Transcript HELLP SYNDROME- A THERAPEUTIC CHALLENGE

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)
H
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 liver´s Glisson´s 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.
HELLP SYNDROME: Risk Factors for maternal
morbidity.
LABORATORY
Platelets< 50.000
CLÍNICAL
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
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 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 1,54g/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.
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 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 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!