The HELLP Syndrome

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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.

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• 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

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• 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.

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HELLP
WEINSTEIN(1982)
H
HEMOLYSIS
EL ELEVATED LIVER
ENZYMES
LP LOW
PLATELETS
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• The reported incidence 2 a 12 .
• Elevated perinatal morbidity and mortality.
• Maternal Mortality 35.

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

• ERITHROCYTIC MORPHOLOGY
• PLATELET DISORDERS
• RENAL COMPROMISE
• HEPATIC DISORDERS
• IMMUNOLOGIC DISORDERS
• GENETIC DISORDERS

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The Causal Factors induce

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

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DIAGNOSIS

• MID-II TRIMESTRE
• FIRST DAYS POSTPARTUM
• Antepartum diagnosis is made in 70 between 27
  and 37 weeks of gestation.

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Criteria for establishing the diagnosis of the
HELLP Syndrome

• HemolysisAbnormal peripherical blood smear
  Elevated Bilirubin gt1.2 mg/dl
• Elevated liver enzymesSGOT gt72 UI / LLDH gt600
  UI / L
• Low PlateletsPlatelet Count lt 100 103 /mm3

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We can also observe

• Excessive body weight increase .
• Pulse pressure amplification.
• Systole pressure gt 140 mmHg, but diastole
  pressure lt 90 mmHg.
• Ophthalmic disorders
• -Minor alterations
• -Cortical blindness (amaurosis)
• -Retinal detachment
• -Vitreous hemorrhage.

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We can also observe

• Elevation of Biomarkers
• -HCG
• -Maternal alfa-fetal protein
• -LDH
• -Serum Haptoglobin

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• 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.

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Clasification of the HELLP Syndrome based on the
platelet count (MISSISSIPPI)1.

• Class 1 Platelet count lt50 000/mm3.
• Class 2 - Platelet count between 50 000 y 100
  000/mm3.
• Class 3 - Platelet count ltbetween 100 000 y 150
  000/mm3.

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• 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.

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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 lt 100 000/mm3
• PARTIAL HELLP One or two of the above.

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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
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PRIMARY RENAL DISEASE Glomerulonefritis
OTHERSHepatic encephalopathiesViral
hepatitisHyperemesis Gravidarum Idiopathic
Thrombocytopenia Renal calculi Peptic
ulcerPielonephritisApendicitisDiabetes
Mellitus
Differential Diagnosis of the HELLP Syndrome
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MANAGEMENT OF THE HELLP SYNDROME
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1. ANTICIPATE THE DIAGNOSIS2. EVALUATE THE
MATERNAL CONDITION3. EVALUATE THE FETAL
CONDITION 4. CONTROL THE HYPERTENSION5. PROFILAX
IS OF CONVULSIONES WITH MgSO46. WATER AND
ELECTROLITIC BALANCE 7. HEMOTHERAPY8. MANAGEMENT
OF LABOR AND DELIVERY9. OPTIMIZE PERINATAL
CARE10.INTENSIVE POSTPARTUM TREATMENT OF THE
PATIENT 11.BE ALERT FOR MULTIPLE ORGAN
FAILURE12.ADVISE ON FUTURE PREGNANCY
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The Maternal Condition can be evaluated by

• Complete Hemogram. If plateletslt150.000/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.

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• 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.

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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.

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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 whengt150mmHg and avoid
  placental hypoperfusion maintaining the diastolic
  BP not less than 80-90 mmHg.

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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.

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• 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
  its photosensitivty and sever rebound effect.

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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. 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.

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Water and Electrolytic Management

• Objectives
• -diuresis of 30-40ml/h.
• -Limit intake of liquids to 150ml/h.
• -Balance of electrolytes.
• REMEMBER
• NEGATIVE BALANCEvasoconstriction.
• EXCESIVE POSITIVE BALANCE pulmonary damage
• Monitorization of volume through pulmonary
  capilar wedge pressure

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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 lt50.000/mm3.

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Hemotherapy

• To avoid postpartum hemorrhage in a transvaginal
  delivery the indication for platelet transfusion
  is a count lt40.000/mm3.
• In the immediate postpartum periodo Maintain
  the count gt50.000/mm3 abdominal deliveries and
  gt20.000/ mm3 in transvaginal deliveries.

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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

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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

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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

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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.

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Postpartum Intensive Care.

• Admision in an obstetrical intensive care unit
  until
• Sustained increase in the platelet count and a
  maintained decrease in LDH.
• Diuresis gt100ml/h for 2 consecutive hours
  without duiretics.
• Well controled BP with systolic pressure ? 150
  mmHg and diastolic pressure lt 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.

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Postpartum Intensive Care - The use of
Dexamethasone

• ANTEPARTUM (0,15mg/kg)10mg IV bid
• - when Platelets lt100.000/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 gt100.000mm3, decreased
  LDH, diuresis gt100 ml/h)

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Be on the lookout for

• Signs of multiple organ failure.
• Complications
• Subcapsular Hematoma
• Subcapsular hepatica hemorrhage
• Hepatic Rupture.
• Therapeutic solutions
• Conservative Procedures
• Surgery.

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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

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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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THANK YOU!