title: Hepatic nodular lymphoid lesion with increased IgG4-positive plasma cells associated with primary biliary cirrhosis: a report of two cases.
abstract: The nodular lymphoid lesion of the liver known as reactive lymphoid hyperplasia or pseudolymphoma is rare and its pathogenesis is unknown. We report two cases of nodular lymphoid lesions of the liver with numerous IgG4-positive plasma cells in patients with primary biliary cirrhosis. Histologically, in both cases, the lesion showed a dense lymphoplasmacytic infiltrate with lymphoid follicles and granulomas. Fibrous tissue was scarce and without a storiform pattern. Obliterative phlebitis was not identified. The IgG4+ plasma cell counts were 82 and 76 per high power field, with an IgG4/IgG ratio of 75 and 64 %, respectively, which qualifies the lesions according to the diagnostic criteria for IgG4-related disease as « probable histological feature of IgG4-related disease ». There were no rearrangements of immunoglobulin heavy-chain genes and plasma cells had a polytypic pattern of kappa and lambda light-chain expression. The non-tumor liver showed primary biliary cirrhosis with destructive cholangitis without IgG4 plasma cells. In both cases, IgG4-related disease was not found in other organs neither at the time of diagnosis nor 3 years later. Serum IgG4 levels normalized after local ablation of the lesions. It seems unlikely that these lesions are a manifestation of IgG4-related disease. However, because the pathogenesis of both nodular lymphoid lesions and IgG4-related disease remains unclear, further studies are needed to elucidate a potential link between nodular lymphoid lesions of the liver and an increased number of IgG4 plasma cells. More definite conclusions will be possible when the pathogenesis of IgG4-related disease has been clarified.
authors.affiliation: Department of Pathology, AP-HP Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, F-75012, Paris, France. jessica.calvo@aphp.fr.|Sorbonne Universités, UPMC, Univ Paris 06, Paris, France. jessica.calvo@aphp.fr.
authors.forename: Jessica
authors.initials: J
authors.lastname: Calvo
authors.affiliation: Department of Hepatology, AP-HP Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, F-75012, Paris, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Carbonell
authors.affiliation: Department of Liver Surgery and Transplantation, AP-HP Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, F-75012, Paris, France.|Sorbonne Universités, UPMC, Univ Paris 06, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Scatton
authors.affiliation: Department of Laboratory Hematology, AP-HP Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, F-75012, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Marzac
authors.affiliation: Department of Hepatology, AP-HP Hôpital Jean Verdier, avenue du 14 juillet, F-93140, Bondy, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Ganne-Carrie
authors.affiliation: Department of Pathology, AP-HP Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, F-75012, Paris, France.|Sorbonne Universités, UPMC, Univ Paris 06, Paris, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Wendum
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007150:Immunohistochemistry / Q000379:methods; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000473:pathology*; D008206:Lymphatic Diseases / Q000175:diagnosis / Q000473:pathology*; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G
keywords: IgG4; Liver; Lymphoid hyperplasia; Primary biliary cirrhosis