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HELLP Syndrome

BASIC INFORMATION

DEFINITION
The HELLP syndrome is a serious variant of preeclampsia. HELLP is an acronym for h emolysis,
elevated l iver function, and l ow platelet count.
It is the most frequently encountered microangiopathy of pregnancy. There are three classes of
the syndrome based on the degree of maternal
thrombocytopenia as a primary indicator of
disease severity:
Class 1:
Platelets 50,000/mm3
Class 2:
Platelets >50,000/mm3 to
100,000/mm3
Class 3:
Platelets >100,000/mm3
ICD-9CM CODES
642.50HELLP, episode of care
642.51HELLP, delivered
642.52HELLP, delivered with postpartum
complications
642.53HELLP, antepartum complications
642.54HELLP, postpartum complications
ICD-10CM CODES
O14.2HELLP syndrome

EPIDEMIOLOGY &
DEMOGRAPHICS


Among women with severe preeclampsia,
6% will manifest with one abnormality suggestive of HELLP syndrome, 12% will develop
two abnormalities, and approximately 10%
will develop all three.
The HELLP syndrome, like preeclampsia, is
rare before 20 wk of gestation.
One third of all cases occur postpartum; of
these, only 80% are typically diagnosed with
preeclampsia before delivery.
RISK FACTORS: Women >35 yr, white, multiparity
RECURRENCE RATE: 3% to 25%
PHYSICAL FINDINGS & CLINICAL
PRESENTATION


Definitive laboratory criteria remain to be
validated prospectively.
Most commonly used criteria include hemolysis defined by the presence of an abnormal
peripheral smear with schistocytes, lactate
dehydrogenase (LDH) >600 U/L, and total
bilirubin >1.2 mg/dl; elevated liver enzymes
as serum aspartate aminotransferase (AST)
>70 U/L and LDH >600 U/L; low platelet
count as less than 100,000/mm3.
Although many women with HELLP syndrome
are asymptomatic, 80% report right upper
quadrant pain and 50% to 60% present with
excessive weight gain and worsening edema.

ETIOLOGY
As with other microangiopathies, endothelial
dysfunction, with resultant activation of the
intravascular coagulation cascade, has been
proposed as the central pathogenesis of HELLP
syndrome.

Magnesium sulfate is administered for seizure prophylaxis regardless of blood pressure.




Blood pressure control is achieved with
agents such as hydralazine or labetalol.
Indwelling Foley catheter to monitor maternal
volume status and urine output.

Dx DIAGNOSIS

CHRONIC Rx

In pregnancies of 34 wk or with class 1
HELLP syndrome, delivery, either vaginal or
abdominal, within 24 hr is the goal.
In the preterm fetus corticosteroid therapy to
enhance fetal lung maturation is indicated.
Some reports have shown temporary amelioration of HELLP severity with the administration of high-dose steroids measured
by increased urine output, improvement in
platelet count, and liver function test.
Judicious use of blood products, especially in
those requiring surgery.
The patient requires intensive observation for
48 hr postpartum; laboratory levels should
begin to improve during this time.

DIFFERENTIAL DIAGNOSIS
Appendicitis
Gallbladder disease
Peptic ulcer disease
Enteritis
Hepatitis
Pyelonephritis
Systemic lupus erythematosus


Thrombotic thrombocytopenic purpura/
hemolytic-uremic syndrome
Acute fatty liver of pregnancy
WORKUP
Because HELLP syndrome is a disease entity
based on laboratory values, initial assessment
is detailed below.
LABORATORY TESTS
Initial assessment of suspected HELLP syndrome should include a complete blood
count to evaluate platelets, urinalysis, serum
creatinine, LDH, uric acid, indirect and total
bilirubin levels, and AST/alanine aminotransferase (ALT).
Tests of prothrombin time, partial thromboplastin time, fibrinogen, and fibrin split products are reserved for women with a platelet
count well below 100,000/mm3.
IMAGING STUDIES
No imaging modalities aid in diagnosis.

Rx TREATMENT
Treatment depends on gestational age of the
fetus, severity of condition, and maternal status.
Stabilization of the mother is the first priority.

ACUTE GENERAL Rx
Assess gestational age thoroughly. Fetal status should be monitored with nonstress tests,
contraction stress tests, and/or biophysical
profile.
Maternal status should be evaluated by history, physical examination, and laboratory
testing.

DISPOSITION
The natural history of this disorder is a rapidly
deteriorating condition requiring close monitoring of maternal and fetal well-being.
REFERRAL
Preterm patients with HELLP syndrome should
be stabilized hemodynamically and transferred
to a tertiary care center. Term patients can be
treated at a local hospital depending on the
availability of obstetric, neonatal, and blood
banking services.

PEARLS &
CONSIDERATIONS

Not all women with HELLP have hypertension


or proteinuria.
Life-threatening hemorrhage is a rare event
in HELLP syndrome. Identifiable risk factors
predictive of a major hemorrhage are thrombocytopenia (<100,000/mm3), AST >70 IU/L,
and previous gestations.

SUGGESTED READINGS
available at www.expertconsult.com
RELATED CONTENT
Eclampsia (Related Key Topic)
Preeclampsia (Related Key Topic)
AUTHORS: SONYA S. ABDEL-RAZEQ, M.D., and
RUBEN ALVERO, M.D.

HELLP Syndrome
SUGGESTED READINGS
Kulungowski AM etal: Hemolysis, elevated liver enzymes, and low platelets syndrome: when is surgical help needed? Am J Surg 198:916-920, 2009.
Norwitz ER etal: Acute complications of preeclampsia, Clin Obstet Gynecol
45(2):308, 2002.

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