HELLP Syndrome Powerpoint from the perspective of obgyn

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1. HELLP SYNDROME:
An obstetrician’s nightmare
By: Dr.Ankita Chauhan
M.S., FMAS
Diploma in Cosmetic Gynaecology

2. HELLP Syndrome
•One of the most severe forms of Preeclampsia & occurs in 4 to 12
% of preeclamptic patients, associated morbidity and mortality
upto 25%
•HELLP stands for: Hemolysis(abnormal smear), Elevated liver
enzymes(serum AST>70 U/L serum LDH >600U/L), and Low
platelets (<100000)
•Multisystem disease with epigastric pain which radiated through
the right upper quadrant and back.
•Clinically challenging because of vague initial symptoms-nausea,
vomiting, headache, malaise, or viral-like symptoms
•Often mistaken for the flu, acidity or gall bladder problems

3. •Syndrome -thrombocytopenia, hemolytic anemia and liver dysfunction
•Probably microvascularendothelial activation and cell injury.
•Defective placental vascular remodeling during weeks 16-22 of
pregnancy with the second wave of trophoblastic invasion into the
decidua results in inadequate placental perfusion.
•The hypoxic placenta then releases various placental factors such as
soluble vascular endothelial growth factor receptor-1 (sVEGFR-1), which
then binds vascular endothelial growth factor (VEGF) and placental
growth factor (PGF), causing endothelial cell and placental dysfunction by
preventing them from binding endothelial cell receptors.
Pathophysiology of HELLP syndrome

4. Pathophysiology of HELLP syndrome
•The etiology of HELLP syndrome is unclear but is thought to be a
systemic inflammatory disorder mediated by a complement
cascade.
•Activation of the coagulation cascade causes consumption of
platelets due to adhesion on to a damaged and activated
endothelium, in addition to microangiopathichemolysis caused by
shearing of erythrocytes as they traversethrough capillaries laden
with platelet-fibrin deposits.
•Multiorganmicrovascularinjury and hepatic necrosis causing liver
dysfunction contribute to the development of HELLP.

5. THEORIES OF CAUSATION
Higher rates of placental maternal vascular supply lesions and small
for gestational age fetus
•Associated with hypertension and/or proteinuria in only 80% of
patients and exhibits different cytokine activation.
•Another hypothesis proposes acute maternal immune rejection due
to immunocompetentmaternal cells coming into contact with a
genetically distinct fetus, altering the maternal -fetal immune
balance and causing endothelial dysfunction, platelet activation and
aggregation, and arterial hypertension.

6. OTHER THEORIES
•Inborn errors of fatty acid oxidative metabolism.
•Placental-instigated acute inflammatory condition targeting the
liver.
•Dysfunction in the complement system via excessive activation or
defective regulation for a given amount of endothelial injury has
been proposed to cause damage to hepatic vessels in HELLP.
•The true pathology remains a mystery.

7. Epidemiology of HELLP syndrome
•Prevalence is 0.1% -0.6% of all pregnancies and in 4-12% of patients with
preeclampsia.
•About 70% of cases occur in 27 to 38 weeks of gestation, or immediately
postpartum in 15 -30% of cases. < 10% prior to 27 weeks
•HELLP -more common with older maternal age groups, avg-25 years. (incontrast,
preeclampsia is most common in younger patients (mean age, 19 yrs)
•Most patients with HELLP syndrome stabilize within 24 -48 hours after delivery,
•The most protracted postpartum recovery time in patients with class 1 disease
•The mortality rate of women with HELLP syndrome is 0 to 24%, with a
perinatal death rate of up to 37%.

8. Subsequent risks-Risk in future
•The recurrence rate is 2-27% in subsequent pregnancies.
•Patients are at increased risk of preeclampsia, preterm
delivery, fetal growth restriction, and placental abruption in
future pregnancies.
•At increased risk of developing hypertension and
cardiovascular disease in future.

9. RISK FACTORS
•Maternal age of older than 34 or
less than 19 years
•Multiparity
•Hypertensive disease during
previous pregnancy
•Maternal hypothyroidism
•Family history of preeclampsia
•Multifetalpregnancy
•Genetic association for increased
predisposition for HELLP syndrome has
been noted.
•Gestational diabetes mellitus
•Chronic hypertension
•Inherited acquired Thrombophilia
•Maternal chronic kidney disease
•Autoimmune disease (SLE /APLA/
RA)
•Pregnancy with Assisted
Reproductive Techniques(OD or
Surrogacy)
•Excessive weight gain during
pregnancy, obesity

10. Staging / classification of HELLP
•Spectrum of presentation
Mild and Self limiting to Fulminant with MOF
•Two common classifications used to predict maternal
morbidity and mortality
•Mississippi and the Tennessee classifications.

11. Mississippi classification
The Mississippi classification divides HELLP syndrome into 3 classes based
on platelet count, AST/ALT levels & LDH levels.
Class -1 Severe Class-2 Moderate Class 3-Mild
PLATELETS <=50000/uL <= 50000-100000 uL <= 1 TO 1.5 lakh/uL
AST/ALT >= 70 IU/L >= 70 IU/L >= 40 IU/L
LDH >= 600 IU/L >= 600 IU/L >=600 IU/L
Bleeding incidence 13% 8% No increasedrisk

12. Tennessee Classification
This classification describes HELLP as either complete or partial
Complete HELLP syndrome
•Platelet count of 100,000/Ulor less
•AST or ALT levels of 70 IU/L or more
•LDH (or bilirubin) (with hemolysis as
evidenced on abnormal peripheral
smear) levels of 600 IU/L (20.2
mg/dL) or more
Partial HELLP Syndrome
•Severe preeclampsia plus one of the
following:
• Elevated liver enzyme levels,
thrombocytopenia, no hemolysis
• Mildly elevated liver enzyme levels,
no thrombocytopenia, no hemolysis
• Normal liver enzyme levels
thrombocytopenia, no hemolysis,
• Hemolysis, liver dysfunction, no
thrombocytopenia

13. Diagnosis of HELLP SYNDROME
•Nonspecific viral syndrome types of symptoms and Malaise
•Nausea, vomiting, and epigastric pain, right upper quadrant pain
headache -the most common symptoms-30-90%
•Sudden Oedema with secondary weight gain
•Dyspnoea (if pulmonary oedema present)
•Visual changes in 10%-20%,
•Jaundice in 5%.
•Preterm labour

14. Signs and Symptoms
•Proteinuria -in 86 -100%
•Uncontrolled Hypertension in 80%.
•15% patients do not present with
•either
•Nondependent edema -periorbital
•or upper-lower extremities 55%-
67%
•Right upper quadrant tenderness
65 -90%
•Oliguria
•Jaundice 5% , Fatigue or
weakness
•Tachycardia, Tachypnea
•Brisk tendon reflexes
•Altered consciousness
•Sudden onset of massive edema
•Lung examination -crepitationsif
Pulmonary edema

15. •When a pregnant patient in the third trimester of pregnancy
or immediate postpartum <7 days of birth presents with the
above symptoms of HELLP syndrome & new-onset
hypertension or proteinuria is noted, certain lab. Tests are
needed to establish the diagnosis.

16. HELLP ALERTS
•Patient can deteriorate rapidly after initial symptoms
•Early diagnosis-best prognosis
•Any vomiting in second half of pregnancy-high degree of
suspicion

17. Maternal morbidity/mortality
Maternal Mortality rate because of HELLP syndrome is 0 -24%, with a
perinatal mortality rate of 35 -37%.
•Class 1 or complete HELLP is associated with the highest incidence of
perinatal morbidity & mortality. (60% of deaths with class 1)
•Cerebral hemorrhage is the most common autopsy finding.
Morbidity includes:
•Disseminated intravascular coagulation (DIC : 15 -62%)
•Placental abruption (16%), Postpartum haemorrhage(12-40%)
•Acute renal failure (36 -50 %)
•Pulmonary edema (6%)
•Patients with HELLP syndrome have a 19% to 27% risk of recurrence in
subsequent pregnancies. Recurrent cases are less severe after two episodes

18. Maternal Complications of HELLP
•Hematologic: DIC, bleeding, hematoma
•Cardiac: Cardiac arrest, myocardial ischemia
•Pulmonary: Pulmonary edema, respiratory failure, pulmonary embolism,
adult respiratory distress syndrome (ARDS)
•CNS: Hemorrhage/stroke, cerebral edema, central venous thrombosis
seizures, retinal detachment
•Renal: Acute renal failure, chronic renal failure requiring dialysis
•Hepatic: Hepatic (usually subcapsular) hematoma with possible rupture,
ascites, nephrogenicdiabetes insipidus
•Infection
•Peripartumbleeding , Maternal death
•Eye: Retinal detachment

19. Neonatal Morbidity/mortality
Neonatal complications:
•Prematurity
•Intrauterine growth retardation (39%)
•Thrombocytopenia (1/3 rdof neonates born to patients with HELLP;
4% of these infants will have intraventricularhemorrhage.
•Respiratory distress syndrome
•Neonatal mortality 9% -24% mainly due to placental abruption,
intrauterine asphyxia, or prematurity.

20. Laboratory Studies
•CBC: Thrombocytopenia, anemia with possible reticulocytosis
•Peripheral smear: Schistocytes, helmet cells, and burr cells secondary to
microangiopathichemolytic anemia
•Liver function tests : Serum AST/ALT levels: bilirubin Elevated secondary to
liver dysfunction
•LDH: Elevated secondary to liver dysfunction or hemolysis
•Haptoglobin: Decreased secondary to hemolysis
•RFT: Elevated BUN/creatinine with acute renal failure
•Coagulation studies : Fibrinogen levels: Low secondary to increased
coagulation, normal prothrombin(PT), 50% may have prolonged activated
partial thromboplastintime (PTT D-dimer: Increased due to fibrinolysis/DIC

21. Cont:-
Tennessee classification is used to diagnose HELLP syndrome and requires the
presence of all three criteria.
Hemolysisconfirmed with at least 2 of the findings:
• Peripheral smear with schistocytes and burr cells
• Serum bilirubin >1.2 mg/dl
• Low serum haptoglobin(<25mg/dl) or LDH> two times the upper level of the
normal.
• Severe anemia with hemoglobin <8 to 10 g/dl depending on the pregnancy
stage, unrelated to blood loss

22. Cont:-
Elevated liver enzymes: AST or ALT > 2 times the upper level of normal:
Low platelets: < 100,000 cells/microL.
• Patients meeting a few of the above criteria are considered to have partial
HELLP syndrome & may progress to meet all criteria and hence should be
monitored carefully.
• HELLP syndrome mainly involves platelet activation without affecting
clotting factors, and hence patients may have normal PT, PTT, and fibrinogen
•DIC may co-exist if PT and PTT are prolonged or if fibrinogen is low.

23. Laboratory tests Possible results CAUSE Recovery to
baseline
Haptoglobin Hemolysis 24-30 hours
LDH Hemolysisor liver dysfunction3-5 days
AST/ALT Liver dysfunction 3-5 days
Bilirubin Hemolysis -
Platelets Consumption 6-11 days
Haemoglobin Hemolysis -
PT Normal -
APTT Liverdysfunction -
D-dimer Increasedcoagulation -
Fibrinogen Fibrinolysis -

24. Differential diagnosis
Elevated liver enzymes with pregnancy
SerumtransaminaseBilirubin Coagulopathy Otherfeatures
Acute Fatty Liver<500 >5 + Hepatic features,
HTN: coma ,renal
failure,
hypoglycemia
IHCP <300 <5 _ Pruritis, increased
bile acids
HELLP >500 <5 + HTN, edema,
thrombocytopenia
Infective Hepatitis>1000 (markedly
elevated)
variable + No HTN,
h/o fever and
vomiting

25. Diagnosis-Imaging studies
•USG abdomen-pelvis-liver: subcapsular hematoma, hepatomegaly
•Kidney: signs of renal causes of hypertension, other changes in renal
parenchyma
•Ascites and pleural effusion
•CT Scanning or MRI –If suspected hematoma-with worsening LFT
•Obstetric USG with Doppler
•Fundoscopy-differentiate chronic and new onset disease and to diagnose
papilledema/hemorrhages as these have ominous prognosis

26. Additional Special Imaging
•2D maternal ECHO: in special situations where at a rsk of developing
cardiovascular complications.
•Chest Xray with shield: ARDS, pulmonary edema or pulmonary
embolism is suspected
•MRI brain :for D/D of eclampsia, venous sinus thrombosis and
cerebrovascular accidents.
•Neuro imaging is not recommended universallyin all cases of
eclampsia

27. Predictors
•Rising BP in 2nd trimester
•Early onset PE
•Abnormal LFT
•Elevated uric Acid levels
•Increased inflammatory markers-CRP, IL-6

28. Management of HELLP
•PREVENTION-Close monitoring in high risk pregnancies
-Low dose asprin
-Manage Chr. Hypertension
-Early detection of PE

29. •Control of hypertension
• Prevent Convulsions
• Fluid and Electrolyte Balance
• Transfusion/ Steroids
• Fetal Surveillance
•Labor and Delivery
•Insertion of drains at time of LSCS

30. Management of HELLP syndrome
•Early and immediate recognition of the diagnosis.
•Pt should be referred to tertiary care centre.
•Stabilize the patient before transferring –
•Take I.V. line
•If seizures & HT are present, should be controlled en route to the hospital.
•Emergency department management includes seizure prophylaxis, hypertension
control, repletion of blood products, as indicated, and general stabilization of
patient condition.
•Intravenous fluids should be given cautiously. Patients with HELLP syndrome may
be volume overloaded and present with edema but are infact intravascularly
depleted

31. Shifting to an equipped facility
•Following measures should be taken
•Magnesium sulphate-Loading dose of Pritchard regimen must be given to
prevent maternal seizures and neuroprotectiveeffects on the fetus/neonate
This information must-be given _ to the referral centre
•Antihypertensivesin the form of tablet-labetolol200 mg orally or Nifedipine20
mg (slow release)
•Steroid Coverage
•Transfer with attendance and monitoring and information to the receiving center
along with the eclampsiakit
•Prompt delivery as it is the only effective treatment.

32. ROLE OF CORTECOSTEROIDS IN HELLP
•Both antepartum and postpartum management
•Reduces intravascular endothelial injury, prevent further hepatocyte death and
platelet activation
•Studies -improved laboratory findings and platelet counts, liver function and
urine output with the use of high -dose dexamethasone
•Intravenous superior to intramuscular steroids and are dose-dependent
•Aggressive therapy with high-dose dexamethasone
•Improve fetal morbidity-reduced incidence of RDS and intraventricular
hemorrhage, as well as maternal morbid

33. DELIVERY GENERAL GUIDELINES
•Antenatal Steroids may increase platelet count
• If at 34 weeks or later & unstable -deliver immediately
• If at 34 weeks or later & stable -consider administration of steroids; evaluate
over 24 -48 hours and deliver
• If at 24 -34 weeks and stable -consider administration of steroids; wait 24 -48
hours & evaluate for delivery based on the maternal -fetal condition
• If < 34 weeks with evidence of maternal or fetal distress, deliver immediately

34. Identification
•Laboratory
evaluation
•Imaging studies
(CT/USG)
•Fetal assessment
Emergent stabilization
•IV access and cautious
IV fluids
•Transfusion of blood or
blood products
Medications
•Antihypertensives
•Magnesium sulphate
•Corticosteroids
Admit to monitored
unit
Delivery
•>= 34 wksand
unstable, consider
steroids
•>= 34wks, stable:
steroids, wait 24-
48hrs and deliver
•<= 34 wks:
steroids as
tolerated and
expedite delivery
based on maternal
and fetal condition

35. Further management:
•Surgery, hematology and renal consults
•Repeat laboratory tests every 6 to 24 hrsto test for remission

36. TAKE HOME MESSAGE
•HELLP is a severe, potentially fatal condition
•Prevention, vigilant monitoring and Early recognition
improves the outcomes.
•DELIVERY IS THE ONLY CURE

37. THANK YOU