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HELLP SYNDROME

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A-MOWAFY 2013

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

HELLP Syndrome sm during pregnancy

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A-MOWAFY 2013

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

+ Postpartum Care

Terminate

Class I (severe)

Class II , Class III (mild to moderate)

Completed 36 weeks

Pregnancy > 36 weeks Pregnancy < 34 weeks

Conserve

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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. Termination of pregnancy

In the following conditions:

a. Class I (severe cases) irrespective of gestational age

b. Pregnancy > 36 weeks

c. Completing conservative management

III. 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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HELLP SYNDROME

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A-MOWAFY 2013

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