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pediatric massive hepatic necrosis

Tuesday 10 May 2011

Synopsis

- fulminant hepatic failure

Etiology

- An etiologic diagnosis is established in 3% of cases.
- Association with aplastic anemia (7%)
- drug reactions (7/45)
- autoimmune hepatitis, type 2 (3/45)
- halothane hepatitis (1/45)
- ischemia/hypotension (1/45)
- mushroom poisoning (1/45)
- mitochondrial disorder (1/45)
- hemophagocytic lymphohistiocytosis (1/45)
- adenoviral hepatitis (1/45).

The extent of necrosis on liver biopsy correlates poorly with that in liver explants (mean difference, 32% +/- 23.8%).

Almost all cases can be classified into one of 2 broad patterns of necrosis:
- (1) zonal coagulative necrosis
- (2) panlobular (nonzonal) necrosis.

These patterns differed significantly with respect to several clinical parameters including sex ratio, peripheral blood white cell count, serum aspartate transaminase and alanine transaminase, conjugated bilirubin, and alkaline phosphatase levels.

Livers with panlobular necrosis show a spectrum of histopathologic findings that included central venulitis (76%), lymphocytic infiltration of large duct/gallbladder epithelium (54%), and syncytial giant cell transformation (18%).

These features are not seen in livers with zonal coagulative necrosis which frequently showed prominent steatosis (91%). Both patterns of necrosis frequently showed ductular proliferation (100%) and cholangiolitis (80%).

The diagnostic yield of ancillary studies (histochemistry, immunohistochemistry, and electron microscopy) was very low (@<@1%).

The small proportion of cases with etiologic diagnoses precluded correlation of clinical and histopathological parameters with specific etiologies.

References

- Clinicopathologic spectrum of massive and submassive hepatic necrosis in infants and children. Kirsch R, Yap J, Roberts EA, Cutz E. Hum Pathol. 2009 Apr;40(4):516-26. PMID: 19121848