HELLP Syndrome

Physiology

  • Similar to TTP/ HUS
  • Probably results from vasospasm leading to microangiopathic hemolysis and endothelial cell damage, with subsequent platelet consumption and fibrin deposition
  • Primary Organ of Involvement: liver
    • Sucapsular hematomas can occur -> hepatic necrosis

Diagnosis

  • Intravascular Hemolysis
    -Normal or increased retic count
    -Elevated LDH
    -Indirect hyperbilirubinemia
    -Decreased haptoglobin
    -Urine Hb-pos (only when hemolysis is severe)
    -Urine bili-neg
    -Urine hemosiderin-pos (only when hemolysis is chronic)
    -Urine urobilinogen-variable

Smear: polychromatophilia/ RBC fragments (schistocytes), helmet cells/nucleated RBC

CBC: thrombocytopenia

LFT’s: elevated

BM Bx: erythroid hyperplasia


Clinical

  • Definition: HELLP = Hemolysis + Elevated Liver Functions + Low Platelets
  • Timing of Onset: usually occurs in late pregnancy/early post-partum period (30% of cases occur in first 24-48 hrs post-partum)
  • Associated with pre-eclampsia/eclampsia in 3-12% of cases

  • Comparison of HELLP, HUS, TTP, DIC, and Acute Fatty Liver of Pregnancy (ALFP) (see Hemolytic-Thrombocytopenic Syndromes)
    -HELLP syndrome must be distinguished from other causes of MAHA, including TTP, HUS, and DIC). Other causes of liver disease in pregnancy include acute fatty liver of pregnancy (AFLP), which often presents with fulminant hepatic failure including hypoglycemia, markedly elevated bilirubin levels, and coagulopathy. Hemolysis is not a common feature in AFLP.

  • Multisystem Involvement:
    1) GI:
    a) RUQ Pain:
    b) Elevated Liver Enzymes: TBili >1.2 mg/dL, LDH >600 IU/L, aspartate aminotransferase >70 IU/L

2) Heme:
a) Microangiopathic Hemolytic Anemia (MAHA): schistocytes on smear due to intravascular hemolysis
b) Thrombocytopenia: plt count <100k/mm3

3) Neuro:
a)

4) Other:
a) Malaise:
b) Non-Dependent Edema:
c) HTN: may be absent early in course
d)


Treatment

  • Conservative Management: best choice in cases with immature fetus
    -However, there is an increased risk of catastrophic placental abruption, renal failure, and/or hepatic rupture

Immediate Delivery: best choice in cases with a viable fetus

Surigcal Management: indicated for shock, severe RUQ pain, and acute abdomen (as these indicate probable rupture of a subcapsular hepatic hematoma)


Prognosis

  • May recur in future pregnancies

References

  • Barton JR, Sibai BM. Care of the pregnancy complicated by HELLP syndrome. Gastroenterol Clin North Am 1992; 219:937-950
  • Fish R. The HELLP syndrome: case report and review of the literature. J Emerg Med 1993; 11:169-174
  • Geary M. The HELLP syndrome. Br J Obstet Gynaecol 1997; 104: 887-891
  • Reubinoff BE, Schenker JG. HELLP syndrome–a syndrome of hemolysis, elevated liver enzymes and low platelet count–complicating preeclampsia-eclampsia. Int J Gynecol Obstet 1991; 36:95-102
  • Sibai BM, Ramadan MK, Usta I, et al. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets. Am J Ostet Gynecol 1993; 1691000-1006