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    Muna Tahlak, MD, FACOG Latifa Hospital

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    Nur Rahmat Wibowo
    Hellp Syndrome Hemolisis, elevated liver enzyme, and low platelet hdf hdehedrerheryher yewryery4reryreggjnmdihvdvbdsf qewgfewgfygfygdsyfgydsgfysgfuigawfygyusgdfyusgfyusgyufgsuygfyusgfyu guyfsgfy usgfuysgyuf

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    Muna Tahlak, MD, FACOG Latifa Hospital

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    Nur Rahmat Wibowo
    227 views44 pages
    Hellp Syndrome Hemolisis, elevated liver enzyme, and low platelet hdf hdehedrerheryher yewryery4reryreggjnmdihvdvbdsf qewgfewgfygfygdsyfgydsgfysgfuigawfygyusgdfyusgfyusgyufgsuygfyusgfyu guyfsgfy usgfuysgyuf

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    HELLP Syndrome

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    Hellp Syndrome Hemolisis, elevated liver enzyme, and low platelet hdf hdehedrerheryher yewryery4reryreggjnmdihvdvbdsf qewgfewgfygfygdsyfgydsgfysgfuigawfygyusgdfyusgfyusgyufgsuygfyusgfyu guyfsgfy usgfuysgyuf

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    227 views44 pages

    Muna Tahlak, MD, FACOG Latifa Hospital

    Uploaded by

    Nur Rahmat Wibowo
    Hellp Syndrome Hemolisis, elevated liver enzyme, and low platelet hdf hdehedrerheryher yewryery4reryreggjnmdihvdvbdsf qewgfewgfygfygdsyfgydsgfysgfuigawfygyusgdfyusgfyusgyufgsuygfyusgfyu guyfsgfy usgfuysgyuf

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    Muna Tahlak, MD, FACOG Latifa Hospital

    Objectives
    Update on the disease
    focus on diagnosis Complications

    timing and mode of delivery


    mortality and morbidity controversial aspects of corticosteroid use

    Latifa Hospital is a tertiary center on average 6000 deliveries per year >80% high risk obstetric care 465 pregnant women had hypertensive disorder (8%)

    Causes of maternal death in developed countries


    Other direct causes of deaths Hypertensive disorders Embolism 21.3 16.1 14.9

    Haemorrhage Abortion
    Ectopic pregnancy Unclassified deaths Sepsis/infection

    13.4 8.2
    4.9 4.8 2.1

    WHO data on maternal mortality

    Team Work

    HELLP Syndrome
    Weinstein regarded signs and symptoms to constitute

    an entity separate from severe preeclampsia and in 1982 named the condition HELLP H = Haemolysis EL = Elevated Liver enzymes LP = Low Platelets currently regarded as a variant of severe preeclampsia or a complication .

    The HELLP syndrome occurs in about 0.5 to 0.9% of

    all pregnancies and in 10 to 20% of cases with severe preeclampsia

    70% of the cases develops before delivery with a peak

    frequency between the 27th and 37th gestational weeks 10% occur before the 27th week 20% beyond the 37th gestational week

    HELLP syndrome usually develops within the first 48 hours in women who have had proteinuria and hypertension prior to delivery

    hypertension and proteinuria absent in 1020% of the cases

    Symptoms
    right upper abdominal quadrant
    epigastric pain nausea and vomiting

    3060% of women have headache


    20% visual symptoms partial HELLP syndrome fewer symptoms less complications

    Reported frequency of signs and symptoms of HELLP syndrome


    Sign/symptom Frequency, percent

    Proteinuria
    Hypertension Right upper quadrant/epigastrict pain Nausea, vomiting Headache Visual changes Jaundice

    86 to 100
    82 to 88 40 to 90 29 to 84 33 to 61 10 to 20 5

    Haemolysis, one of the major characteristics of the disorder, is due to a microangiopathic haemolytic anaemia

    Normal peripheral blood smear

    Microangiopathic smear

    H(hemolysis)
    high LDH concentration
    unconjugated bilirubin low or undetectable haptoglobin concentration is a

    more specific indicator. Low haptoglobin concentration (< 1 g/L < 0.4 g/L)

    Elevated Liver enzymes(EL)


    Elevation of liver enzymes may reflect the haemolytic

    process as well as liver involvement. Haemolysis contributes to the elevated levels of LDH enhanced asparate aminotransferase (AST) and alanine aminotransferase (ALAT) levels are mostly due to liver injury

    Low platelet(LP)
    Thrombocytopenia < 150109/L)
    caused by gestational thrombocytopenia (GT) (59%) immune thrombocytopenic purpura (ITP) (11%)

    preeclampsia (10%)
    HELLP syndrome (12%). PLTs < 100109/L are relatively rare in preeclampsia and

    gestational thrombocytopenia, frequent in ITP and obligatory in the HELLP syndrome (according to the Sibai definition)

    Diagnosis
    Many different criteria
    Biochemical markers

    Clinical
    Preeclampsia ELLP

    Diagnostic criteria
    two major definitions for diagnosing the HELLP

    syndrome

    Professor Baha Sibai

    Professor and Chairman of the Department of Obstetrics and Gynecology at the University of Cincinnati College of Medicine leading authority in the care and treatment of women with preeclampsia and eclampsia, has published more than 500 peer-reviewed articles

    Tennessee Classification System


    Platelets 100109/L
    AST 70 IU/L LDH 600 IU/L

    Sibai has proposed strict criteria for "true" or

    "complete" HELLP syndrome Intravascular haemolysis is diagnosed by abnormal peripheral blood smear, increased serum bilirubin ( 20.5 mol/L or 1.2 mg/100 mL) and elevated LDH levels (> 600 units/L (U/L)

    Mississippi classification Class 1


    Platelets 50109/L
    AST 70 IU/LAST LDH 600 IU/L

    Mississippi classification Class 2


    Platelets 100109/L

    50109/L AST or ALT 70 IU/L LDH 600 IU/L

    Mississippi classification Class 3


    Platelets 150109/L

    100109/L AST or ALT 40 IU/L LDH 600 IU/L

    Differential diagnosis
    viral hepatitis
    cholangitis and other acute disease ITP

    acute fatty liver of pregnancy (AFLP)


    haemolytic uremic syndrome (HUS) thrombotic thrombocytopenic purpura (TTP) systemic lupus erythematosus (SLE)

    Complications reported in the HELLP syndrome


    Maternal complications Eclampsia Abruptio placentae Occurrence (%) 49 920

    DIC Acute renal failure


    Severe ascites Cerebral oedema Pulmonary oedema

    556 736
    411 18 310

    Complications reported in the HELLP syndrome


    Maternal complications Subcapsular liver hematoma Liver rupture Occurrence (%) Between 0.9% and <2% >200 cases or about 1.8%

    Cerebral infarction Cerebral Haemorrhage


    Maternal death Wound hematoma/infection2 Retinal detachment

    Few case reports 1.540


    125 714 1

    Maternal Mortality
    Stroke Cardiac Arrest DIC ARDS Renal failure Sepsis Hepatic Rupture 45% 40% 39% 27% 27% 24% 20%

    Hypoxic encephalopathy 15% Contributing factors to deaths in 54 women with HELLP syndrome From Isler and co-authors,1999

    Complications reported in the HELLP syndrome


    Foetal/neonatal complications Perinatal death IUGR 7.434 3861

    Preterm delivery Neonatal thrombocytopenia


    RDS

    70 (15% < 28 gestational weeks) 1550


    5.740

    Management of pregnant women with HELLP syndrome


    Immediate delivery
    > 34 weeks' gestation or later Nonreassuring tests of fetal status

    Presence of severe maternal disease: multiorgan

    dysfunction, DIC, liver infarction or hemorrhage, renal failure, or abruptio placenta

    27 to 34 weeks of gestation
    Delivery within 48 hours evaluation

    stabilization
    steroid treatment for fetal lung maturity

    Steroid use
    no clear evidence of any effect of corticosteroids on

    substantive clinical outcomes. insufficient evidence for the routine use The use of corticosteroids only to increase rate of recovery in platelet count if considered clinically worthwhile.
    Cochrane Review of 11 trials comparing corticosteroids

    with placebo/no treatment

    before 24 weeks' gestation, termination of pregnancy

    should be strongly considered

    Method of Delivery
    Vaginal
    Cesarean section

    Anesthesia Choice
    According to ACOG
    Regional anesthesia is preferred for women with

    preeclapmsia and eclampsia General anesthesia carries more risk than regional

    Anesthesia Choice
    What platelet count is adequate for regional

    anesthesia? No absolute answer Platelet counts >100,000/ul are acceptable to most anesthesiologists Platelet counts in 50,000-100,000 range are potential candidates according to ACOG

    Risk of spinal or epidural hematoma


    Paralysis

    Frederick P. Zuspan, M.D. 19222009

    An internationally recognized authority in the field of maternal-fetal medicine An expert on preeclampsia In the 1960s, Zuspan pioneered the use intravenous magnesium sulfate to prevent convulsions in women with preeclampsia. His treatment protocol was adopted internationally and is still used to treat preeclampsia nearly 50 years later

    there's an empty plaque at Chicago's famous Lying-in

    Hospital waiting for the engraved name of the person who discoveres the cause.

    Summary
    HELLP syndrome is unique to pregnancy
    HELLP syndrome develops in approximately 1 of 1000

    pregnancies overall and 10 to 20 percent of pregnancies with severe preeclampsia/eclampsia Delivery and supportive management is cure Multidisciplinary approach Tertiary center

    Summary
    outcome for mothers with HELLP syndrome is

    generally good, but serious complications can occur Recommendations are against giving dexamethasone for treatment

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