This document discusses HELLP syndrome, a variant of preeclampsia characterized by hemolysis, elevated liver enzymes, and low platelets. It provides details on the pathogenesis, diagnosis, classification, clinical presentation, differential diagnosis, and management of HELLP syndrome. Regarding management, the document outlines the three main options - immediate delivery at or after 34 weeks, delivery within 48 hours with stabilization and steroids from 27-34 weeks, and expectant management for less than 48-72 hours before 27 weeks with steroids. The role of steroids in improving maternal and neonatal outcomes is also discussed.

2.  The spectrum of disease resulting from
pathophysiology of pre-eclampsia
continues to challenge diagnostic
accuracy of clinicians.
 Out of pre- eclampsia’s various
manifestations, a specific entity is HELLP
syndrome.

3.  The acronym HELLP was coined by
Weinstein in 1982 to describe a
syndrome consisting of Hemolysis,
Elevated liver enzymes and Low
platelet count.

4.  It is a syndrome that is characterized by
hepatic endothelial disruption followed
by platelet activation, aggregation and
consumption, ultimately resulting in
ischemia and hepatocyte death.

5.  HELLP Syndrome - 0.2 to 0.6% of all
pregnancies.
 Pre Eclampsia – 5 to 7% of all
pregnancies.
 Sibai et al reporetd 20% incidence of
HELLP in women with pre eclampsia.
 70% cases diagnosed in antenatal period
while 30% after delivery.

6.  The findings of this multisystem disease are
attributed to-
› Abnormal vascular tone
› Vasospasm
› Coagulation defects
 This vasculopathy either limited to hepatic
segment or diffusely throughout liver.
 More commonly involves smaller terminal
arterioles yielding a characteristic histological
features

7.  Periportal or focal parenchymal necrosis in
which hyaline deposits of fibrin like material
↓
Obstruction of hepatic blood flow
↓
Periportal necrosis
Intra hepatic hemorrhage
Subcapsular hematoma
Eventual rupture of Glisson’s capsule

8.  Hemolysis is due to a microangiopathic haemolytic
anaemia (MAHA).
The term is now commonly described as thrombotic
thrombocytopenic purpura.
More recently the term thrombotic microangiopathy
is used to describe the syndrome charecterized by
› Hemolytic anemia
› RBC fragmentation
› Thrombocytopenia
› Thrombotic lesions in small blood vessels

9.  Red cell fragmentation caused by high-velocity
passage through damaged endothelium appears
to represent the extent of small vessel
involvement with intima damage, endothelial
dysfunction and fibrin deposition.
 Presence of fragmented (schizocytes) or
contracted red cells with spicula (Burr cells) in
the peripheral blood smear reflects the
haemolytic process and strongly suggests the
development of MAHA.

10.  Peripheral smear shows-
› Spherocytosis
› Schizocytes
› Reticulocytosis
› Anisocytosis
› Triangular cells
› Helmet cells
› Burr cells
› Polychromasia

11.  Destruction of red blood cells by haemolysis causes
increased serum lactate dehydrogenase (LDH) levels
and decreased haemoglobin concentrations.
 Haemoglobinaemia or haemoglobinuria is
macroscopically recognizable in about 10% of the
women.
 Liberated haemoglobin is converted to unconjugated
bilirubin in the spleen or may be bound in the plasma
by haptoglobin.
 The haemoglobin-haptoglobin complex is cleared
quickly by the liver, leading to low or undetectable
haptoglobin levels in the blood, even with moderate
haemolysis.

12.  Low haptoglobin concentration can be used
to diagnose haemolysis and is the preferred
marker of haemolysis.
 Thus, the diagnosis of haemolysis is supported
by high LDH concentration and the presence
of unconjugated bilirubin, but the
demonstration of low or undetectable
haptoglobin concentration is a more specific
indicator.

13.  Platelets
(PLTs) < 150·109/L) in
pregnancy may be caused by-
› Gestational thrombocytopenia (GT) (59%),
› Immune thrombocytopenic purpura (ITP)
(11%),
› Preeclampsia (10%),
› HELLP syndrome (12%)

14.  PLTs < 100·109/L are relatively rare in
preeclampsia and gestational
thrombocytopenia, frequent in ITP and
obligatory in the HELLP syndrome
(according to the Sibai definition).
 Decreased Platelet count in the HELLP
syndrome is due to their increased
consumption.
 Platelets are activated, and adhere to damaged
vascular endothelial cells, resulting in increased
platelet turnover with shorter lifespan.

15.  DIC is the primary process in HELLP
syndrome as suggested by some
investigators but most patients show
no abnormality on coagulation studies.
 Patient who develop DIC generally are
having well developed HELLP
syndrome.

16.  The diffuse systemic nature & aetiopathology of
HELLP syndrome explained by possibility that
Pre eclampsia intrinsically is an immunologically
mediated systemic disorder.
 Abnormal T & B lymphocyte function observed
in patient with HELLP Syndrome .
 There is an increased neutrophil- endothelial
adhesiveness in pre- eclamptic patients
↓
Explains diffuse vascular implication of disease
process

17. HELLP Syndrome Pre Eclampsia
Parity Multiparous Nulliparous
Age > 25yrs < 20yrs or > 45 yrs
Other relevant •White race •Family history of PIH
history •H/O Poor •Chronic hypertension
pregnancy •Diabetes mellitus
outcome •Multifetal gestation
•Less Antenatal visit

18. TENNESSEE CLASSIFICATION
Based on laboratory criteria
1. Platelet count < 100,000/µL
2. AST ≥ 70 IU/L & LDH ≥ 600 IU/L
3. Hemolysis on peripheral smear
Partial HELLP Full HELLP
Any 2 of 3 criteria All of 3 criteria

19. CLASS I
› Platelet ≤ 50,000/µL(severe thrombocytopenia)
› AST ≥ 70 IU/L
› LDH ≥ 600 IU/L
› Hemolysis on smear
CLASS II
› Platelet 50,000/µL to100,000/µL (moderate
thrombocytopenia)
› AST ≥ 70 IU/L
› LDH ≥ 600 IU/L
› Hemolysis on smear

20. CLASS III
› Platelet 100,000/µL to150,000/µL (mild
thrombocytopenia)
› AST ≥ 40 IU/L
› LDH ≥ 600 IU/L
› Hemolysis on smear

21.  Patients with FULL HELLP syndrome –
› Are at higher risk for complication like DIC.
› Should be considered delivery within 48 hrs.
 Patients with PARTIAL HELLP syndrome –
› Candidates for conservative management
 Patients with CLASS-I HELLP are at higher risk
for maternal morbidity and mortality than
CLASS-II & III.
 Class III HELLP syndrome is considered as a
clinical significant transition stage or a phase of
the HELLP syndrome which has the ability of
progression.

22. CLINICAL FEATURES
 The clinical presentation in most cases is
vague & may be missed completely if higher
degree of suspicion is not maintained.
 About 7% of cases presents before 27 weeks,
46% cases before 37 weeks and 14% presents
at term
 With postpartum presentation, the onset is
typically within first 48 hrs of delivery.

23.  Right sided upper abdominal pain or pain around
stomach 86-90%
 Nausea 45-85%
 Headache 50%
 Malaise 80-90%
Signs
 Right upper quadrant tenderness 86%
 Increased blood pressure 67%
 Protenuria 85-90%
 Edema 55-65%

24.  Young, White woman
 ≥25yrs
 Multiparous
 Heavy with severe generalized edema
 2nd or 3rd trimester
 c/o Rt. Upper quadrant pain since few days for which
she might be taking antacids.
 c/o malaise since few days, which may be out of
proportion to the discomfort expected by the stage of
pregnency
 Blood pressure is only slightly raised.
 Edema & proteinuria may or may not be present

25.  So, any pregnant woman presenting
in OPD with malaise or viral like
illness in 2nd or 3rd trimester should
be evaluated with CBC and Liver
function tests

26.  Thrombocytopenia occurs first followed by
raised liver enzymes and last is hemolysis.
 Laboratory criteria for diagnosis —
1. Low platelets - < 100,000/µL
2. Elevated liver enzymes – AST > 70 IU/L
- LDH > 600 IU/L
3. Hemolysis – Abnormal peripheral smear
- Total bilirubin > 1.2mg%

27.  Leukocytosis
 Coagulation factors
› If DIC is not present – PT , aPPT, S. Fibrinogen will be
normal
› If fibrinogen < 300 mg/dl along with other lab abnormality –
DIC is suspected
› Positive D-dimer test is more sensitive indicator of sub
clinical coagulopathy and may be positive before other
coagulation studies are abnormal.
› Proteinuria
 S. uric acid – raised
 Hypogycemia- persistent, profound hypoglycemia
inspite of repeated glucose transfusion is peculiar to
advanced HELLP syndrome.

28. 1. Diseases related to pregnancy
› Benign thrombocytopenia of pregnancy
› Acute fatty liver of pregnancy (AFLP)
2. Infectious and inflammatory diseases, not
specifically related to pregnancy:
› Virus hepatitis
› Cholangitis
› Cholecystitis
› Upper urinary tract infection
› Gastritis
› Gastric ulcer
› Acute pancreatitis

29. 3. Thrombocytopenia
› Immunologic thrombocytopenia (ITP)
› Folate deficiency
› Systemic lupus erythematosus (SLE)
› Antiphospholipid syndrome (APS)
4. Rare diseases that may mimic HELLP
syndrome
› Thrombotic thrombocytopenic purpura (TTP)
› Haemolytic uremic syndrome (HUS)

30. Maternal complications
 Eclampsia
 Abruptio placentae
 DIC
 Acute renal failure
 Severe ascites
 Cerebral oedema
 Pulmonary oedema
 Wound hematoma/infection

31.  Subcapsular liver hematoma
 Liver rupture
 Hepatic infarction
 Recurrent thrombosis
 Retinal detachment
 Cerebral infarction
 Cerebral Haemorrhage
 Maternal death

32.  Perinatal death
 IUGR
 Preterm delivery
 Neonatal thrombocytopenia
 RDS

33.  Spontaneous rupture of a Subcapsular liver haematoma in
pregnancy is a rare, but life threatening complication.
 Occurs 1 in 40,000 to 1 in 250,000
deliveries and about 1% to < 2% of
the cases with the HELLP syndrome.
 Rupture most often occurs in the
right liver lobe.
 The symptoms are sudden-onset
severe pain in the epigastric and
right upper abdominal quadrant radiating
to the back, right shoulder pain, anaemia and hypotension.
 The condition may be diagnosed by ultrasound, CT or
magnetic resonance imaging (MRI) examination.
 Hepatic rupture may also occur in the post-partum period.

34. Identification - clinical features
- lab findings
- D/D from other condition
Admission to hospital Stabilization
•IV line ,Cross match
•Catheterization
•Respi assessment
Transport to tertiary care
centre or latency for 24- Fetal assessment
48 hrs (NST,BPP,Color doppler )
Termination of
Conservative approach for 48-72
pregnancy
hrs (<32wks POG, Partial
HELLP,Tertiary health cenre)
Rebound / Resolution Monitor by lab Ix
Stop MgSO4 24 hrs of delivery
Continue antihypertensive & steroid

35.  In general, there are three major options for the
management of women with severe preeclampsia and
HELLP syndrome
 These include:
1) Immediate delivery which is the primary choice at 34
weeks' gestation or later.
2) Delivery within 48 hours after evaluation, stabilization
of the maternal clinical condition and Steroid treatment.
At 27 to 34 weeks of gestation, this option appears
appropriate and rational for the majority of cases.
3) Expectant (conservative) management for more than
48–72 hours may be considered in pregnant women
before 27 weeks' gestation. In this situation, Steroid
treatment is often used, but the regimens vary
considerably.

36. <32 wks 32-34wks >34wks
↓ ↓ ↓
Admit & conservative Mx Steroids Deliver
↓ ↓ No
Manage Pt based on Is pt eligible for conservative Mx ?
Clinical response during ↓ Yes
Period of observation Counsel pt abt benefit of continuing of
pregnency for ≥2 wks for lung maturity
↓
Worsens Stable Transfer pt to tertiary care centre
↓ ↓
Deliver Monitor pt in tertiary care centre

38.  Whereas delivery is the mainstay of treatment
for the HELLP syndrome, Steroid treatment is a
possible addendum. Present alternatives for
Steroid treatment are:
1) Standard steroid treatment on maternal HELLP.
2) High-dose dexamethasone treatment of the
mother.
3) Treatment with repeated doses to reduce
maternal morbidity and hastening recovery.

39.  Benefit of steroid treatment for the HELLP
syndrome was first reported in 1984.
 Mech. Of Action- Unknown
 Proposed mech - diminish oedema, inhibit
endothelial activation and reduce endothelial
dysfunction
↓
 Prevention of thrombotic
microangiopathic anaemia,
 Inhibition of cytokine production
↓
induce anti-inflammatory
effects in the HELLP syndrome

40.  Benefit from steroid treatment of the HELLP
syndrome was reported in a publication from
1993 where less frequent grade III and IV IVH,
necrotizing enterocolitis (NEC), retrolental
fibroplasia and fewer neonatal deaths were
observed.
 In addition to accelerate foetal lung maturity,
antenatal steroid has been used to reduce the
risk of IVH and NEC in selected cases of the
HELLP syndrome.

41.  IV steroid- appear to have more rapid onset
of action than IM better outcome in
improving urine output & laboratory values.
 Dose –increases platelet count when given in
high doses
 Duration –Duration of action of this
medication is limited.
Patient may experience a worsening of their
laboratory studies 48-72 hrs after dosing with
steroid .– REBOUND PHENOMENON

42.  For most of patient with HELLP syndrome-
10mg IV dexamethasone every 12 hrs until delivery
& then
10 mg IV dexamethasone every 12 hrs for additional 3
doses post partum.
 For selected high risk cases with profound
thrombocytopenia with CNS dysfunction.
20mg IV dexamethasone every 6hrs up to 4 doses

43.  In first largest randomized double blind, placebo
controlled (dexamethasone versus placebo)
study of 132 women by Fonseca et al. reported
shorter mean hospitalization but no significant
differences were found in recovery of platelet
counts or liver enzymes.
 A Cochrane analysis from 2004 concluded that
steroid treatment did not affect maternal
mortality and outcomes such as placental
abruption, pulmonary oedema and liver
complications.

44. Steroid is not curative but may
create a WINDOW OF
OPPORTUNITY for
intervention before maternal
condition may again deteriorate.

45.  Platelet transfusion – is required eithr before or
after delivery, in presence of bleeding from
puncture site, wound and intra peritoneal
bleeding.
If platelet count <40,000/µL, 6 – 10 U of platelet is
required.
 PCV and FFP – required if coagulopathy is
present.

46.  Antithrombin III transfusion- correct
hypercoagulability, stimulate prostacyclin
production, regulate thrombin-induced
vasoconstriction, improve foetal status.
 In contrast to the use of heparin, antithrombin
has not been shown to increase the risk of
bleeding.
 The potential benefit from antithrombin
treatment of women with HELLP syndrome
might be a reasonable objective to be tested in
future well designed multicenter studies.

47.  In most women with a HELLP syndrome, the
maternal PLT counts continue to decrease
immediately post-partum with an increasing trend
on the third day.
 About 30% of the HELLP syndromes develop after
birth.
 The time of onset ranged from few hrs to 7 days;
the majority within the first 48 hoursafter delivery.
 In post-partum HELLP syndrome, risk of renal
failure and pulmonary oedema is significantly
increased.

48.  Since early post-partum administration of high-dose
CS might accelerate recovery , its routine
administration is highly advocated (10 mg of
dexamethasone every 12 hours)
 However, a randomized study showed that
adjunctive use of intravenous dexamethasone for
postpartum patients with severe preeclampsia did
not reduce disease severity or duration.
 There was no difference in maternal morbidity,
duration of hospital stay, need for rescue scheme or
the use of blood products between the groups.
 These findings did not support the use of
dexamethasone in the puerperium for recovery of
women with HELLP .

49.  Women with a HELLP syndrome who
demonstrate progressive elevation of bilirubin
or creatinine for more than 72 hours after
delivery may benefit from plasma exchange
with fresh frozen plasma.
 In the case of continuing haemolysis, persistent
thrombocytopenia and hypoproteinaemia,
post-partum erythrocyte and thrombocyte
substitution, as well as albumin
supplementation, are standard treatment
regimens

50.  Sibai has shown that oral
contraceptives are safe in
women with a prior HELLP
syndrome.
 Women with a history of the
HELLP syndrome carry an
increased risk of at least 20%
(range 5–52%) that some form
of gestational hypertension will
recur in a subsequent gestation

51.  Women with a history of HELLP
syndrome at or before 28 weeks'
gestation during the index pregnancy are
at increased risk for several obstetric
complications (preterm birth, pregnancy-
induced hypertension and increased
neonatal mortality) in a subsequent
pregnancy.

52.  HELLP Syndrome and its management
still poses a problem in modern
obstetrics
 Precise diagnosis and early treatment
with non-mineral corticosteroides such
as Dexamethasone may help achieve
favorable maternal and perinatal results.

53. THANK YOU