Sclerosing hyaline necrosis of the liver in Bloom syndrome.

Abstract:

: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.

journal_name

Arch Pathol Lab Med

authors

Wang J,Cornford ME,German J,French SW

doi

10.1043/0003-9985(1999)123<0346:SHNOTL>2.0.CO;2

subject

Has Abstract

pub_date

1999-04-01 00:00:00

pages

346-50

issue

4

eissn

0003-9985

issn

1543-2165

journal_volume

123

pub_type

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