Sclerosing hyaline necrosis of the liver in Bloom syndrome

Bloom syndrome is a rare autosomal recessive disorder characterized by norm ally proportioned but strikingly small body size, a characteristic facies a nd photosensitive facial skin lesion, immunodeficiency, and a marked predis position to development of a variety of cancers. We describe here, we belie ve for the first time, pronounced sclerosing hyaline necrosis with Mallory bodies in the liver of a patient with Bloom syndrome. Mallory bodies are cy toplasmic eosinophilic inclusions, which are more common in visibly damaged , swollen hepatocytes in various liver diseases but are never found in norm al liver. The possible pathogenesis of this finding in Bloom syndrome is di scussed.