Hellp syndrome– a therapeutic Challenge

HELLP Syndrome– A Therapeutic Challenge

ERITHROCYTIC MORPHOLOGY

• ERITHROCYTIC MORPHOLOGY

• PLATELET DISORDERS

• RENAL COMPROMISE

• HEPATIC DISORDERS

• IMMUNOLOGIC DISORDERS

• GENETIC DISORDERS

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

• 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

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

• *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 .

• Bilirubin .

• Unconjugated bilirubin is increased due to the hemolysis but rarely above 1-2 mg%.

• 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.

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.

• 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 1,5-4g/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.

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:

• Sustained increase in the platelet count and a maintained decrease in LDH.

• Diuresis >100ml/h for 2 consecutive hours without duiretics.

(3) Well controled BP with systolic pressure 150 mmHg and diastolic pressure < 100 mmHg.

• (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.

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