The HELLP The HELLP Syndrome– Syndrome–

A Therapeutic A Therapeutic ChallengeChallengeJOHN ESSIEN M.D.JOHN ESSIEN M.D.

JESSICA BARDALES MITAC M.D.JESSICA BARDALES MITAC M.D.J.M RODRÍGUEZ FERNÁNDEZ J.M RODRÍGUEZ FERNÁNDEZ

M.D.M.D. EMILIO ORTEGA CALLAVA EMILIO ORTEGA CALLAVA

M.D.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.

WEINSTEINWEINSTEIN(1982)

HH HEMOLYSIS

ELEL ELEVATED LIVER ENZYMES

LPLP LOW PLATELETS

HELLPHELLP

•The reported incidence 2 a 12 %.

•Elevated perinatal morbidity and

mortality.

•Maternal Mortality 35%.

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

ERITHROCYTIC MORPHOLOGY

PLATELET DISORDERS

RENAL COMPROMISE

HEPATIC DISORDERS

IMMUNOLOGIC DISORDERS

GENETIC DISORDERS

Other Factors to considerOther Factors to consider :

The Causal Factors induce:

Thrombocytopenia

Microangiopathic Hemolytic Anemia

Periportal necrosis and distension of the liver´s Glisson´s capsule.

DIAGNOSISDIAGNOSIS• MID-II TRIMESTRE

• FIRST DAYS POSTPARTUM

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

and 37 weeks of gestation.and 37 weeks of gestation.

Criteria for establishing the

diagnosis of the HELLP Syndrome

HemolysisAbnormal peripherical blood smear Elevated Bilirubin >1.2 mg/dl

Elevated liver enzymesSGOT >72 UI / LLDH >600 UI / L

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

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.

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 encephalopathiesViral hepatitis

Hyperemesis Gravidarum Idiopathic Thrombocytopenia

Renal calculi Peptic ulcer

PielonephritisApendicitis

Diabetes Mellitus

Differential Diagnosis of the HELLP Syndrome

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 MANAGEMENT OF THE HELLP

SYNDROMESYNDROME

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.

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.

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. 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: Sustained increase in the platelet count and

a maintained decrease in LDH. Diuresis >100ml/h for 2 consecutive hours

without duiretics. Well controled BP with systolic pressure

150 mmHg and diastolic pressure < 100 mmHg.

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)

Signs of multiple organ failure.

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

Therapeutic solutions: - Conservative Procedures- Surgery.

Be on the lookout for:

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

ConclusionsHELLP 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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