labor group of patients was only 3 days. There were no maternal deaths in
140 Journal of Perinatology (1999) 19(2) 138 –143
A Review of HELLP Syndrome Curtin and Weinstein

this series but forms of maternal morbidity included eclampsia, anti- that platelet counts continued to decrease until 24 to 48 hours after
hypertensive treatment, temporary hemiplegia, and postpartum deep delivery. A platelet count of .100,000/ml was spontaneously
venous thrombosis. The perinatal mortality rate was 19.6% with all achieved within 72 hours of the platelet nadir in most patients in their
deaths being stillbirths. study. LDH levels peaked at 24 to 48 hours after delivery with a down-
Visser and Wallenburg16 compared 128 patients with HELLP ward trend in LDH by the fourth postpartum day. Martin et al.28 have
syndrome to 128 patients with preeclampsia at a gestational age of also advocated the use of dexamethasone in the postpartum period to
,34 weeks. They treated each group with bedrest, pharmacologic accelerate recovery. They performed a retrospective study of 43 women
vasodilatation, and plasma volume expansion under central hemody- with postpartum HELLP syndrome who were treated with dexametha-
namic monitoring. They noted no difference in pregnancy prolonga- sone (10 mg IV at 12-hour intervals until disease remission) com-
tion or perinatal mortality between the two groups and concluded that pared with 237 similar patients who received no steroids. They re-
their data did not support a general recommendation of prompt ter- ported more rapid normalization of platelet counts and LDH values
mination of pregnancy in HELLP syndrome. There were no maternal and a reduction in transfusion, respiratory therapy, invasive hemody-
deaths in either group. The mothers with HELLP syndrome had signif- namic monitoring, morbidity secondary to infection, and length of
icantly more hemorrhagic problems, mainly wound hematomas postpartum hospital course. One prospective randomized trial evalu-
following cesarean section. The authors noted that 43% of patients ated the routine initiation of dexamethasone in patients with postpar-
with HELLP syndrome had a complete antepartum resolution. In this tum HELLP syndrome.29 The steroid-treated group had a significantly
group of patients median prolongation of pregnancy was 21 days as increased platelet count by 30 hours postpartum in comparison to
compared to 10 days in the entire group of HELLP patients. They had controls. There was no difference in the incidence of abnormal uter-
11 stillbirths with a median birth weight of 680 gm among the HELLP ine bleeding or wound infection in the treated and untreated groups.
patients. Although fetal distress was recognized before stillbirth in 10 The authors found no significant difference in blood pressure, urinary
of 11 cases, the authors did not intervene because fetal growth restric- output, LDH, AST, or ALT values between the dexamethasone-treated
tion at an early gestational age was believed to portend a poor prog- patients and controls at any time by 72 hours postpartum.
nosis for meaningful neonatal survival. Unfortunately, one stillbirth,
caused by placental abruption, occurred at 34 weeks with a birth Management of Selected Complications
weight of 2370 gm. Anemia
Magann and associates26 evaluated the effect of antepartum Blood components utilized in patients with HELLP syndrome have
corticosteroids in preterm HELLP syndrome in a prospective, random- included packed red cells, fresh frozen plasma, and platelets. The
ized study of 25 patients with HELLP syndrome. Twelve pregnancies decision to transfuse red blood cells should be based on clinical as-
with HELLP syndrome, a mean gestational age of 30.7 weeks, and a sessment of the patient’s status rather than an arbitrary hemoglobin
mean admission platelet count of 69,300/ml were given dexametha- or hematocrit value. There are relatively few specific clinical indica-
sone (10 mg) intravenously every 12 hours until delivery. The control tions for the use of fresh frozen plasma.30 Coagulopathy without an
group was 13 HELLP patients with a mean gestational age of 32.8 obvious source of bleeding, such as placental abruption or subcapsu-
weeks and a mean platelet count of 106,800/ml who received no ste- lar liver hematoma, is uncommon in HELLP syndrome.23 Therefore
roids. There were no patients with platelet counts of ,50,000/ml the need for FFP transfusion in HELLP syndrome would be fairly
included in this study. Delivery was performed for a deteriorating infrequent.
maternal or fetal condition, gestational age .34 weeks, or a platelet
count of #50,000/ml. The authors found the dexamethasone-treated Thrombocytopenia
group had significantly increased urinary output and platelet counts Platelet transfusion therapy is an area of medicine based largely on
and reduced ALT and LDH levels over time in comparison to the un- consensus rather than evidence-based guidelines.31,32 Therapeutic
treated group. The steroid group had a longer interval from initiation platelet transfusion is believed to be indicated in patients with signifi-
to delivery than the control group (41 versus 15 hours). The authors cant bleeding if the platelet disorder is likely to be causing or contrib-
noted that steroids stabilized disease in HELLP syndrome and that the uting to this bleeding and the platelet count is ,50,000/ml.31 Al-
effect was temporary. though a platelet count of ,50,000/ml in patients undergoing
Expectant management, temporizing or corticosteroid treatment surgery is frequently cited as a threshold for prophylactic platelet
cannot be advocated when there is a reasonable chance of fetal sur- transfusion,33 the risk of bleeding has not been defined. Platelet trans-
vival and the diagnosis of HELLP syndrome is based on strict crite- fusion is believed to be contraindicated in thrombotic thrombocytope-
ria.23 The lowest perinatal mortality rates occur with aggressive nic purpura and hemolytic uremic syndrome32; these disorders are
management.2,22 characterized by platelet consumption, and are in this respect, similar
to HELLP syndrome.
Post Partum Course Roberts and colleagues,34 in a retrospective, descriptive study of
Martin and colleagues27 evaluated the time course of disease resolu- intrapartum platelet counts in HELLP syndrome, reported an antepar-
tion in 158 postpartum patients with HELLP syndrome. They found tum platelet count of #40,000/ml to be predictive of postpartum
Journal of Perinatology (1999) 19(2) 138 –143 141
Curtin and Weinstein A Review of HELLP Syndrome

bleeding problems. The types of bleeding problems encountered were count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol
from abdominal incision sites after cesarean delivery, and episiotomy 1982;142:159 – 67.
sites after vaginal delivery. Mucus membrane bleeding was not a 3. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal
clinically significant problem and no patients had central nervous morbidity and mortality in 442 pregnancies with hemolysis, elevated liver en-
system bleeding. The effect of prophylactic platelet transfusion was zymes, and low platelets. Am J Obstet Gynecol 1993;169:1000 – 6.
studied in patients with platelet counts of ,40,000/ml. They found 4. Magann EF, Perry KG, Chauhan SP, Graves GR, Blake PG, Martin JN. Neonatal
no difference in postpartum bleeding problems between the patients salvage by weeks gestation in pregnancies complicated by HELLP syndrome. J
who received prophylactic platelet transfusion and those who did not. Soc Gynecol Invest 1994;1:206 –9.
Furthermore, the postpartum platelet counts in the transfused patients
5. Raval DS, Co S, Reid MA, Pildes R. Maternal and neonatal outcome of pregnan-
were not significantly different from the patients who were not trans- cies complicated with maternal HELLP syndrome. J Perinatol 1997;17:266 –9.
fused. The data suggest that prophylactic platelet transfusion in
HELLP syndrome is not of value in preventing bleeding complications 6. Van Beek E, Peters LLH. Pathogenesis of preeclampsia: a comprehensive model.
Obstet Gynecol Surv 1998;53:233–9.
and that transfused platelets are quickly consumed.
7. Paternoster DM, Stella A, Simioni P, Mussap M, Plebani M. Coagulation and
Subcapsular Hematoma plasma fibronectin parameters in HELLP syndrome. Int J Gynecol Obstet 1995;
Subcapsular liver hematoma has been reported in 1% of pregnancies 50:263– 8.
complicated by HELLP syndrome and may result in maternal death.3
8. Dekker GA, de Vries JIP, Doelitsch PM, et al. Underlying disorders associated with
Computed tomography, magnetic resonance imaging, or ultrasound severe early-onset preeclampsia. Am J Obstet Gynecol 1995;173:1042– 8.
can be used to detect and monitor this complication.35 Barton and
Sibai35 have recommended close observation in women with unrup- 9. Haeger M, Unander M, Andersson B, Tarkowski A, Arnestad JP, Bengtsson A.
Increased release of tumor necrosis factor-alpha and interleukin-6 in women
tured subcapsular hematoma, provided that maternal condition is
with the syndrome of hemolysis, elevated liver enzymes and low platelet count.
stable. Hematoma size can be followed by serial computed tomogra-
Acta Obstet Gynecol Scand 1996;75:695–701.
phy or ultrasound until the defect resolves. Ruptured liver hematoma
is a surgical emergency with evacuation and drainage of the hema- 10. Crawford JM. The liver and the biliary tree. In: Cotrans RS, Kumar V, Robbins SL,
toma, packing as needed and suturing of lacerations if possible.35 editors. Robbins Pathologic Basis of Disease, 5th Edition. Philadelphia: WB
Saunders & Co.; 1994. p. 875.
11. Aarnoudse JG, Houthoff HJ, Weits J, Vellenga E, Huisjes HJ. A syndrome of liver
Recurrence Risk in Subsequent Pregnancies damage and intravascular coagulation in the last trimester of normotensive
Sullivan et al.21 found, in patients with a prior pregnancy affected by pregnancy: a clinical and histopathological study. Br J Obstet Gynaecol 1986;93:
HELLP syndrome and a nadir platelet count of #100,000/ml, a 19% 145–55.
incidence of HELLP syndrome and a 23% incidence of preeclampsia- 12. Barton JR, Riely CA, Adamec TA, Shanklin DR, Khoury AD, Sibai BM. Hepatic
eclampsia in subsequent pregnancies. Sibai and colleagues36 at the histopathologic condition does not correlate with laboratory abnormalities in
University of Tennessee reported recurrent preeclampsia in 19% but HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count).
recurrent HELLP syndrome in only 3%. Am J Obstet Gynecol 1992;167:1538 – 43.
13. Schorr-Lesnick B, Lebovic E, Dworkin B, Rosenthal WS. Liver diseases unique to
pregnancy. Am J Gastroenterol 1991;86:659 –70.
Conclusion
14. Minakami H, Oka N, Sato T, Tamada T, Yasuda Y, Hirota N. Preeclampsia: a
Widespread recognition and earlier diagnosis of HELLP syndrome as a microvesicular fat disease of the liver? Am J Obstet Gynecol 159:1043–7.
variant of severe preeclampsia have lead to an improvement in mater-
nal and perinatal outcome. Unfortunately, the only cure for HELLP 15. Dani R, Mendes GS, de Laurentys Medeiros J, Peret FJ, Nunes A. Study of the liver
changes occurring in preeclampsia and their possible pathogenetic connection
syndrome is delivery, even at early gestational ages. Further advance-
with acute fatty liver of pregnancy. Am J Gastroenterol 1996;91:292– 4.
ment in prevention and possibly treatment of the disorder will rest
upon a more complete understanding of the etiology and pathogene- 16. Visser W, Wallenburg HCS. Temporising management of severe pre-eclampsia
sis of preeclampsia. At this time, the clinician is advised to consider with and without the HELLP syndrome. Br J Obstet Gynaecol 1995;102:111–7.
aggressive management with expeditious delivery when treating a 17. Morris MW, Davey FR. Basic examination of blood. In: Henry JB, editor. Clinical
patient with HELLP syndrome. Diagnosis and Management by Laboratory Methods, 19th Edition. Philadelphia:
WB Saunders & Co.; 1996. p. 579.
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A Review of HELLP Syndrome Curtin and Weinstein

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