HELLP Syndrome:Updates on Management

Report
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
21.3
Hypertensive disorders
16.1
Embolism
14.9
Haemorrhage
13.4
Abortion
8.2
Ectopic pregnancy
4.9
Unclassified deaths
4.8
Sepsis/infection
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 10–20% of the cases
Symptoms
 right upper abdominal quadrant
 epigastric pain
 nausea and vomiting
 30–60% 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
86 to 100
Hypertension
82 to 88
Right upper quadrant/epigastrict pain
40 to 90
Nausea, vomiting
29 to 84
Headache
33 to 61
Visual changes
10 to 20
Jaundice
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 < 150·109/L)
 caused by gestational thrombocytopenia (GT) (59%)
 immune thrombocytopenic purpura (ITP) (11%)
 preeclampsia (10%)
 HELLP syndrome (12%).
 PLTs < 100·109/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 ≤ 100·109/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 μmol/L or ≥ 1.2 mg/100 mL) and elevated LDH
levels (> 600 units/L (U/L)
Mississippi classification
Class 1
 Platelets ≤ 50·109/L
 AST ≥ 70 IU/LAST
 LDH ≥ 600 IU/L
Mississippi classification
Class 2
 Platelets ≤ 100·109/L
≥ 50·109/L
 AST or ALT ≥ 70 IU/L
 LDH ≥ 600 IU/L
Mississippi classification
Class 3
 Platelets ≤ 150·109/L
≥ 100·109/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
Occurrence (%)
Eclampsia
4–9
Abruptio placentae
9–20
DIC
5–56
Acute renal failure
7–36
Severe ascites
4–11
Cerebral oedema
1–8
Pulmonary oedema
3–10
Complications reported in the
HELLP syndrome
Maternal complications
Occurrence (%)
Subcapsular liver hematoma
Between 0.9% and <2%
Liver rupture
>200 cases or about 1.8%
Cerebral infarction
Few case reports
Cerebral Haemorrhage
1.5–40
Maternal death
1–25
Wound hematoma/infection2
7–14
Retinal detachment
1
Maternal Mortality
Stroke
45%
Cardiac Arrest
40%
DIC
39%
ARDS
27%
Renal failure
27%
Sepsis
24%
Hepatic Rupture
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
7.4–34
IUGR
38–61
Preterm delivery
70 (15% < 28 gestational weeks)
Neonatal thrombocytopenia
15–50
RDS
5.7–40
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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