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Review Article

National Journal of Medical and Dental Research, April-June 2013: Volume-1, Issue-3, Page 62-65

HELLP Syndrome - A Therapeutic Challenge Case Report


With Review of Literature
Satyavir Singh Dhama A, Suchitra Malhotra A, Mohinder Kumar B, Gaurav JainC
A
Associate Professor, Dept of Anaesthesia, Teerthanker Mahaveer Medical College,
Moradabad, U.P.
B
Associate Professor, Dept of General Surgery, Teerthanker Mahaveer Medical
College, Moradabad, U.P.
C
Assistant Professor, Dept of Anaesthesia, Teerthanker Mahaveer Medical College,
Moradabad, U.P.
Manuscript Reference
NJMDR_107_13

No.

Abstract:
HELLP Syndrome is a life threatening obstetric emergency usually considered to be
a variant or complication of pre eclampsia. Both conditions usually occur during the
later stages of pregnancy or sometimes after child birth. We are here, reporting a
case of 25 year old woman who presented with classical HELLP syndrome in which
prompt diagnosis and appropriate anesthetic management helped us to reduce
morbidity and mortality related with HELLP syndrome. The policy that prevention
is far more important than cure coupled with proper pre anesthetic evaluation will
help to reduce the incidence of anesthetic complications arising during .regional
anesthesia. The role of regional anesthesia in management of HELLP syndrome is
reviewed.
Keywords: HELLP Syndrome, Spinal Anesthesia, Platelet Count
Natl J Med Dent Res 2013; 1(3) : 62-65

Case Report:

Date of submission: 16 January 2013


Date of Editorial approval: 23 January 2013
Date of Peer review approval: 16 March 2013
Date of Publication: 30 June 2013
Conflict of Interest: Nil; Source of support: Nil
Name and addresses of corresponding author:
Dr Satyavir Singh Dhama ,MD (Anaesthesia),
Department of Anaesthesia ,
Teerthanker Mahaveer Medical College & Research
Centre,
Moradabad, India.
Mobile: - 919719120071,
E-mail: malhotramsfrcs@yahoo.co.in

A 25 year old primigravida with 35 weeks of


gestation was admitted in the hospital with
upper abdomen pain, nausea, vomiting and
blurred vision. On examination, her pulse
rate was 125/min, B.P was 210/112 mm Hg,
SPo2 was 95% and patient had associated
pedal edema. The diagnosis of HELLP
syndrome with severe pre- eclampsia was
made on the basis of complete laboratory
and radiological investigation which
showed
Hb 7.3 gm/dl, TLC 9000/ml,
Blood urea 80mg/dl, creatinine 1.8 .mg/dl,
bleeding time 3.5 min, clotting time 8 min,
urine examination showed severe protein
urea ., platelet counts 63000/ml, liver
enzymes AST 1452 IU/l, ALT 1102 IU/l and
LDH 1388 IU/l, PT 18 control 13sec, INR
1.5, Serum bilirubin 1.5 D-dimer-8000.

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Ultrasound showed hepatomegaly, 35


weeks+/- 1 week live fetus in uterus,
oligohydramnios, and weight of the fetus
was 2.20 kg.
Patient was advised to arrange three units
of fresh platelets and two units of fresh
blood. Based on our previous experience
and literature on HELLP syndrome, it was
decided to conduct the delivery by caesarean
section (LSCS) under spinal anesthesia.
Patient was pre loaded with 1000ml of
Ringer lactate (RL) and 20 mg of labetalol
was given as intravenous (IV) bolus, along
with magnesium sulphate 5gm IV bolus and
12 mg betamethasone 12 hourly.
Spinal anesthesia was given in left lateral
position with full aseptic precautions.

National Journal of Medical and Dental Research, April-June 2013: Volume-1, Issue-3, Page 62-65

Bupivacaine heavy0.5 %, 1.75 ml was given. The course


of the anesthesia and surgery was uneventful up to the
delivery of the baby. Excessive oozing of blood from
uterus, abdominal incision and hematuria was observed
just after placental removal. Whole surgical procedure
was completed in just half an hour. Blood loss was
.approximately 1.5 liters. Two unit of fresh blood and
two units platelets transfusion were given postoperatively
the patient was shifted to intensive care unit (ICU) and
platelet transfusion continued in ICU. Blood sample sent
from ICU showed haemoglobin-6.8gm/dl, platelet -73000/
ml,sodium-148meq/dl,potassium-5.5meq/dl,Creatinine2.2mg/dland Urea -82mg/dl. As hemoglobin and platelets
were low it was decided to transfuse two units of fresh
platelets and two units fresh blood. The patient recovered
rapidly and was shifted to obstetrics ward. On the 4th day
the repeat investigation were found to be within normal
limits and patient was discharged on the 10th day after
removing the sutures.

Discussion:
HELLP syndrome is a life threatening obstetric
complication usually associated with pre eclampsia. Its
incidence is 0.5% to 0.9% of all pregnancies. It is related
with severe pre eclampsia (10% to 20%). Both conditions
occur after twenty weeks of gestation and may occur after
5 to 12 weeks of childbirth. HELLP is acronym for; H
haemolysis, EL -elevated liver enzymes, LP - low platelets.
The term HELLP syndrome was first used by Louis
Weinstein in 1982 [1]. Although the same clinical features
and investigations were noticed in 1954 by Pritchard [2].
The onset was gradual. Clinical features include severe
headache (30%), malaise (90%); nausea, vomiting (30%);
band pain around upper abdomen (65%), and edema.
Disseminated intravascular
coagulation is also seen
in about 20%of all women with HELLP syndrome [3].
However, the path physiology is dynamic .Preeclampsia
is a common and severe disease in pregnancy, a major

cause of maternal and fetal morbidity and mortality. The


main features of the disease are de novo hypertension
after the 20th gestational week and proteinuria, and it is
frequently accompanied by edema and other subjective
symptoms. The origin of the disease is the placenta, but
its sequel affect multiple organ systems[4] that produce a
number of outcomes, whether the patient has evidence of
thrombocytopenia and elevated liver function tests alone or
whether fragmented RBCs are present in the blood smear,
the serum bilirubin is elevated or other abnormalities
such as coagulopathy or renal insufficiency are present
with greater involvement of the endothelium of the liver
in preeclampsia, more red cells are hemolyzed and more
hepatic ischemia resulted in higher bilirubin levels and
impaired coagulation tests. Hypertension in pregnancy is
increasing in prevalence and incidence and its treatment
becoming more common place Associated complications
of pregnancy, including end-organ damage, preeclampsia,
eclampsia, and postpartum eclampsia, are leading sources
of maternal and fetal morbidity and mortality, requiring
an emergency physician to become proficient with their
identification and treatment [5].
HELLP syndrome
is complicated with acute renal failure in 84% cases
.Pregnant women who present with hellp syndrome can be
misdiagnosed in the initial stages thereby increasing the
risk of liver failure and morbidity. In a patient with possible
hellp syndrome following blood tests should be performed.
Full blood count, liver function tests including enzymes,
renal function tests, coagulogram, serum electrolytes, and
D-dimer .We conducted all the above investigations in our
patient and we diagnosed our patient was suffering from
HELLP syndrome as D-dimer was positive. A positive
D- dimer test in a patient of preeclampsia is predictive of
hellp syndrome [6]. D dimer is a more sensitive indicator
of sub clinical coagulopathy and may be positive before
other coagulation studies become abnormal. According to
Missisippi classification [7, 8] and Tenesse classification
the disease can be classified as mild, moderate and severe
the same is summarized in Table-1.

Table: 1
Mild

Moderate

Severe

Platelet count

150000to 100000

100000,>50,000

<50000

AST/ALT

>40 IU

>70IU/L

>70 IU

LDH

>600IU/L

>600IU/L

>600IU/L

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National Journal of Medical and Dental Research, April-June 2013: Volume-1, Issue-3, Page 62-65

Sibai has proposed strict criteria for true or complete


HELLP syndrome platelet <100000, AST>70IU/L,
LDH>600IU/L [9]. Our patient fell into moderate type of
hellp syndrome. The only effective treatment of HELLP
syndrome is immediate delivery of baby either by caesarean
section or normal vaginal route [9]. Vigil De Gracia et al
recommended that neuraxial anaesthesia may be safely
administered in the patient with HELLP syndrome without
DIC or prolonged Prothrombin time [10]. We decided to
perform caesarean section under spinal anaesthesia even
though d-dimer and prothrombin time were marginally
raised because of our past experience in similar cases. Sibai
et al [9] also recommended Spinal/epidural anaesthesia in
HELLP syndrome if there is hemodynamic stability as
epidural anesthesia is risky in patients with coagulopathy.
This case was operated under spinal anesthesia because
patient was hemo- dynamically stable. Administration of
corticosteroid can minimize the degree of intravascular
endothelial injury and improve blood flow while decreasing
hepatocyte and platelet consumption in HELLP syndrome
[11]. O Brien et al assessed the beneficial effects of steroids
on the frequency of complications after regional anesthesia
[12]. A study done by Keltor J G Hunter DJS reported that
only platelet count is not responsible for complication in
hellp syndrome but it was the platelet function that was
responsible ,but it is not proved till date because platelet
function is not easy to evaluate[13, 14]. Marcellver cauteren
et. al conducted a study[15,16,17,18] on HELLP syndrome
from 1993-2008 at Antwrep university They showed that
anesthesia of choice for caesarean section was combined
spinal epidural anesthesia (CSEA). Epidural anesthesia
was only given in selected cases where a catheter was
already placed for painless delivery. They made a guideline
that if platelet count was >90.000/ml any type of anesthesia
could be given. But in case of platelet count <60,000/ml
only general anesthesia should be given. If platelet count
is between 60,000/ml to 90,000/ml choice of anesthesia
depended on the anesthetist.

Conclusion:
Spinal anesthesia can be given safely in HELLP syndrome
with platelet count up to 60,000/ml as platelet functions are
not impaired and spinal anaesthesia, per say is otherwise
safer and cost effective than general anaesthesia.

References:

1. Wenstein L. Syndrome of hemolysis, elevated liver


enzymes and low platelet count: serious consequence
of hypertension in pregnancy. Am J Obstet Gynaec ol
1982 :142:159-67.
2. Prichard JA, Weisman R Jr, Ratnoff OD, Vosburgh GJ.
Intravascular hemolysis, thrombocytopenia and other
hematologic abnormalities associated with severe
toxaemia of pregnancy Engi Med 1954: 250:89-98.
3. Crosyby ET. Obstetrical anaesthesia for patients with
haemolysis elevated liver enzymes and low platelets.
Can J Anaes 1991:38:227-33.
4. Alasztics B. KuKor Z. Panczel Valents. The
pathophysiology of Pre-eclampsia in view of two stage
model. Ory Hetil 2012:29:153(30)1167-76.
5. Deak TM, Moskovitz JB. Emerg Med Clin North Am.
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G. Pedured
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syndrome Reprod Ser 2007;14:192-6.
8. Hawkins JL, Koonia L M, Palmer SK, GibbsC P.
Anaesthesia related deaths during obstetric deliveri in
the United States 1979-1990.Anaesthesiology 1997;
86:277-84.
9. Sibai B, Taslima MM, E l-Nazar A. Maternal Perinatal
outcome associated with the syndrome of haemolysis,
elevated liver enzymes and low platelet in sever preeclampsia. Amj obstet Gynaecol 1986; 1,55:501-9.
10. Vigil-De Gracia P, Silva S, Montufar C. Anaesthesia
in pregnant women with HELLP syndrome. Int j
Gynaecol obstet 2001; 74:23-7.
11. Rolbin SH, Abbot D. Musclow E. Epidural Anaesthesia
in pregnant women with low platelet counts Obstet
Gynecol 1998. 71:918-20.

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National Journal of Medical and Dental Research, April-June 2013: Volume-1, Issue-3, Page 62-65

12. O. BrienJM, Shumate SA, Satchwell SL, Milligan DA,


Barton JR. Maternal benefits of patients with HELLP
syndrome: impact on the rate of regional anaesthesia.
13. Sanli K, Kayaca N, Yeqin A. Application of regional
anaesthesia in hellp syndrome(Turkish) Genel Tip
Dreg 2005;15:81-4.
14. Beilin Y, Zahn J,Comerford M. Safe epidural analgesia
in thirty parturients with platlet counts between 69000
and 98000/mm3.Anesth Analg 1997;85:385-8.
15. Vigil-De Gracia P, Silva S, Montufar C. Anaesthesia
in pregnant women with HELLP syndrome. Int J
Gynaecol obstet 2001; 74:23-7.

16. Palit S, Palit G, Vercauteren M, Jacquemyn Y. The


choice of anaesthetic technique for C-section in
patients with HELLP syndrome: a retrospective
analysis .RAPM 2008; 33:S.
17. Welter P, De Buck F, Vandermeersch E. Characteristics,
obstetric and anaesthetic outcome of the syndrome of
Hemolysis, Elevated Liver enzymes , Low Platlets
(HELLP) in a tertiary care referral center. Acta
Anaesthesiol Belg 2008; 50:117.
18. Moen V, Dahlren N, Irestedt L. Severe neurogical
complications after central neuraxial blocade in
Sweden.

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