1. Relevance of C5b9 immunostaining in the diagnosis of neonatal hemochromatosis.
- Author
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Dubruc E, Nadaud B, Ruchelli E, Heissat S, Baruteau J, Broué P, Debray D, Cordier MP, Miossec P, Russo P, and Collardeau-Frachon S
- Subjects
- Biomarkers analysis, France, Hemochromatosis diagnosis, Humans, Infant, Newborn, Liver pathology, Predictive Value of Tests, Prognosis, Retrospective Studies, Complement Membrane Attack Complex analysis, Hemochromatosis immunology, Immunohistochemistry, Liver immunology
- Abstract
Background: Neonatal hemochromatosis caused by a gestational alloimmune mechanism or gestational alloimmune liver disease (GALD) is a rare perinatal disorder characterized by intra- and extrahepatic iron overload. It is believed to result from complement-mediated liver injury, in which the classical complement pathway is activated by maternal antibody/fetal antigen complexes, leading to hepatocyte lysis by the membrane attack complex C5b9. According to some authors, C5b9 expression in more than 75% of liver parenchyma is specific for GALD., Methods: We conducted a retrospective multicentric immunohistochemical study with anti-C5b9 in GALD cases (n = 25) and non-GALD cases with iron overload (n = 36) and without iron overload (n = 18)., Results: C5b9 was expressed in 100% of GALD cases but involved more than 75% of the liver parenchyma in only 26% of the cases. C5b9 was detected in 26.75% of the non-GALD cases with more than 75% of positive parenchyma in maternal erythrocytic alloimmunization, herpes and enterovirus hepatitis, bile acid synthetic defect, DGUOK mutation, Gaucher disease, cystic fibrosis, and giant-cell hepatitis with autoimmune hemolytic anemia., Conclusion: Diagnosis and therapeutic management of GALD cannot only be based on C5b9 expression in liver samples as it is not specific of this disease.
- Published
- 2017
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