The HELLP

Syndrome–
A Therapeutic
Challenge
JOHN ESSIEN M.D.
JESSICA BARDALES MITAC M.D.
J.M RODRÍGUEZ FERNÁNDEZ M.D.
EMILIO ORTEGA CALLAVA M.D.
HOSPITAL GINECOBSTÉTRICO PROVINCIAL
CAMAGÜEY.
Pre-eclampsia - Is a
multisystemic, idiopathic disorder
specific to the pregnancy and
puerperium of the human species. It
is characterized by the clinical triad
of:
•Hypertension
•Proteinuria
•Edema
Literature dating from the XIXth
century report:
• Very unusual varieties of severe pre-
eclampsia with complicated progress.
• These unusual descriptions of pre-
eclampsia are recognised today as the
HELLP Syndrome.

Today:
• HELLP Syndrome is considered to be an
association of characteristic hepatic and
hematologic disorders.
WEINSTEIN(1982)
WEINSTEIN HELLP

H HEMOLYSIS

EL ELEVATED LIVER ENZYMES

LP LOW PLATELETS
•The reported incidence 2 a 12 %.

•Elevated perinatal morbidity and

mortality.

•Maternal Mortality 35%.

 HELLP SYNDROME : POSIBLE
PATHOPHYSIOLOGY
 CAUSAL AGENTES : Increase in volume., Fetal
presence / decidual cell?, Vasospasm?, Deficiente
vascular repair?, Idiopathic?
 
Vasculo-endothelial Disorder
 
Platelet Agregation/Consumption
 
Fibrin Activation/Consumption
 
Selective organic Isquemia/nsuficiency
 
Variable Manifestations
 Other Factors to consider :
 ERITHROCYTIC MORPHOLOGY

 PLATELET DISORDERS

 RENAL COMPROMISE

 HEPATIC DISORDERS

 IMMUNOLOGIC DISORDERS

 GENETIC DISORDERS
The Causal Factors induce:

 Thrombocytopenia

 Microangiopathic Hemolytic
Anemia

 Periportal necrosis and distension

of the liver´s Glisson´s capsule.
 DIAGNOSIS
• MID-II TRIMESTRE

• FIRST DAYS POSTPARTUM

• Antepartum diagnosis is
made in 70% between 27
and 37 weeks of gestation.
 Criteria for establishing the
diagnosis of the HELLP Syndrome

 Hemolysis
Abnormal peripherical blood smear
Elevated Bilirubin >1.2 mg/dl

 Elevated liver enzymes

SGOT >72 UI / L
LDH >600 UI / L

 Low Platelets
Platelet Count < 100 × 103 /mm3
 We can also observe:
 Excessive body weight increase .
 Pulse pressure amplification.
 Systole pressure > 140 mmHg,
but diastole pressure < 90 mmHg.
 Ophthalmic disorders
-Minor alterations
-Cortical blindness (amaurosis)
-Retinal detachment
-Vitreous hemorrhage.
 We can also observe:

 Elevation of Biomarkers:
-HCG
-Maternal alfa-fetal protein
-LDH
-Serum Haptoglobin
 The presence of these
disorders in an
hypertensive woman
with epigastric and/or
right hypochondrial
pain, nausea, vomiting;
as well as hemolysis,
will help in making the
right diagnosis.
Clasification of the HELLP Syndrome
based on the platelet count
(MISSISSIPPI)1.

 Class 1 – Platelet count <50

000/mm3.

 Class 2 - Platelet count between

50 000 y 100 000/mm3.

 Class 3 - Platelet count <between

100 000 y 150 000/mm3.
 Hemolysis + Liver disfunction

*LDH ≥ 600 UI/l

*ASAT (SGOT) and/or ALAT (SGPT)≥ 40

UI/l

*ALL HAVE TO PRESENT

Magann E.F., Martín J.N. – Twelve Steps to Optimal Management of HELLP
1

Syndrome. Clinical Obstetrics and Gynecology. Lippincott Williams & Wilkins,

Philadelphia, 1999. Vol. 42 No. 3: 532-50.
 Another classification based on the
partial or complete expression of the
HELLP Syndrome(MEMPHIS)1.
 Complete HELLP –
*Microangiopathic hemolytic anemia
in women with severe pre-
eclampsia  
*LDH ≥ 600 UI / L
*SGOT ≥ 70 UI/l
* Thrombocytopenia < 100 000/mm3
 PARTIAL HELLP–
One or two of the above.
Differential Diagnosis of the HELLP Syndrome

THROMBOTIC MICROANGIOPATHIES
-Thrombotic thrombocytopenic purpura
- Microangiopathic hemolytic anemia
induced by sepsis or drugs
- Hemolytic Uremic Syndrome
FIBRINOGEN CONSUMPTION
DISORDERS– CID
-Acute fatty liver
-Sepsis
- Severa Hypovolemia / Hemorrhage
(Abruptio/Amniotic fluid embolism)
CONNECTIVO TISSUE DISORDERS
-Systemic Lupus Erithematosus
Differential Diagnosis of the HELLP
Syndrome
*PRIMARY RENAL DISEASE
Glomerulonefritis
*OTHERS
Hepatic encephalopathies
Viral hepatitis
Hyperemesis Gravidarum
Idiopathic Thrombocytopenia

Renal calculi
Peptic ulcer
Pielonephritis
Apendicitis
Diabetes Mellitus
HELLP SYNDROME: Risk Factors for maternal
morbidity.

LABORATORY CLÍNICAL
Platelets< 50.000 Epigastric pain

LDH >1400 UI/L Nauseas

CPK > 200 UI/L Vomitng

ALT > 100 UI/L Eclampsia

AST > 150 UI/L Severe hypertension

Creatinine > 1.0 Abruptio Placentae

MANAGEMENT OF THE HELLP

SYNDROME
1. ANTICIPATE THE DIAGNOSIS
2. EVALUATE THE MATERNAL CONDITION
3. EVALUATE THE FETAL CONDITION
4. CONTROL THE HYPERTENSION
5. PROFILAXIS OF CONVULSIONES WITH MgSO4
6. WATER AND ELECTROLITIC BALANCE
7. HEMOTHERAPY
8. MANAGEMENT OF LABOR AND DELIVERY
9. OPTIMIZE PERINATAL CARE
10.INTENSIVE POSTPARTUM TREATMENT OF THE
PATIENT
11.BE ALERT FOR MULTIPLE ORGAN FAILURE
12.ADVISE ON FUTURE PREGNANCY
 The Maternal Condition can be
evaluated by:
 Complete Hemogram. If
platelets<150.000/mm3 requieres
more study.
 Liver Enzymes. The elevation of
the transaminases and LDH is a sign
of hepatic disfunction.
 Renal function. Deficencies in
renal function are observed in late
stages of the illness. Creatinine and
Uric acid levels are variable.
 Bilirubin. Unconjugated bilirubin is
increased due to the hemolysis but
rarely above 1-2 mg%.
 Serial evaluation laboratory
parameters every 12 to 24 hours or
more if necessary.
 Differential diagnosis with othere
pathologies.
 Evaluating the Fetal
Condition
 Determine the gestational age.

 Evaluate fetal well-being: Non-stress test,

Tolerance to contracction test and/or
biophysical profile.

 Use corticosteroids between 24 and 34

weeks to improve fetal pulmonary
maturity/neonatal pulmonary function as
well as maternal and perinatal results.
 Controlling the
hypertension
 80-85% of patients with HELLP
need control of their BP to avoid
significant maternal and perinatal
morbidity and mortality.
 Treat systolic BP when>150mmHg
and avoid placental hypoperfusion
maintaining the diastolic BP not
less than 80-90 mmHg.
 Choice of hypotensive
medication
 Hydralazine: Bolus of 5-10 mg IV every
20-40 min. If uneffective or unavailable,
use labetalol, nifedipine o sodium
nitroprussiate.
 Labetalol: Initial bolus of 20 mg IV, with
increases in dosage until a satisfactory BP
is obtained or up to maximum dose of 300
mg.
 Nifedipina oral(not sublingual) at usual
dosage.
 Sodium Nitroprussiate is a fast
acting hypotensive agent(venous and
arterial) which can be used in an
hypertinsive crisis when all other
hypotensive drugs have failed
Loading dose: 0,25 μg/kg/min,
increasing upto 10 μg/kg/min.
Above this dose there is a greater
risk of cyanide intoxication of the
fetus. When using, remember it’s
photosensitivty and sever rebound
effect.
 Preventing Convulsions
 MgSO4: Initial bolus of 4-6g IV,
followed by a continous infusion at 1,5-
4g/h, individualized according to the
patient. Continue 48 horas o more
postpartum until clinical and laboratory
signs of improvement are obtained.

 If contraindications of MgSO4 exist, use

Phenytoin.
Phenytoin Loading dose: 15 mg/kg at
40 mg/min with continous monitorization
of the cardiac function and BP every 5
minutes. The therapeutic range is 10-20
μg/ml.
 Water and Electrolytic Management
 Objectives:
-diuresis of 30-40ml/h.
-Limit intake of liquids to 150ml/h.
-Balance of electrolytes.

REMEMBER

NEGATIVE BALANCE=vasoconstriction.
EXCESIVE POSITIVE BALANCE= pulmonary
damage
 Monitorization of volume through
pulmonary capilar wedge pressure
 Hemotherapy
The base of hemotherapy in
patients with HELLP is the
transfusion of platelets.

 The usual dose is one unit per every

10 kg of corporal weight.
 Spontaneous bleeding occurs in
most cases with a platelet count of
<50.000/mm3.
 Hemotherapy

 To avoid postpartum hemorrhage

in a transvaginal delivery the
indication for platelet transfusion is
a count <40.000/mm3.

 In the immediate postpartum

periodo : Maintain the count
>50.000/mm3 abdominal deliveries
and >20.000/ mm3 in transvaginal
deliveries.
 Hemotherapy
 The aggresive use of
Dexamethasone in patients with
HELLP and severe
thrombocytopenia has eliminated
virtually all need for platelet
transfusion.

 Other therapeutic alternatives:

-Plasmaphersis
-Immunoglobulins
 Management of labor and
delivery
When considering termination of
gestation in a patient with HELLP,
determine:
 Gestational age.
 Maternal and fetal conditions.
 Fetal presentation.
 Cervical maturity
 Management of labor and
delivery

If transabdominal delivery is
requiered, perform:
 Vertical skin incision.
 Corporeal incision of the uterus
(due to scarse development of
the inferior segment and
abnormal presentationes).
 Spontaneous delivery of the
placenta to avoid hemorrhage
 Optimizing perinatal care.

 The main risk for the fetus in

pregnancies with HELLP is it´s
prematurity.
 The use of corticosteroids
decreases the morbidity associated
with pulmonary immaturity in
preterm babies.
 Delivery should be in a center with
capability of treating these children
with a major risk of
cardiopulmonary instability.
 Postpartum Intensive Care.
 Admision in an obstetrical intensive care
unit until:
(1) Sustained increase in the platelet count and a
maintained decrease in LDH.
(2) Diuresis >100ml/h for 2 consecutive hours
without duiretics.
(3) Well controled BP with systolic pressure 
150 mmHg and diastolic pressure < 100
mmHg.
(4) Obvious clinical improvement and absence of
complications.
 The absence of improvement of the
thrombocytopenia within 72-96 hours
postpartum indicates severe compromise of
compensatory mechanisms and possibel
MULTIPLE ORGAN FAILURE.
Postpartum Intensive Care - The use
of Dexamethasone

 ANTEPARTUM: (0,15mg/kg)10mg IV bid

- when Platelets <100.000/mm3
- if Platelets 100.000-50.000/mm3 AND
Eclampsia, Severe Hypertension,
Epigastric Pain
 POSTPARTUM: 10mg IV bid for 2 dosis, then
5mg bid for 2 additional doses:
- when steroids were used in antepartum
- suspend when there is clinical and
laboratory improvement (platelets
>100.000mm3, decreased LDH, diuresis
>100 ml/h)
 Be on the lookout for:

 Signs of multiple organ failure.

 Complications:
- Subcapsular Hematoma
- Subcapsular hepatica hemorrhage
- Hepatic Rupture.

 Therapeutic solutions:
- Conservative Procedures
- Surgery.
 Advising on future
pregnancies.
 The risk of
recurrence of
preeclampsia
-eclampsia is 42-
43% and for the
HELLP syndrome:
19-27%.
 The risk of
recurrence of
preterm delivery is
high, about 61%.1
 Conclusions
 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.
THANK YOU!