Pregnancy Associated Fulminant Hepatic Failure; a Clinicopathologic Study
BV Naini, CR Lassman. David Geffen School of Medicine at UCLA, Los Angeles, CA
Background: Pregnancy associated fulminant hepatic failure is a rare but fatal condition for which emergent liver transplantation (LT) is the only viable option. The etiology is broad and includes distinct entities of acute fatty liver of pregnancy (AFLP), preeclampsia/eclampsia and HELLP (hemolysis, elevated liver enzymes and low platelet) syndrome; the histologic features of these entities have been described in the literature based on studies performed more than a decade ago.
Design: We performed a retrospective study of all cases of pregnancy associated liver failure necessitating transplantation at our institution from 1984-2008. Eight cases were identified, the largest single-center report of LT for pregnancy related liver diseases. Clinical data including the gestational age at the onset of liver dysfunction, date of delivery and LT, and laboratory studies including viral and autoimmune serologies were collected by medical record review. We performed a comprehensive histologic exam of the explants.
Results: Patients ranged in age from 23-37. None had prior clinical history of liver disease, and all presented with fulminant hepatic failure. Six delivered live healthy babies (4 C/S, 2 vaginal), and 2 had fetal loss (1 stillbirth). The onset of liver failure relative to LT ranged from a few days to 1.5 months. LT in 1 case happened at 18 weeks of gestation; for the remainder, the date of LT ranged from 2 days to 1.5 months post partum. Histologic evaluation of all cases showed hepatocellular necrosis ranging from patchy zone 3 to massive panlobular necrosis. Four cases had moderate to severe portal lymphocytic infiltrate; viral and autoimmune serologies were negative in 3 of 4 cases; 1 case had positive surface and core hepatitis B antibodies but no surface antigen. Steatosis ranged from none (5 cases), mild pericentral (2 cases) to moderate macrovesicular steatosis (1 case). Sinusoidal fibrin thrombi were not identified in any of the cases. One case demonstrated a background of bridging fibrosis with an elevated copper and a presumptive diagnosis of Wilson's disease was made.
Conclusions: In our experience of 8 patients with pregnancy associated liver failure and a clinical diagnosis of HELLP syndrome, all showed extensive necrosis but none showed the characteristic histologic features described for AFLP, eclampsia or HELLP syndrome. Three cases had infiltrates suggestive of hepatitis but had negative serologies, and one case had Wilson's disease.
Category: Liver & Pancreas
Monday, March 9, 2009 9:30 AM
Poster Session I Stowell-Orbison/Autopsy Award # 209, Monday Morning