Sunday, July 5, 2009

Preeclampsia and HELLP Syndrome (HELLP Syndrome)

HELLP Syndrome

The etiology and pathogenesis of preeclampsia and HELLP syndrome remain unclear. Some theories center on the explanation that abnormal placentation results in placental ischemia and the production of a circulating toxin that causes endothelial cell injury.[5] The injury is believed to cause vascular constriction within multiple organ systems, activation of the coagulation system, increased capillary permeability, and platelet activation with platelet consumption in the microvasculature, all resulting in hypertension, proteinuria, edema, and thrombocytopenia. Why certain women with severe preeclampsia develop HELLP syndrome is unclear, but it has been postulated that these women may have more endothelial injury with greater activation of the coagulation system. Immunologic factors have also been proposed as the underlying initiator of preeclampsia and HELLP syndrome. Maternal cell-mediated immune response to pregnancy with cytokine-mediated endothelial damage may be an important factor.

HELLP syndrome occurs across all ethnicities, races, socioeconomic classes, and age ranges. It is seen in both primigravidas and multiparous patients. Presenting symptoms include epigastric or right upper quadrant pain (65%), nausea and vomiting (50%), malaise (90%), and nonspecific viral syndrome-like symptoms; some patients may present with hematuria or gastrointestinal bleeding.[3] Hypertension and proteinuria may be absent or only slightly abnormal. The differential diagnosis is vast and includes acute fatty liver of pregnancy, appendicitis, gallbladder disease, gastroenteritis, hemolytic uremic syndrome, hepatic encephalopathy, systemic lupus erythematosus, thrombotic thrombocytopenic purpura, and viral hepatitis.

HELLP Syndrome

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HELLP Syndrome Diagnosis (HELLP Syndrome)

Hellp Syndrome

There is also considerable debate regarding the definition, diagnosis, incidence, etiology, and management of HELLP syndrome. It is a multisystem disease that is characterized by microangiopathic hemolytic anemia, hepatic dysfunction, and thrombocytopenia. Sibai estimated it to affect 2% to 20% of patients labeled with severe preeclampsia and 10% of women with eclampsia. It is often insidious in onset and not accompanied by the usual signs of preeclampsia, hypertension, and proteinuria, and therefore is easy to miss in its earlier, milder form. Criteria to establish the diagnosis of HELLP syndrome generally accepted are as follows:

  • Hemolysis: abnormal peripheral smear, increased bilirubin <> 600 IU/L;
  • Elevated liver enzymes: aspartate aminotransferase (AST) ≥ 72 IU/L, lactate dehydrogenase (LDH) > 600 IU/L; and
  • Thrombocytopenia: platelet count <>Hellp Syndrome

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