In a pregnant woman presenting with thrombocytopenia, the possibility of HELLP syndrome should always be considered by the treating clinician so as to initiate the therapy at the earliest to prevent the high perinatal mortality and postpartum morbidity. Here we report an unusual case of young Primigravida (postpartum) who presented at Indraprastha Apollo hospitals, New Delhi with altered sensorium, paraperesis, DIC and septic shock. On evaluation she was found to have HELLP syndrome for which Plasmapheresis was given and patient showed remarkable improvement.

1. AN UNUSUAL CASE OF POSTPARTUM THROMBOCYTOPENIA

2. Case Report
AN UNUSUAL CASE OF POSTPARTUM THROMBOCYTOPENIA
J M Dua*, Sudha Kansal*, Nishant Kanodia** and Prashant Nasa***
*Senior Consultant, **Junior Consultant, ***Registrar, Department of Internal Medicine/Critical Care,
Correspondence to: Dr J M Dua, Senior Consultant, Department of Internal Medicine, Indraprastha Apollo Hospitals,
In a pregnant woman presenting with thrombocytopenia, the possibility of HELLP syndrome should always be
considered by the treating clinician so as to initiate the therapy at the earliest to prevent the high perinatal
mortality and postpartum morbidity. Here we report an unusual case of young Primigravida (postpartum) who
presented at Indraprastha Apollo hospitals, New Delhi with altered sensorium, paraperesis, DIC and septic
shock. On evaluation she was found to have HELLP syndrome for which Plasmapheresis was given and
patient showed remarkable improvement.
Key words: HELLP- Hemolysis, Elevated liver enzyme, Low platelet count.
INTRODUCTION
Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India.
Sarita Vihar, New Delhi 110 076, India.
HELLP syndrome, characterized by hemolysis,
elevated liver enzyme levels and a low platelet count, is an
obstetric complication, first described by Louis Weinstein
in 1982 [1]. Many investigators consider the syndrome to
be a variant of preeclampsia, but it may be a separate entity.
The pathogenesis of HELLP syndrome remains unclear.
Early diagnosis is critical because thea morbidity and
mortality rates associated with the syndrome have been
reported to be as high as 25%. Platelet count appears to be
the most reliable indicator of the presence of HELLP
syndrome.
CASE REPORT
A twenty-year old previously healthy female with
normal ANC, who had BP = 160/110 mm Hg followed by
GTCS, underwent emergency LSCS and delivered healthy
2.5 Kg male baby. Postpartum she developed altered
sensorium, low urine output, and hypotension (78/30
mmHg) and was managed with inotropes at a hospital in
Agra. Her investigations revealed Hb -5.6, Platelet count-
51000, TLC-31300, Urea/Creatnine 67.9/1.7, Na/k-134/
6.3 SGPT-391, D-dimer-11.5. She was shifted to Apollo
Hospital Delhi. On admission, she was drowsy, not
following verbal commands and had quadriparesis with
recurrent GTCS. Her BP-110/68 mmHg, P-118/min,
Anemia (+++), other systemic examinations were normal.
Treatment was started with antiepileptics, antibiotics and
mechanical ventilation, inotropes were continued with
sedation.
Her investigations revealed -Hb 4.6, TLC 33800,
Platelet count 1.1 lac (after 2 platelet transfusion) U/C
130/4.3, Na/k 145/5.8, PT 13.5/13, INR 1.1, PTT 27.5/33
Sr Bil 1.55/0.84, AST/ALT -1927/1200, ALP -112 D-dimer
5.3, FDP > 20, LDH 4434, Lactate 8.2, ECG
showed tall T waves V2-V6, USG abd- uterus bulky,
Haemoperitoneum,? Ant uterine wall perforation.2 D
Echo revealed mild MR, TR, LVEF 65% CVP 5, LVEDP-N
and SVR 900. Provisional Diagnosis: Eclampsia,
HELLP syndrome, DIC and Sepsis. Gynecology opinion
taken and advice noted for evacuation of
haemoperitoneum once coagulopathy settles. Haemo
dialysis was given for deranged renal functions and
metabolic acidosis. Multiple packed red cells, platelet on
cell separators and fresh frozen plasma’s were transfused.
In view of worsening sensorium, persistent hemolysis,
worsening Platelet count and renal functions-
Plasmapheresis was started with - 100% replacement.
Antibiotics and antifungal were given according to
culture reports.
Repeat investigations after plasmapheresis revealed -
Hb 7.0, TLC 23200, Platelet count 58000 (35700), AST/
ALT 87/107, Urea/Creat 108/4.7, Na/K 149/3.5, Bil 2.58/
0.96. Neurologically patient started spontaneous eye
opening, but no comprehension, no motor response and
DTR absent in all limbs. Consultation was taken from
Neurologist and NCCT Brain was done which revealed-non
hemorrhagic Infarct in B/L Parasaggital region and
centrum semiovale. Patient was tracheostomised,started
on inj.fraxiparine and shifted out to semi ICU. As patient
developed fever, MRI Brain (Fig.1. a & b) was done
Apollo Medicine, Vol. 7, No. 1, March 2010 64

3. Case Report
(a) (b) (c)
Fig.1. (a) MRI brain, (b) MRI brain-T2 flair- B/L-Parasaggital, occipitoparietal and high frontal infarcts (c) MRV brain-Cortical
65 Apollo Medicine, Vol. 7, No. 1, March 2010
which revealed-B/L Parasaggital, occipitoparietal and
high frontal infarcts and MRV (Fig.1c) showed Cortical
Venous Thrombosis.
After 25th day of treatment she regained
consciousness and started responding to verbal commands
with power 1/5 UL with little finger movements and 1/5 in
LL and no fever. Ambulation started on wheel chair and
she was put on oral feeds.
By 35th day patient was having full comprehension,
started verbalizing but remained quadriparetic and was
discharged on 45th day. Repeated follow ups in OPD
showed gradual neurological improvement and by 2
months she started lifting her hands with power 4/5 in UL
and 1/5 left LL and 2/5 right LL and Plantars B/L
extensors. Repeat lab tests were-AST/ALT 56/123, Hb
13.5g, Platelet count 2.9 lac and treatment continued with
ASA, clopidrogel, iron, calcium and multivitamin
supplements. Thereafter further follow up was not
undertaken.
DISCUSSION
The acronym HELLP was coined in 1982 to describe a
syndrome consisting of hemolysis, elevated liver enzyme
levels and low platelet count [1]. The pathogenesis of
HELLP syndrome is not well understood. The findings of
this multisystem disease are attributed to abnormal
vascular tone, vasospasm and coagulation defects [2]. Till
date, no common precipitating factor has been found. The
syndrome seems to be the final manifestation of some
insult that leads to microvascular endothelial damage and
intravascular platelet activation. Thus begins a cascade
that is only terminated with delivery.
The hemolysis in HELLP syndrome is a
microangiopathic hemolytic anemia.
The elevated liver enzyme levels in the syndrome are
thought to be secondary to obstruction of hepatic blood
flow by fibrin deposits in the sinusoids.
The thrombocytopenia has been attributed to increased
consumption and/or destruction of platelets.
Patients who develop DIC, approximately 0.2 to 0.6%
of all pregnancies [3], generally do so in the setting of
well-developed HELLP syndrome. When preeclampsia is
not present, diagnosis of the syndrome is often delayed
[4].The risk factors for HELLP syndrome differ from
those associated with preeclampsia (Table 1). The
syndrome presents antepartum in 69% and postpartum in
31% of patients [2]. It generally presents in the third
trimester of pregnancy, although in 11% it occurs at less
than 27 weeks of gestation [5]. With postpartum
presentation, the onset is typically within the first 48 hours
after delivery.
Patients present with generalized malaise, epigastric
pain, nausea, vomiting, and with headache [3]. The
differential diagnosis of HELLP syndrome includes acute
Venous Thrombosis
Table 1. Predisposing factors for HELLP syndrome
and preeclampsia
HELLP syndrome Preeclampsia
Multiparous Nulliparous
Maternal Age>25 years <20 years of >45 years
White race Family history of
preeclampsia
History of poor pregnancy Minimal prenatal care
outcome Diabetes mellitus
Chronic hypertension
Multiple gestation

4. Case Report
fatty liver of pregnancy, thrombotic thrombocytopenic
purpura and hemolytic uremic syndrome. Many woman
with this syndrome are initially misdiagnosed with other
disorders, such as cholecystitis, esophagitis, gastritis,
hepatitis or idiopathic thrombocytopenia [3].
The three chief abnormalities found in HELLP
syndrome are hemolysis, elevated liver enzyme levels and
a low platelet count. The serum transaminase levels may
be elevated to as high as 4,000 U per L, but milder
elevations are typical. Platelet counts can drop to as low as
6,000 per mm3, but any platelet count less than 150000 per
mm3 needs attention. The platelet count is the best
indicator of the HELLP syndrome. A positive D-dimer test
in the setting of preeclampsia has recently been reported to
be predictive of patients who will develop HELLP
syndrome [6]. The D-dimer is a more sensitive indicator of
subclinical coagulopathy and may be positive before
coagulation studies are abnormal.
The present classification and diagnostic criteria of
HELLP syndrome is shown in Table 2.
Patients with class 1 HELLP syndrome are at higher
risk for maternal morbidity and mortality than patients
with class 2 or 3 HELLP syndrome [5].
MANAGEMENT
Once the diagnosis of HELLP syndrome has been
established, the best markers to follow are the maternal
LDH and platelet count [7]. The peak lactate
dehydrogenase level shows the beginning of recovery and
subsequent normalization of the platelet count [7]. The
incidence of hemorrhagic complications is higher when
platelet counts are less than 40,000 per mm [8].
Prompt recognition of HELLP syndrome and timely
initiation of therapy are vital to ensure the best outcome
Apollo Medicine, Vol. 7, No. 1, March 2010 66
for mother and fetus. The treatment approach should be
based on the estimated gestational age and the condition of
the mother and fetus (Fig. 2) [9].
Patients with HELLP syndrome should be routinely
treated with corticosteroids. Patients treated with
dexamethasone exhibit longer time to delivery and
facilitates postnatal maturity of fetal lungs [10].
Corticosteroid therapy should be instituted in patients
with HELLP syndrome who have a platelet count of less
than 100,000 per mm3 and should be continued until liver
function abnormalities are resolved and the platelet count
is greater than 100,000 per mm3.
Patient should be treated prophylactically with
magnesium sulfate to prevent seizures.
Antihypertensive therapy should be initiated if blood
pressure is consistently greater than 160/110 mm Hg. This
reduces the risk of maternal cerebral hemorrhage,
placental abruption and seizure. The goal is to maintain
diastolic blood pressure between 90 and 100 mm Hg. The
most common antihypertensive agent’s hydralazine,
labetalol and nifedipine have been used with success.
A hypertensive crisis may be treated with a continuous
infusion of nitroglycerin or sodium nitroprusside.
Between 38% and 93% of patients with HELLP
syndrome receive some form of blood product [11].
Patients do not require transfusion unless the platelet
count drops to less than 20,000 per mm3. Patients who
undergo cesarean section should be transfused if their
platelet count is less than 50,000 per mm3.
Patients with DIC should be given fresh frozen plasma
and packed red blood cells. Plasmapheresis has been
successful in patients with severe laboratory abnormalities
(i.e., a platelet count <30,000 per mm3 and increased liver
function values). In these patients, plasmapheresis has
resulted in an increase in the platelet count and a decrease
in the lactate dehydrogenase level [11,12].
The mortality rate for women with HELLP syndrome
is approximately 1.1% [5]. From 1 to 25 % of affected
women develop serious complications such as DIC,
placental abruption, adult respiratory distress syndrome,
hepatorenal failure, pulmonary edema, subcapsular
hematoma and hepatic rupture. Infant morbidity and
mortality rates range from 10 to 60%; depending on the
severity of maternal disease [3]. Infants affected by
HELLP syndrome are more likely to experience
intrauterine growth retardation and respiratory distress
syndrome [13]. Life threatening neurological
complications of the HELLP syndrome are rare. It
Table 2. Diagnostic criteria of HELLP syndrome
HELLP Tennessee Mississippi
class classification classification
1 Platelets d100-109/L Platelets d50.109/L
AST t70 IU/L AST or ALT t70 IU/L
LDH t600 IU/L LDH t600 IU/L
2. Platelets d100.109/L
t50.109/L
AST or ALT t70 IU/L
LDH t600 IU/L
3. Platelets d150.109/L
t1000.109/L
AST or ALT t40 IU/L
LDH t600 IU/L

5. Case Report
Management of HELLP Syndrome
p p p
EGA <32 weeks EGA 32 to 34 weeks EGA 34 weeks
Administer a corticosteroid Administer a corticosteroid
67 Apollo Medicine, Vol. 7, No. 1, March 2010
includes cerebral or brain stem hemorrhage, thrombosis
and infarctions or cerebral oedema and had been reported
in post partum period [14].
Patients who have had HELLP syndrome have a 19 to
27% risk of developing the syndrome in subsequent
pregnancies[15]. Patients with class 1 HELLP syndrome
have the highest risk of recurrence [15]. Patients who have
had HELLP syndrome may subsequently use oral
contraceptive pills safely [16]. Patients who develop
atypical early-onset preeclampsia or HELLP syndrome
should be screened for the presence of antiphospholipid
antibodies [17].
To date, neither calcium nor aspirin has been
specifically studied in patients with HELLP syndrome.
Although our case showed no deterioration or increase
any complications but has only done benefits till date with
the use of aspirin with clopidrogel and calcium
supplement. One of the studies had suggested that aspirin
therapy might be helpful in selected patients with early-onset
severe preeclampsia [18]. Similarly several other
studies had suggested that calcium might be useful in
preventing preeclampsia in high-risk patients of HELLP
syndrome [19, 20].
REFERENCES
1. Weinstein L. Syndrome of hemolysis, elevated liver
enzymes and low platelet count: a severe consequence
of hypertension in pregnancy. Am J Obstet Gynecol
Manage the patient
based on the clinical
response during a period
of observation
Patient’s
condition
worsens
Patient’s
condition is
stable
Deliver Monitor the
patient in a
tertiary care
facility
Is the patient eligible for
conservative
management?
Deliver
No
Yes
Counsel the patient about the potential
benefit of continuing the pregnancy for
two more weeks to allow more time for
fetal lungs to mature
Transfer the patient to a tertiary
care facility that has a neonatal
intensive care unit
Patient’s condition
worsens
Patient’s condition
is stable
Deliver Monitor the patient
in a tertiary care
facility
p p
p
p
p p
p p
p
p
p
p
p p
p p
Fig 2. Approach to the management of patients with HELLP syndrome.
o
p

6. Case Report
1982;142:159-167.
2. Sibai BM. The HELLP syndrome (hemolysis, elevated
liver enzymes, and low platelets): much ado about
nothing? Am J Obstet Gynecol 1990;162: 311-316.
3. Wolf JL. Liver disease in pregnancy. Med Clin North Am
1996;80:1167-1187.
4. Gleeson R, Farrell J, Doyle M, Walshe JJ. HELLP
syndrome: a condition of varied presentation. Ir J Med
Sci 1996;165:265-267.
5. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM,
Friedman SA. Maternal morbidity and mortality in 442
pregnancies with hemolysis, elevated liver enzymes, and
low platelets (HELLP syndrome). Am J Obstet Gynecol
1993;169:1000-1006.
6. Neiger R, Trofatter MO, Trofatter KF Jr. D-dimer test for
early detection of HELLP syndrome. South Med J
1995;88:416-419.
7. Martin JN Jr, Blake PG, Perry KG Jr, McCaul JF, Hess
LW, Martin RW. The natural history of HELLP syndrome:
patterns of disease progression and regression. Am J
Obstet Gynecol 1991;164:1500-1509.
8. Roberts WE, Perry KG Jr, Woods JB, Files JC, Blake PG,
Martin JN Jr. The intrapartum platelet count in patients
with HELLP (hemolysis, elevated liver enzymes, and low
platelets) syndrome: is it predictive of later hemorrhagic
complications? Am J Obstet Gynecol 1994;171: 799-
804.
9. Sibai BM, Frangieh AY. Management of severe
preeclampsia. Curr Opin Obstet Gynecol 1996;8:110-
113.
10. Magann EF, Graves GR, Roberts WE, Blake PG,
Morrison JC, Martin JN Jr. Corticosteroids for enhanced
fetal lung maturation in patients with HELLP syndrome:
impact on neonates. Aust N Z J Obstet Gynaecol
1993;33:131-135.
11. Martin JN Jr, et al. Plasma exchange for preeclamspia. I.
Postpartum use for persistently severe preeclampsia-eclampsia
Apollo Medicine, Vol. 7, No. 1, March 2010 68
with HELLP syndrome. Am J Obstet Gynecol
1990;162:126-137.
12. Katz VL, Watson WJ, Thorp JM Jr, Hansen W, Bowes
WA Jr. Treatment of persistent postpartum HELLP
syndrome with plasmapheresis. Am J Perinatol 1992; 9:
120-122.
13. Dotsch J, Hohmann M, Kuhl PG. Neonatal morbidity and
mortality associated with maternal haemolysis, elevated
liver enzymes and low platelets syndrome. Eur J Pediatr
1997;156: 389-391.
14. Altamura C, et al. Postpartum cerebellar infarction and
haemolysis, elevated liver enzymes, low platelet
(HELLP) syndrome. Neurol Sci 2005, 26: 40-42.
15. Sullivan CA, Magann EF, Perry KG Jr, Roberts WE,
Blake PG, Martin JN Jr. The recurrence risk of the
syndrome of hemolysis, elevated liver enzymes, and low
platelets (HELLP) in subsequent gestations. Am J Obstet
Gynecol 1994;171:940-943.
16. Sibai BM, Taslimi MM, el-Nazer A, Amon E, Mabie BC,
Ryan GM. Maternal-perinatal outcome associated with
the syndrome of hemolysis, elevated liver enzymes, and
low platelets in severe preeclampsia-eclampsia. Am J
Obstet Gynecol 1986;155:501-509.
17. Munday DN, Jones WR. Pregnancy complicated by the
antiphospholipid syndrome. Aust N Z J Obstet Gynaecol
1993;33:255-258.
18. Caritis S, et al. Low-dose aspirin to prevent preeclampsia
in women at high risk. National Institute of Child Health
and Human Development Network of Maternal-Fetal
Medicine Units. N Engl J Med 1998; 338: 701-705.
19. Lopez-Jaramillo P, Delgado F, Jacome P, Teran E, Ruano
C, Rivera J. Calcium supplementation and the risk of
preeclampsia in Ecuadorian pregnant teenagers. Obstet
Gynecol 1997;90:162-167.
20. Bucher H, et al. Effect of calcium supplementation on
pregnancy-induced hypertension and preeclampsia: a
meta-analysis of randomized controlled trials. JAMA
1996; 275: 1113-1117.

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