Premium
Hepatocyte inclusions of δ 1 ‐antichymotrypsin in a patient with partial deficiency of δ 1 ‐antichymotrypsin and chronic liver disease
Author(s) -
LINDMARK B.,
MILLWARDSADLER H.,
CALLEA F.,
ERIKSSON S.
Publication year - 1990
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/j.1365-2559.1990.tb01107.x
Subject(s) - endoplasmic reticulum , hepatocyte , immunohistochemistry , liver disease , pathology , biology , chemistry , medicine , microbiology and biotechnology , biochemistry , in vitro
We present a case of chronic liver disease with selective and exclusive hepatocyte endoplasmic reticulum storage of δ 1 ‐antichymotrypsin in the form of granules, detected by specific immunohistochemistry at the light microscopy level and corresponding to material found in dilated endoplasmic reticulum of hepatocytes by electron microscopy. The patient had intermediate deficiency of δ 1 ‐antichymotrypsin. Thus, the hepatocyte accumulation of δ 1 ‐antichymotrypsin may indicate the presence of an export block resembling that of a closely‐related protein, namely PiZ δ 1 ‐antitrypsin. It is proposed that hepatocyte storage of δ 1 ‐antichymotrypsin may be an expression of an inborn error of metabolism bearing the characteristics of endoplasmic reticulum storage diseases such as PiZ δ 1 ‐antitrypsin deficiency and hereditary hypofibrinogenaemia.