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International Journal of Hematology
Published in: International Journal of Hematology 5/2010

01-12-2010 | Case Report

Lymphadenopathy of IgG4-related sclerosing disease: three case reports and review of literature

Authors: Ikuo Shimizu, Kentaro Nasu, Keijiro Sato, Hiromitsu Ueki, Daigo Akahane, Masahiko Sumi, Mayumi Ueno, Naoaki Ichikawa, Naoko Asano, Masaru Kojima, Hikaru Kobayashi

Published in: International Journal of Hematology | Issue 5/2010

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Abstract

Immunoglobulin (Ig) G4-related sclerosing disease is a recently described syndrome characterized by lymphoplasmacytic infiltration of exocrine glands or extranodal tissues and elevated serum IgG4. We report three cases of lymphadenopathy secondary to IgG4-related sclerosing disease. Histologic features of involved lymph nodes included interfollicular immunoblasts and plasma cells, similar to Castleman’s disease. The percentage of IgG4-/IgG-positive plasma cells in the three patients was markedly elevated (30, 50, and 60%). Administration of prednisolone led to remission in every case. One of three cases was consulted to our hospital due to suspected diagnosis of angioimmunoblastic T cell lymphoma (AITL). The case demonstrates many clinical and pathologic similarities between IgG4-related sclerosing disease and AITL. Pathological similarities between AITL and the lymphoplasmacytic subtype of IgG4-sclerosing disease have recently been reported. It is important to accurately diagnose IgG4-related lymphadenopathy given its ready response to steroid therapy and the potential for misdiagnosing lymphoma on clinical grounds.
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Metadata
Title
Lymphadenopathy of IgG4-related sclerosing disease: three case reports and review of literature
Authors
Ikuo Shimizu
Kentaro Nasu
Keijiro Sato
Hiromitsu Ueki
Daigo Akahane
Masahiko Sumi
Mayumi Ueno
Naoaki Ichikawa
Naoko Asano
Masaru Kojima
Hikaru Kobayashi
Publication date
01-12-2010
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 5/2010
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-010-0721-7

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