HELLP syndrome


DEFINITION

PHYSICAL FINDINGS

  • Definitive laboratory criteria remain to be validated prospectively.
  •  Most commonly used criteria include hemolysis defined by:
    • 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; Platelet count < 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.

DDx

  • 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:
    • CBC to evaluate platelets
    • CMP
    • Urinalysis
    • 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.

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
  • 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

CHRONIC Rx

  • In pregnancies of 34 wk or with class 1 HELLP syndrom = delivery, either vaginal or abdominal, within 24 hr is the goal.
  • In the preterm fetus (< 34 wk) 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.

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 predictative of a major hemorrhage are thrombocytopenia (>100,000/mm3), AST > 70 IU/L, and previous gestations.