title: Enterocolic lymphocytic phlebitis of the cecal pole and appendix vermiformis with increase of IgG4-positive plasma cells.
abstract: Here we describe the clinicopathological course of a 20-year-old female patient with enterocolic lymphocytic phlebitis (ELP) of the appendix vermiformis and cecal pole with increase of IgG4-positive plasma cells. The patient presented with acute abdomen, suspicious of acute appendicitis. Diagnostic laparoscopy showed tumefaction of the cecal pole and appendix vermiformis. Histologic examination revealed mural thickening and a dense lymphoplasmocytic, partly obliterative infiltrate of the veins with sparing of the arteries, diagnostic of ELP. In addition, we found an elevated number of IgG4-positive plasma cells blended in with the lymphocytes. The IgG4-to-IgG ratio accounted for >40 %. This case meets the histopathological criteria requested for IgG4-related disease (IgG4-RD) and thus opens the possibility that ELP might be part of the IgG4-RD spectrum.
authors.affiliation: Institute of Clinical Pathology, University Hospital Zurich, Schmelzbergstrasse 12, 8091, Zurich, Switzerland.
authors.forename: Sarah
authors.initials: S
authors.lastname: Comtesse
authors.forename: Juliane
authors.initials: J
authors.lastname: Friemel
authors.forename: René
authors.initials: R
authors.lastname: Fankhauser
authors.forename: Achim
authors.initials: A
authors.lastname: Weber
mesh_terms: D000328:Adult; D001065:Appendix / Q000098:blood supply* / Q000473:pathology; D002432:Cecum / Q000098:blood supply* / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D010689:Phlebitis / Q000276:immunology* / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology*
chemical_list: D007074:Immunoglobulin G