Lectin-reactive alpha-fetoprotein in patients with tyrosinemia type I and hepatocellular carcinoma.

• Despite the introduction of 2-(2-nitro-4-trifluormethyl-benzoyl)-1,3-cyclohexandion into the treatment of hereditary tyrosinemia type I (HT1), patients remain at risk of developing hepatocellular carcinoma (HCC). Serial total alpha-fetoprotein (AFP) levels are used to monitor the individual patient. Lectin-reactive alpha-fetoprotein (L3-AFP) is an AFP isoform that is expressed by malignant liver tumors. We investigated whether the analysis of L3-AFP could lead to earlier detection of HCC in HT1 compared with judgement based on total AFP alone. AFP electrophoresis using lectin-containing agarose gel identifies L3-AFP by the affinity of its specific carbohydrate chain to lectin. We report the retrospective analysis of sequential serum samples of 12 patients with HT1 and histologically proven HCC. AFP isoforms could be identified in all 12 patients. In 6 patients, the L3-AFP increased before the total AFP. In 3 patients, the rise in L3-AFP was parallel to the rise of total AFP; and in 3 patients, the L3-AFP was raised after the total AFP or did not increase at all. We were able to identify 6 of 12 patients with an early increase in the new tumor marker. Lectin-affinity electrophoresis may have a role in discriminating benign liver disease from HCC in HT1. We suggest the further evaluation of L3-AFP in HT1.

Keyword

Adolescent

Biological Markers

blood

Carcinoma

Hepatocellular

blood

etiology

Child

Child

Preschool

Electrophoresis

Agar Gel

methods

Follow-Up Studies

Humans

Infant

Lectins

metabolism

Liver Neoplasms

blood

etiology

Protein Isoforms

blood

Retrospective Studies

Tumor Markers

Biological

blood

Tyrosinemias

blood

complications

alpha-Fetoproteins

metabolism

Publication and Content Type

ref (subject category)

art (subject category)