Liver and intrahepatic bile ducts-nontumor

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Liver and intrahepatic bile ducts-nontumor

Systemic disease/conditions

Pregnancy

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 16 May 2012, last major update May 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

Acute fatty liver of pregnancy

General
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● Minimal to modest hepatic dysfunction due to defect in mitochondrial fatty acid oxidation that may progress to hepatic failure
● Usually presents in third trimester of primiparas with bleeding, nausea, vomiting, jaundice, coma
● Incidence of 1 case per 15,000 pregnancies
● 20% have coexisting preeclampsia
● Usually mild but may cause death

Treatment
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● Terminate pregnancy

Gross description
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● Pale, yellow, small liver

Micro description
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● Microvesicular steatosis in zones 2 or 3 (vacuoles may be very small)
● Also marked ballooning of hepatocytes and macrovesicular fat
● In severe cases, hepatocyte dropout, reticulin collapse, portal tract inflammation

Positive stains (frozen tissue)
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● Fat stains (Oil red O, Sudan black)

Differential diagnosis
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● Reye’s syndrome, salicylate intoxication, Jamaican vomiting sickness, Labrea hepatitis, yellow fever, carnitine deficiency

Additional references
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● World J Gastroenterol 2006;12:7397, Lancet 2010;375:594

Intrahepatic cholestasis of pregnancy

General
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● Second leading cause of gestational jaundice (after viral hepatitis)
● Usually occurs late in pregnancy
● Estrogenic hormones may inhibit hepatocellular bile secretory activity
● Mother at risk for gallstones, malabsorption
● Associated with higher incidence of fetal distress, stillbirths, prematurity
● Symptoms: pruritis in third trimester, dark urine, light stools, jaundice
● Laboratory: elevated serum bilirubin (< 5 mg/dl), usually conjugated; mildly elevated alkaline phosphatase

Micro description
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● Mild cholestasis without cirrhosis

Preeclampsia and eclampsia

General
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● Preeclampsia: complication of pregnancy in third trimester, usually primiparas, with maternal hypertension, proteinuria, peripheral edema, coagulation abnormalities, DIC
● Case report of woman in mid-30’s with preeclampsia and subcapsular liver hematoma at 23 weeks gestation (Arch Pathol Lab Med 2003;127:1639, CT, gross and micro image)
● Ecclampsia: preeclampsia, seizures and hyperreflexia; leading cause of maternal death, often due to delay in diagnosis
● Liver disease as part of HELLP syndrome: Hemolysis, Elevated Liver enzymes, Low Platelets; associated with 4-12% of cases of preeclampsia; microangiopathic hemolysis with thrombocytopenia; 10-60% infant mortality due to placental abruption, intrauterine ischemia or prematurity
● Laboratory: early mild increase in liver enzymes, late coagulopathy

Treatment
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● Termination of pregnancy in severe cases

Gross description
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● Pale, firm liver with small red hemorrhagic patches (hematoma dissecting under Glisson’s capsule and causing hepatic rupture)

Gross images
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Small hemorrhagic infarcts and atrophy

Micro description
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● Periportal fibrin deposits with hemorrhage into space of Disse, causing periportal hepatocellular necrosis
● Less commonly bile inspissation in canaliculi and ductules, steatosis, portal lymphoplasmacytic infiltrate

Micro images
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Focal hepatocellular necrosis with fibrin thrombi (image on right)

Differential diagnosis
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● Acute viral hepatitis

Additional references
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● Obstet Gynecol Clin North Am 2010;37:269

Viral hepatitis

General
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● Leading cause of gestational jaundice, although also most common cause of jaundice in all women in this age group
● Similar to hepatitis in nongestational cases
● Risks of hepatitis B to fetus include acute infection, chronic carrier state, hepatocellular carcinoma

End of Liver and intrahepatic bile ducts-nontumor > Systemic disease/conditions > Pregnancy

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