Fibrinogen storage disease without hypofibrinogenemia associated with estrogen therapy.
BACKGROUND
Cytoplasmic inclusion bodies within hepatocytes may have different etiologies, including the Endoplasmic Reticulum Storage Diseases (ERSDs). ERSD is a pathological condition characterized by abnormal accumulation of proteins destined for secretion in the endoplasmic reticulum of hepatocytes; it may be congenital (primary) or acquired (secondary). Fibrinogen storage disease is a form of ERSD.


METHODS
We present a case of fibrinogen storage disease secondary to estrogen replacement therapy. Its causal relationship to the drug is shown by histological, immunohistochemical and ultrastructural studies of paired liver biopsies obtained during and after the drug therapy.


CONCLUSIONS
The liver biopsies of patients with idiopathic liver enzyme abnormalities should be carefully evaluated for cytoplasmic inclusion bodies and, although rare, fibrinogen deposits.
Simsek
Z
Z
Gastroenterology Department, Gazi University, Faculty of Medicine, Ankara, Turkey. zahidesimsek@yahoo.com
Ekinci
O
O
Cindoruk
M
M
Karakan
T
T
Degertekin
B
B
Akyol
G
G
Unal
S
S
D000328:Adult; D004721:Endoplasmic Reticulum / Q000378:metabolism*; D015914:Estrogen Replacement Therapy / Q000009:adverse effects*; D005260:Female; D005340:Fibrinogen / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000648:ultrastructure; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology; D008659:Metabolic Diseases / Q000139:chemically induced* / Q000150:complications / Q000378:metabolism / Q000473:pathology; D008854:Microscopy, Electron; D009336:Necrosis
D005340:Fibrinogen