HELLP syndrome

1. HELLP SYNDROME
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HELLP Syndrome
sm during pregnancy
DEFINITION:
The term HELLP syndrome is used to describe preeclampsia in association with Hemolytic
anemia, Elevated Liver enzyme levels, and Low Platelet count. The diagnosis is not always
clear, and the syndrome may be confused with other medical conditions. Any patient
diagnosed with HELLP syndrome should be considered to have severe preeclampsia.
INCIDENCE:
 0.5-0.9% of all pregnancies
 10-20% of women with severe preeclampsia and 30% of cases associated with eclampsia
 HELLP usually occurs in Caucasian women over the age of 25 in association with hypertensive
disorders with pregnancy
CRITERIA FOR DIAGNOSIS
I. Hemolytic anemia “H”
 Schizocytosis ; fragmentation of RBCS
 Bilirubin > 1.2 mg/dl
II. Elevated liver enzymes “EL”
 SGOT > 72 IU/L
 LDH > 500 IU/L
III. Low plattlet count “LP”
 Plattlet count < 100.000 mm3
CLASSES “GRADES”
 Class I : “severe”
 Plattlets<50.000 mm3
 Altered liver enzymes
 Evidences of hemolysis
 Class II : “moderate”
 Plattlets 50.000 – 100.000 mm3
 Class III : “mild”
 Plattlets 100.000 – 150.000 mm3
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2. HELLP SYNDROME
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DIFFERENTIAL DIAGNOSIS
HELLP syndrome may be easily confused with many other medical conditions, particularly
when the patient is normotensive, differential diagnosis include:
1. Biliary colic and cholecystitis
2. ITP
3. GERD and peptic ulcer
4. Acute fatty liver of pregnancy
5. Appendicitis
6. Cerebral hemorrhage
7. Diabetes insipidus
8. Gastroenteritis
9. Glomerulonephritis
10. Hemolytic uremic syndrome
11. Hyperemesis gravidarum
12. Pancreatitis
13. Pyelonephritis
14. Systemic lupus erythematosus
15. Thrombophilias
16. Viral hepatitis
MANAGEMENT
Early diagnosis
Class II , Class III
Class I
(mild to moderate)
(severe)
Pregnancy > 36 weeks
Terminate
Pregnancy < 34 weeks
Completed 36 weeks
Conserve
+ Postpartum Care
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3. HELLP SYNDROME
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I. Early Diagnosis
 All cases with abnormal high blood pressure ± proteinuria should have liver enzymes
and plattlet , however; HELLP syndrome may develop in the absence of signs
 High risk patients include:
a. Elderly multipara
b. Wide pulse pressure eg; 160/90 mm/Hg
c. Visual symptoms ; blurring of vision ….. etc
d. Warning symptoms ; headache, epigastric and right upper quadrant pain … etc
e. Mild mid-trimestric elevation of serum α feto-protein
 Laboratory investigations suggesting early HELLP syndrome in high risk patients:
a. LDH> 6000 IU/L
b. AST > 150 IU/L
c. ALT > 100 IU/L
d. Bilirubin > 1.2 mg/dL
e. Plattlets < 150.000 mm3
f. Uric acid > 8 mg/dL
II.
III.
Termination of pregnancy
In the following conditions:
a. Class I (severe cases) irrespective of gestational age
b. Pregnancy > 36 weeks
c. Completing conservative management
Conservative management :
Indicated in mild to moderate cases < 34 weeks, conservative management includes:
a. Control of blood pressure; as PET
b. Prevention of eclamptic fits;
 Giving magnesium sulfate to all cases of HELLPsyndrome
 Action : - prevent progression of HELLP syndrome – decreases the effect on
Plattlets and RBCs
c. Corticosteroid therapy;
 Dose: dexamethasone 10 mg tablets/twice daily
 Action: - enhance lung maturity – improve Plattlets and liver functions
 N.B: withdrawal should be gradual to avoid postpartum rebound effect on Plattlets
and liver enzymes
d. Nitric oxide donation; improves the manifestation of HELLP syndrome
e. Fluid therapy (fluid and electrolyte balance)
 (glucose+saline+10% ringers lactate) 100 mL/hour
 Monitoring: by the following:
o Fluid chart
o Serum electrolyte daily
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4. HELLP SYNDROME
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o CVP; to maintain pressure between 8-12 cm/H2O and to avoid volume
overload
o N.B: - too little fluids → increases vasospasm → renal injury
- too much fluids → pulmonary edema
f. Plattlets transfusion;
 When Plattlets < 50.000 m3 and patient is going to do CS
Or Plattlets < 20.000 m3 and patient will deliver vaginally
 Each unit increases plattlet count by 10.000 m3 So 6 – 10 units are very effective
 Aggressive corticosteroid therapy decreases the need for plattlet transfusion
g. Packed RBCs when haematocrite value < 30%
h. Plasmapheresis:
 Life-saving procedure if deterioration continue inspite of all above measure
 Fresh frozen plasma is used in plasma exchange
 Action: remove debris of RBCs hemolysis and Plattlets
 Aggressive corticosteroid therapy decreases the need for plasmapheresis
IV.
Postpartum care :
HELLP syndrome may be first discovered postpartum. Once discovered treatment must be
in obstetric intensive care unit till:
1. Blood pressure well-contolled (dialstolic< 100 mm/Hg)
2. Urine output > 100 mL/hour
3. Maternal Plattlets increases and LDH decreases
4. Clinical improvement of any complications
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