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Classification of Medicina et al for Hereditary Disorders of Fibrinogen Associated with Hepatocytic Inclusion Bodies

Description

Medicina et al classified patients with hereditary fibrinogen disorders associated with inclusion bodies within hepatocytes. The inclusions represent accumulations of fibrinogen unable to exit from the endoplasmic reticulum. The authors are from Spedali Civili Brescia in Italy and Christchurch University in New Zealand.


 

Features in common:

(1) other family members affected

(2) hypofibrinogenemia

(3) chronic liver disease, eventually progressing to cirrhosis

(4) inclusion within the cytoplasm of hepatocytes that react with antibodies to fibrinogen

 

Parameters:

(1) appearance of the inclusions

(2) missing portions of the fibrinogen

Inclusions within Hepatocytes

Missing Portions

Type

fiberglass-like (irregular or elongated)

none

I

single large inclusion filling the entire cytoplasm

B-beta, D, E

II

single or multiple, small, glassy and eosinophilic

B-beta, D, E

III

 

where:

• Fibrinogen is composed of A-alpha, B-beta and gamma chains, plus the D and E fragments.

 


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