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neonatal giant cell hepatitis

Monday 15 November 2010

Synopsis

- extramedullary hematopoiesis (74%), including both myelopoiesis and erythropoiesis
- portal and lobular inflammation mild-to-absent in 95% of cases
- lobular cholestasis (from mild to moderate) with predominantely canalicular pattern (84%).
- bile ducts hypoplastic (32%) but not absent or reduced in numbers.
- mild focal ductular proliferation (18%)
- portal fibrosis or pericellular fibrosis (30%; advanced in 8%).

Idiopathic (49%),

Secondary

- hypopituitarism (16%)
- biliary atresia (8%)
- Alagille syndrome (6%)
- bile salt defects (6%)
- other entities (5%)

Of note, the biopsy findings did not readily distinguish between the different etiologies with the exception that bile duct hypoplasia was more common in cases of hypopituitarism. A pan-hypopituitarism is the most common recognizable clinical association with neonatal giant cell hepatitis.

References

- Neonatal giant cell hepatitis: histological and etiological findings. Torbenson M, Hart J, Westerhoff M, Azzam RK, Elgendi A, Mziray-Andrew HC, Kim GE, Scheimann A. Am J Surg Pathol. 2010 Oct;34(10):1498-503. PMID: 20871223