HELLP

Syndrome
A Therapeutic
Challenge
Layali Jodeh
Razan Malhees
th
5 year mdical
students

Pre-eclampsia
multisystemic, idiopathic disorder specific
to the pregnancy and puerperium of the
human species. It is characterized by the
presence of ;
Hypertension
Proteinuria

Literature dating from the XIXth

century report:
Very unusual varieties of severe preeclampsia with complicated progress.

These unusual descriptions of preeclampsia 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

HEMOLYSIS

EL

ELEVATED LIVER ENZYMES

LP

LOW PLATELETS

The reported incidence

0.2-0.6%

Approximately 4 to 12 percent of patients with

preeclampsia develop superimposed HELLP syndrome.

Elevated

perinatal

morbidity

mortality.
Maternal Mortality

35%.

and

Factors to consider :
ERITHROCYTIC MORPHOLOGY
PLATELET DISORDERS
RENAL COMPROMISE
HEPATIC DISORDERS
IMMUNOLOGIC DISORDERS
GENETIC DISORDERS

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

The Causal Factors induce:

Thrombocytopenia
Microangiopathic Hemolytic Anemia
Periportal necrosis and distension of
the livers Glissons capsule.

DIAGNOSIS
Third TRIMESTRE
FIRST DAYS POSTPARTUM
31%
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 (reveal

spherocytes, schistocytes, triangular cells

and burr cells )
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 .
Ophthalmic disorders
-Minor alterations
-Cortical blindness (amaurosis)
-Retinal detachment
-Vitreous hemorrhage.

We can also observe

Alternation in biomarkers
Increase in ;
-Maternal alfa-fetal protein
-LDH
Decrease in ;
-Serum Haptoglobin
-Hematocrit

Clinical Presentation
Approximately 90 percent of
patients present with
generalized malaise
65 percent with epigastric
pain
30 percent with nausea and
vomiting
31 percent with headache.

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.

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.

MANAGEMENT OF THE HELLP

SYNDROME

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

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
%.
Differential
pathologies.

diagnosis

with

othere

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 2040 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
g/kg/min,
increasing
upto
10
g/kg/min. Above this dose there is a
greater risk of cyanide intoxication
of the fetus. When using, remember its
photosensitivty and sever rebound effect.

Preventing Convulsions
MgSO4: Initial bolus of 4-6g IV,
followed by a continous infusion at 1,54g/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

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
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
timing of delivery
if > 34 weeks gestation, deliver
if < 34 weeks gestation,

administer corticosteroids, then

deliver in 48 hours

Optimizing perinatal care.

The main risk for the fetus in
pregnancies with HELLP is its
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 bsence

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.

Be on the lookout for:

Signs of multiple organ failure.

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

Hepatic Rupture
The incidence of hepatic rupture varies from one
in 40,000 to one in 250,000 pregnancies .
Hepatic infarction is even more rare and
commonly involves the right lobe.
It is believed to be a continuum of preeclampsia,
in which areas of coalescing hemorrhage result in
thinning of the capsule and intraperitoneal
hemorrhage.

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!