HELLP Syndrome A Therapeutic Challenge Dr.Z.Naderi Iran University of Medical Siences

HELLP HELLP SyndromeSyndrome

A Therapeutic A Therapeutic ChallengeChallenge

Dr.Z.NaderiDr.Z.NaderiIran University of Iran University of Medical SiencesMedical Siences

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.

WEINSTEINWEINSTEIN(1982)

HH HEMOLYSIS

ELEL ELEVATED LIVER ENZYMES

LPLP LOW PLATELETS

HELLPHELLP

•The reported incidence 0.1-0.8%

•Approximately 10 to 20 percent of patients with

preeclampsia develop superimposed HELLP syndrome.

•Elevated perinatal morbidity and

mortality.

•Maternal Mortality 35%.

Risk Factors Previous history of preeclampsia or

HELLP

Variety of genetic variants

Nulliparity is not a risk factor for HELLP Syndrom .

ERITHROCYTIC MORPHOLOGY

PLATELET DISORDERS

RENAL COMPROMISE

HEPATIC DISORDERS

IMMUNOLOGIC DISORDERS

GENETIC DISORDERS

Factors to considerFactors to consider :

HELLP SHELLP SYYNDROME NDROME : POSIBLE : POSIBLE PATHOPHYSIOLOGYPATHOPHYSIOLOGY

 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

• Third TRIMESTRE

(28-36 W )

• FIRST DAYS (48 h)POSTPARTUM 30%

• Antepartum diagnosis is Antepartum diagnosis is made in 70% between 27 and made in 70% between 27 and

37 weeks of gestation.37 weeks of gestation.

HELLP Syndrom

HELLP Syndrom

Only 20 % of postpartum patients with HELLP had evidence of preeclampsia antepartum .

Criteria for establishing the

diagnosis of the HELLP Syndrome

HemolysisAbnormal peripherical blood smear (reveal spherocytes, schistocytes, triangular cells and burr cells )Elevated Bilirubin >1.2 mg/dl

Elevated liver enzymesSGOT >70 UI / LLDH >600 UI / L

Low PlateletsPlatelet Count < 100 × 103 /mm3

We can also observe

Excessive body weight increase .

Ophthalmic disorders -Minor filterations -Cortical blindness (amaurosis)-Retinal detachment-Vitreous hemorrhage.

We can also observe

Alternation in biomarkersAlternation 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

40-90% percent with epigastric pain

30-85% percent with nausea and vomiting

30-60% percent with headache,visual changes ,jaundice 5%,ascites .

Clinical presentation

BP>140/90 mmhg and proteinuria are present in approximateiy 85 % of cases .

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

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.

Differential Differential Diagnosis of the Diagnosis of the

HELLP Syndrome 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

Differential Diagnosis of the HELLP Syndrome

*PRIMARY RENAL DISEASE Glomerulonefritis

*OTHERSHepatic encephalopathiesViral hepatitisHyperemesis Gravidarum Idiopathic Thrombocytopenia Renal calculi Peptic ulcerPielonephritisApendicitisDiabetes Mellitus

MANAGEMENT OF THE MANAGEMENT OF THE

HELLP SYNDROMEHELLP SYNDROME

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.

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.

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.

Choice of hypotensive medication

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.

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

Preventing Convulsions MgSOMgSO44: 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 PhenytoinPhenytoin.

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

GA 30-32 w with unfavorable cervix

cesarean Delivery

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.

(1)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.

Postpartume course

Decreasing PLT continue until 24-48 h

Serum LDH usually peaked 24-48 h

Maternal outcome

Abruptio placentae 16% Acute renal failure 8% Pulmonary edema 6% Subcapsular hematoma 1% Retinal detachment 1%

Signs of multiple organ failure.

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

Be on the lookout for:

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.

Hepatic Rupture

Epigastric pain, shoulder pain ,nausea ,vometing Swelling of abdomen ,hemoperitoneum ,shock Aminotransferases = 4000-5000 IU/L

CT and MRI is more dependable than ultrasonography

Hepatic Rupture

Management :

volume replacement and blood transfusion

stability in the size of hematoma and normal lab tests

out patient follow up

Hepatic Rupture

Advising on future pregnancies.

The risk of recurrence of preeclampsia -eclampsia is 20% and for the HELLP syndrome: 2-6%

The risk of recurrence of preterm delivery is high, about 70%.1

Advising on future pregnancies

Maternal lab parameters don’t predict fetal mortality .

Maternal HELLP doesn’t affect fetal/neonatal liver function .

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.

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