HELLP syndrome

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This syndrome, a complication of pre-eclampsia, is due to hepatic ischemia giving rise to periportal hemorrhage and necrosis together with microan-giopathic hemolytic anemia and thrombocyto-penia (Weinstein, 1982). The mnemonic ''HELLP'' therefore stands for: Hemolysis, Elevated Liver enzymes and Low Platelets. These patients present with epigastric pain and in severe cases are usually obviously ill because of associated complications including renal failure (characterized by rising urea, creatinine and the passage of small quantities of bloodstained or ''Coke'' colored urine) and eclampsia. Many women with HELLP syndrome, however, seem to have unremarkable clinical disease.

Liver failure may arise from conditions that mimic pre-eclampsia such as thrombotic throm-bocytopenic purpura and acute fatty liver of pregnancy (see below) (Atlas et al., 1982; Kaplan, 1985). Obstetric cholestasis and viral hepatitis may also enter the differential diagnosis in milder cases of HELLP syndrome. Distinguishing between these conditions may be difficult but the hallmark of pre-eclamptic disease is that it resolves after delivery (Chandran et al., 1992).

Subcapsular liver hematoma is a rare complication of the HELLP syndrome. The surface of the liver is covered in petechial hemorrhages that may coalesce to form one large hematoma. Rupture of a hematoma is a life-threatening complication that presents with right-upper quadrant pain and sudden hypovolemia. Management is usually surgical and involves packing the abdomen to put pressure on the bleeding surface, drainage of the subdiaphragmatic spaces with wide bore drains, aggressive blood product replacement and careful attention to body temperature control. Cautery, resection and suturing of the liver are not recommended as methods of controlling the bleeding. Arterial embolization has been used and may be an option in selected cases. Recent use of Factor VII replacement in massive obstetric hemorrhage has been suggested and may also be an option (Bouwmeester et al., 2003; Danilos et al., 2003).

Coagulopathy is an uncommon feature of HELLP syndrome but thrombocytopenia and impaired platelet function both give rise to impaired coagulation. Prolonged partial thromboplastin times and INRs are more likely to occur in association with acute fatty liver than HELLP syndrome. Hypoglycemia may occur in some cases but is more characteristic of acute fatty liver of pregnancy.

The management of HELLP syndrome is delivery while the associated complications (renal failure, eclampsia and respiratory distress) may require critical care on their individual merits. Thrombocytopenia should reach a nadir within 72 h of delivery and if it is persistent beyond this point, a search for alternative diagnoses should commence (e.g. sepsis, folate deficiency, throm-botic thrombocytopenic purpura, systemic lupus erythematosus). Although steroids have been advocated as a means of accelerating the resolution of postpartum eclampsia, the studies involved are small and show no reduction in mortality.

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