HELLP Syndrome:Updates on Management
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Transcript HELLP Syndrome:Updates on Management
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