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HELLP syndrome: an atypical presentation


Caroline L. Stella, MD; Khurram M. Malik, MD; Baha M. Sibai, MD

H ELLP (hemolysis, elevated liver


enzymes and low platelets) syn-
drome accounts for 0.2-0.6% of all preg-
HELLP syndrome without proteinuria or hypertension is rare. We present the case of a
33-year-old primigravid who initially was diagnosed with pulmonary embolism. The
nancies. This condition comprises 10% patient was readmitted with a diagnosis of subcapsular hematoma and eventually
of the cases of severe preeclampsia and diagnosed with HELLP syndrome with a sequela of hepatic rupture.
approximately 50% of eclampsia cases.
Key words: atypical presentation, HELLP syndrome, hepatic rupture
Preeclampsia, eclampsia, and HELLP
syndrome afflict many women and result
in a large percentage of maternal and 122/82 mmHg, a pulse of 84, and a respi- to severe intensity, duration of 6 hours
perinatal morbidities. It is important ratory rate of 20. Physical examination and intensity of 8/10. The patient had a
that clinicians make an accurate diagno- was pertinent with reproducible mid- blood pressure of 136/71 mmHg, pulse
sis of these conditions, to prevent any ad- sternal chest wall tenderness. Other find- of 68, and a respiratory rate of 24, with
verse outcome. In general, most women ings included hemoglobin of 13.7 g/dL, direct tenderness at the right upper
will have a classical presentation of pre- hematocrit of 41%, platelet count of quadrant without rebound or guarding.
eclampsia (hypertension and protein- 179,000 mm3, creatinine of 0.8 mg/dL, A right upper quadrant sonogram re-
uria) at ⬎ 20 weeks gestation. However, aspartate aminotransferase (AST) of 94 vealed a suspicious subcapsular hema-
recent studies suggested that some IU/L, alanine transaminase (ALT) of 153 toma (Figure 1) confirmed by an ab-
women will develop preeclampsia with- IU/L, and total bilirubin of 0.5 mg/dL. dominal CT scan (Figure 2). The
out the manifestations of classical symp- Subsequently, urinalysis was ordered patient’s laboratory findings were as fol-
toms. Atypical cases are those that may and noted to be negative for protein. A
develop prior to 20 weeks, or present lows: hematocrit 35%, platelet count
helical chest CT scan showed decreased 142,000 mm3, AST 192 IU/L, and ALT
with signs and symptoms of preeclamp- attenuation in the distal branches of both
sia without hypertension or proteinuria, 183 IU/L. Low molecular weight heparin
right and left lower lobe pulmonary ar- was discontinued. After 8 hours, the pa-
or those cases with subtle abnormalities teries, suspicious of bilateral pulmonary
in laboratory findings. tient’s platelet count dropped to 41,000
emboli. Venous Doppler studies of the mm3 and hematocrit was 27%. Due to
lower extremities, however, were nega-
C ASE R EPORT worsening maternal status, an emergent
tive. Fetal ultrasound revealed an esti-
We present the case of a 33-year-old pri- placement of an inferior vena cava filter
mated fetal weight of ⬍ 10th percentile
migravida at 31 weeks gestation with a and a cesarean section were performed.
and a biophysical profile of 8/8. The peri-
chief complaint of sudden shortness of A viable male infant was delivered with
natologist consulted recommended cor-
breath. The patient also complained of Apgars of 5 and 7 at 1 and 5 minutes and
ticosteroids for fetal lung maturity and
an intense, sharp, stabbing midsternal a birthweight of 1250 g. Intraoperative
twice weekly follow-up. The diagnosis at
chest pain (intensity 9/10) radiating to findings revealed massive hemoperito-
this time was pulmonary embolism. The
the back. The patient indicated that the patient was initially anticoagulated with neum and a diffusely congested liver
pain intensified with deep breathing or intravenous heparin (therapeutic dose) with 2 capsular hematomas covering ar-
when lying flat on her back/stomach. and was subsequently maintained on low eas of spontaneous rupture. Hemostasis
The patient also has a history of smok- molecular weight heparin at 10,000 units was achieved by coagulating the de-
ing, which continued during pregnancy. every 12 hours. The following day, the nuded liver surfaces using argon beam.
In the emergency room, the patient patient’s platelet count subsequently The postoperative diagnosis was HELLP
presented with a blood pressure of dropped to 100,000 mm3. This was at- syndrome. Magnesium sulfate was given
tributed to heparin use. Platelet count for seizure prophylaxis. The patient was
subsequently increased to 139,000 mm3 transfused with multiple packed red
From the Division of Maternal-Fetal (Table). The patient was discharged blood cells and other blood products.
Medicine, University of Cincinnati College home 2 days later on low molecular The patient was referred to our institu-
of Medicine, Cincinnati, OH. tion’s transplant team for possible liver
weight heparin at 10,000 units every 12
Received Sept. 21, 2007; revised Dec. 11, hours. transplantation. Follow-up abdominal
2007; accepted Dec. 21, 2007.
The patient returned to the emergency CT scan (Figure 3) showed a subcapsular
Reprints not available from the authors.
room 5 days later with a chief complaint hematoma without a noticeable increase
0002-9378/free in size. The patient was discharged after
of acute epigastric pain radiating to the
© 2008 Mosby, Inc. All rights reserved.
doi: 10.1016/j.ajog.2007.12.034 back. The patient indicated that the pain 11 days in stable condition. On fol-
began 6 hours prior and with moderate low-up visits with her obstetrician, the

e6 American Journal of Obstetrics & Gynecology MAY 2008


www.AJOG.org Case Report

TABLE
Trends in the laboratory values
Platelets Total bilirubin Creatinine Urine
Day protein (mm3) AST (IU/L) ALT (IU/L) (mg/dL) (mg/dL) (mg/dL)
First admission
.......................................................................................................................................................................................................................................................................................................................................................................
1 179,000 194 153 0.5 0.8 (⫺)
.......................................................................................................................................................................................................................................................................................................................................................................
2 100,000 83 112 0.4 0.6 (⫺)
.......................................................................................................................................................................................................................................................................................................................................................................
a
3 139,000 (⫺) (⫺) (⫺) (⫺) 0
................................................................................................................................................................................................................................................................................................................................................................................
Second admission
.......................................................................................................................................................................................................................................................................................................................................................................
1 142,000 192 183 0.4 0.6 30
................................................................................................................................................................................................................................................................................................................................................................................
1, 8 hours 41,000 1280 740 1.4 (⫺) (⫺)
.......................................................................................................................................................................................................................................................................................................................................................................
2 66,000 1069 79 1.6 0.6 (⫺)
.......................................................................................................................................................................................................................................................................................................................................................................
11 287,000 86 198 1.8 0.5 30
................................................................................................................................................................................................................................................................................................................................................................................
⫺, not done
a
After steroid administration.
Stella. HELLP syndrome. Am J Obstet Gynecol 2008.

patient was reported to exhibit normal haptoglobin levels, elevated lactate dehy- rupture in its early stages because of its
physical findings. drogenase (LDH) levels, and a signifi- varied presentation.7 The key to diagno-
cant drop in hemoglobin levels.2 The sis is, therefore, a high index of
C OMMENT second component of the HELLP syn- suspicion.
To date, there is considerable variation drome triad is elevated liver enzymes. A crucial point to making the diagno-
in the definition and terminology of This is likewise defined imprecisely de- sis of HELLP syndrome is the consider-
HELLP syndrome.1 The diagnostic crite- pending on the literature reviewed. ation of hepatic rupture secondary to an-
ria used is not only variable but inconsis- Weinstein3 defined elevated liver en- ticoagulant therapy. Numerous case
tent.2 Hemolysis, defined as the presence zymes as abnormal serum levels of AST, reports allude to hepatosplenic rupture
of microangiopathic hemolytic anemia ALT, and bilirubin values. However, the secondary to antithrombolytic agents,
is the hallmark of HELLP syndrome.3 clear-cut cut-off levels were not specifi- warfarin, and intravenous heparin and
The expected laboratory findings in- cally defined. In subsequent clinical newer unfractionated heparin prepara-
clude microangiopathic hemolysis, ab- studies, abnormal AST and ALT levels tions such as tinzaparin.8-11 In a case re-
normal peripheral smear (schistocytes, were reported to be 17 to 72 IU/L, while port of a splenic rupture secondary to in-
burr cells, echinocytes), elevated serum abnormal platelet count ranged from
bilirubin (indirect form), low serum 75,000 to 150,000 mm3.4-6 Of note, hy-
pertension and/or proteinuria may be FIGURE 2
absent in 10-15% of preeclamptic Abdominal CT scan showing a
FIGURE 1 patients.2 low-attenuation fluid collection
Right upper sonogram showing Our case report attempts to illustrate of the liver consistent
a complex right hepatic the diagnostic dilemma that a clinician with subcapsular hematoma
subcapsular fluid collection faces in diagnosing an atypical presenta-
tion of HELLP syndrome. As illustrated
by this case, HELLP syndrome can occur
in the setting of a normotensive patient
without proteinuria, but with other
symptoms present (right upper quad-
rant pain, epigastric pain, shortness of
breath, and retrosternal chest pain).
HELLP syndrome is not always evident
in cases wherein the expected hemolysis,
Image published with permission of Kyuran Ann thrombocytopenia, or liver enzyme ele- Image published with permission of Kyuran Ann
Choe, MD. vation occurs singularly. It is likewise ex- Choe, MD.
ceedingly difficult to diagnose hepatic

MAY 2008 American Journal of Obstetrics & Gynecology e7


Case Report www.AJOG.org

bocytopenia, and mild elevations in liver by HELLP (hemolysis, elevated liver enzyme
FIGURE 3 levels, and low platelet count) syndrome
enzymes are possible manifestations of
Abdominal CT scan showing a classification. Am J Obstet Gynecol 1999;
early HELLP syndrome. In this particu-
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lar case, the patient subtly presented with 2. Sibai BM. Diagnosis, controversies and man-
with further development of
shortness of breath, chest pain, and ele- agement of the HELLP syndrome. Obstet Gy-
areas with increased
vated liver enzymes unaccompanied by necol 2004;103:981-91.
attenuation within the
thrombocytopenia or hemolysis. When 3. Weinstein L. Syndrome of hemolysis, ele-
hematoma consistent with vated liver enzymes, and low platelet count: a
the succeeding laboratory results mani-
organizing blood severe consequence of hypertension in preg-
fested HELLP syndrome, the initial diag-
nancy. Am J Obstet Gynecol 1982;142:
nosis of pulmonary embolism was still 159-67.
maintained. Third, the rebound increase 4. Tompkins MJ, Thiagarajah S. HELLP (hemo-
in platelets was attributed to steroid ad- lysis, elevated liver enzymes, and low platelet
ministration only.4 Hence, the diagnosis count) syndrome: the benefit of corticosteroids.
of HELLP syndrome was eliminated by Am J Obstet Gynecol 1999;181:300-9.
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of high-dose corticosteroid therapy for patients
Last, on readmission, the fact that the pa-
with HELLP (hemolysis, elevated liver enzymes
tient exhibited a severe right upper and low platelet count) syndrome. Am J Obstet
Image published with permission of Kyuran Ann quadrant pain secondary to subcapsular Gynecol 2000;183:921-4.
Choe, MD. hematoma should have clued the clini- 6. Weinstein L. Preeclampsia/eclampsia
cian to a finding of HELLP syndrome. with hemolysis, elevated liver enzymes and
Fortunately, the patient survived such a thrombocytopenia. Obstet Gynecol 1985;66:
devastating and potentially fatal sequela 657-60.
travenous heparin use, anticoagulation 7. Weinstein L. It has been a great ride: the his-
therapy spanned 5 days.12 In contrast, of liver rupture. tory of HELLP syndrome. Am J Obstet Gynecol
intravenous heparin treatment in our In summary, we describe a case of 2005;193:860-3.
patient lasted only for a day. It is indeed a HELLP syndrome with an atypical pre- 8. Blankenship JC, Indeck M. Spontaneous
truly rare event when an organ sponta- sentation initially diagnosed and treated splenic rupture complicating anticoagulant or
as pulmonary embolism. We therefore thrombolytic therapy. Am J Med 1993;94:
neously ruptures without any detectable
recommend a rational stepwise ap- 433-7.
lesion. Arguably, this supports a theoret- 9. Dizadji H, Hammer R, Strzyz B, Weisen-
ical prior pathology in this patient’s liver proach toward the diagnosis of HELLP
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Additionally, the subcutaneous heparin maternal and perinatal morbidity and Norgren L. Spontaneous rupture of liver and
mortality. In a patient presenting with spleen with severe intra-abdominal bleeding
dose that our patient received is not re-
any unexplainable laboratory abnormal- during streptokinase treatment of deep venous
ported anywhere in literature as a dose thrombosis. Vasa 1977;6:369-71.
that could potentially cause the rupture ity of either hemolysis or elevated liver
11. Taccone FS, Starc JM, Sculier JP. Splenic
of an intraabdominal viscera.13 It would enzymes or low platelets, the index of spontaneous rupture (SSR) and hemoperito-
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There were several pitfalls of diagnosis returns with worsening symptoms, we
capsulaire rate saine au cours d’héparinisation
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e8 American Journal of Obstetrics & Gynecology MAY 2008