Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jul 16, 2015; 3(7): 650-654
Published online Jul 16, 2015. doi: 10.12998/wjcc.v3.i7.650
Perifollicular granulomas with IgG4 plasmacytosis: A case report and review of literature
Li Liang, Jain Zhou, Lei Chen
Li Liang, Jain Zhou, Lei Chen, Department of Pathology, the University of Texas Medical School at Houston, Houston, TX 77030, United States
Li Liang, Department of Pathology, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
Author contributions: Liang L and Chen L designed the report, collected the patient’s clinical data and wrote the paper; Zhou J collected the patient’s clinical data and revised the manuscript.
Supported by Department of Pathology, the University of Texas Health Science Center at Houston, United States.
Institutional review board statement: This study was conducted with an approved IRB protocol from the University of Texas Medical School in Houston, Texas.
Informed consent statement: This study was exempted by the Committee for Protection of Human Subjects.
Conflict-of-interest statement: None.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Lei Chen, MD, Department of Pathology, the University of Texas Medical School at Houston, 6431 Fannin, MSB 2.136, Houston, TX 77030, United States. lei.chen.1@uth.tmc.edu
Telephone: +1-713-5664690
Received: January 26, 2015
Peer-review started: January 28, 2015
First decision: March 20, 2015
Revised: April 13, 2015
Accepted: May 16, 2015
Article in press: May 18, 2015
Published online: July 16, 2015
Abstract

Perifollicular granuloma is a unique histologic feature and whether it is associated with immunoglobulin G4 (IgG4)-related disease is controversial. We report a case of a 38-year-old man who presented with worsening left eye pain, proptosis, tearing, gritty sensation, blurred vision and multiple lymphadenopathy. An axillary lymph node resection showed reactive follicular and interfollicular lymph node hyperplasia, and increased eosinophils and plasma cells (at least 80% of IgG+ plasma cells were positive for IgG4). A distinct feature was the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. The human herpes virus 8 was not detected by immunohistochemistry. In addition, an extensive panel of special stains, immunohistochemistry, and flow cytometry was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG4-related sclerosing disease-associated lymphadenopathy. Further laboratory testing showed a significant increase of serum immunoglobulin E (> 23000 IU/mL) and slight increase of total IgG, but normal serum IgG4. Even though perifollicular granuloma is a nonspecific histopathologic feature and can be seen in other diseases, such as nodular lymphocyte predominant Hodgkin lymphoma, IgG4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.

Keywords: Immunoglobulin G4-related disease, Lymphadenopathy, Plasma cells, Granuloma

Core tip: We report a case of a 38-year-old man who presented with worsening left eye pain and multiple lymphadenopathy. An axillary lymph node resection showed increased eosinophils and plasma cells (at least 80% of immunoglobulin (Ig)G+ plasma cells were positive for IgG4 and the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. An extensive workup was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG4-related sclerosing disease-associated lymphadenopathy. Thus, IgG4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.