hellp syndrome - medical sciences | pri · entire liver leads to hellp syndrome . classical...
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HELLP Syndrome Dr.Sreevani
PG
OBG Dept
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Introduction
Definition
Pathogenesis
Signs & symptoms
Diagnosis
Complications
Management
Summary
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Introduction
The acronym HELLP was coined by Weinstein in 1982 to describe a syndrome consisting of
Hemolysis,
Elevated Liver enzymes and
Low Platelet count.
It is a variant of severe pre-eclampsia or a complication of it.
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Definition
It is a syndrome that is characterised by
preeclampsia,
hepatic endothelial disruption,
platelet activation, aggregation and consumption,
resulting in microangiopathic hemolysis, ischemia and hepatocyte death.
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Incidence
0.5 to 0.9% of all pregnancies
10 to 20% of cases with severe preeclampsia.
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Pathogenesis
Pathogenesis of preeclampsia-
◦ Endothelial dysruption
◦ Abnormal vascular tone
◦ Vasospasm
◦ Coagulation defects
Involves smaller terminal arterioles
This vasculopathy if involves single segment or entire liver leads to HELLP syndrome
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Classical histolgical lesion in Liver
Periportal or focal parenchymal necrosis with deposits of hyalin like material
Intra hepatic haemorrhage
Subcapsular haematoma
Eventual rupture of Glisson’s capsule
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Hemolysis
It is due to thrombotic microangiopathy
Endothelial dysfunction
Intimal damage, foam cell, hyaline & fibrin deposition
Vessel wall narrowing
Fragmentation of red cells
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Peripheral smear shows
Schizocytes, burr cells, hemet cells, etc
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Haemolysis cont…
Increase in serum LDH & decrease in Hb concentrations
Haemoglobinemia & haemoglobinuria
Unconjugated bilirubinaemia
Haptoglobin levels – low or undetectable(more specific indicator)
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Thrombocytopenia
Platelet count < 150,000/cmm
Due to increased consumption
DIC is the primary process in HELLP syndrome
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Immune system disorder theory
Abnormal humoral as well as cell mediated immune dysfunction is also observed in patients with HELLP syndrome
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Risk factors
Multiparity
Age >25 yrs
White race
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CLASSIFICATION
Tennessee Classification System
Based on laboratory criteria
1. Platelet count < 100,000/cmm
2. AST >70 IU/L & LDH > 600 IU/L
3. Hemolysis on peripheral smear
Partial HELLP Full HELLP
Any 2 of 3 criteria All of 3 criteria
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Mississippi classification
Class1 Class 2 Class3
Platelet count(cmm)
<50,000 50,000 - 100,000
>100,000
AST > 70 IU/L > 70 IU/L
>40 IU/L
LDH >600 IU/L >600 IU/L
>600 IU/L
Hemolysis on smear
present present present
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Diagnosis
Clinical features
◦ 70% of the cases develop between the 27th and 37th gestational weeks
◦ 20% beyond the 37th gestational week
◦ 10% occur before the 27th week
◦ With postpartum presentation, onset is typically within first 48 hrs of delivery
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Symptoms
Right sided upper abdominal or epigastric pain (86-90%)
Nausea (45-85%)
Headache (50%)
Malaise (80-90%)
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Signs
Protenuria (85-90%)
Right upper quadrant tenderness (86%)
Increased blood pressure (67%)
Edema (55-65%)
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Laboratory findings
Low platelets <100,000/cmm
Elevated liver enzymes – AST >70 IU/L
Hemolysis – abnormal peripheral smear
Total bilirubin >1.2 mg%
PT, aPTT, S. Fibrinogen - if abnormal, DIC is suspected
S. uric acid is raised
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Differential diagnosis
Diseases related to pregnancy
◦ Benign thrombocytopenia of pregnancy
◦ Acute fatty liver of pregnancy
Infectious and inflammatory diseases, not specifically related to pregnancy
◦ Viral hepatitis
◦ Cholangitis
◦ Cholecystitis
◦ Gastritis,gastric ulcer
◦ Acute pancreatitis
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Complications
Maternal
Subcapsular liver hematoma & liver rupture
◦ DIC
◦ Acute renal failure
◦ Cerebral edema
◦ Pulmonary edema
◦ Wound hematoma/infections
◦ Retinal detachment
◦ Cerebral infarction & haemorrhage
◦ Maternal death
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Fetal/neonatal complications
Perinatal death
IUGR
Preterm delivery
Neonatal thrombcytopenia
RDS
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Management
Admission to hospital
Stabilization
Evaluation
Secure IV line
Transfusion of Blood and blood products
Catheterization
Respiratory assessment
Fetal assessment(NST, BPP, colour doppler)
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Immediate delivery:
◦ > 34 weeks' gestation or later
◦Nonreassuring fetal status
◦ Presence of severe maternal disease: multiorgan dysfunction, DIC, liver infarction or hemorrhage, renal failure.
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27 to 34 weeks of gestation
◦ Deliver within 48 hrs after stabilization and evaluation
◦ Steroid treatment for fetal lung maturity
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Before 27 weeks
◦Termination of pregnancy should be strongly considered.
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Summary
HELLP syndrome is unique to pregnancy
0.5 to 0.9% of all pregnancies
Delivery and supportive management is cure
Multidisciplinary approach
Tertiary care
Outcome is generally good if intervened early
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