Sclerosing hyaline necrosis of the liver in Bloom syndrome.
Bloom syndrome is a rare autosomal recessive disorder characterized by normally proportioned but strikingly small body size, a characteristic facies and photosensitive facial skin lesion, immunodeficiency, and a marked predisposition to development of a variety of cancers. We describe here, we believe for the first time, pronounced sclerosing hyaline necrosis with Mallory bodies in the liver of a patient with Bloom syndrome. Mallory bodies are cytoplasmic eosinophilic inclusions, which are more common in visibly damaged, swollen hepatocytes in various liver diseases but are never found in normal liver. The possible pathogenesis of this finding in Bloom syndrome is discussed.
D000328:Adult; D001816:Bloom Syndrome / Q000473:pathology*; D006801:Humans; D008107:Liver Diseases / Q000473:pathology*; D008297:Male; D009336:Necrosis; D012598:Sclerosis / Q000473:pathology