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Enterocolic lymphocytic phlebitis of the cecal pole and appendix vermiformis with increase of IgG4-positive plasma cells

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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.

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The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Institute of Clinical Pathology, University Hospital Zurich, Schmelzbergstrasse 12, 8091, Zurich, Switzerland

    Sarah Comtesse, Juliane Friemel & Achim Weber

  2. Surgical Clinic, Waid City Hospital, Zurich, Switzerland

    René Fankhauser

Authors
  1. Sarah Comtesse
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  2. Juliane Friemel
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  3. René Fankhauser
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  4. Achim Weber
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Correspondence to Achim Weber.

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Comtesse, S., Friemel, J., Fankhauser, R. et al. Enterocolic lymphocytic phlebitis of the cecal pole and appendix vermiformis with increase of IgG4-positive plasma cells. Virchows Arch 464, 113–116 (2014). https://doi.org/10.1007/s00428-013-1507-0

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  • DOI: https://doi.org/10.1007/s00428-013-1507-0

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