Journal of Clinical and Experimental Hematopathology
Case Study
Ocular Adnexal IgG4-Producing Mucosa-Associated Lymphoid Tissue Lymphoma Mimicking IgG4-Related Disease
Yasuharu SatoKoh-ichi OhshimaKatsuyoshi TakataXingang HuangWei CuiKyotaro OhnoTadashi Yoshino
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Volume 52 (2012) Issue 1 Pages 51-55

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Abstract

IgG4-related disease is a recently proposed clinical entity with several unique clinicopathological features. A chronic inflammatory state with marked fibrosis, which can often be mistaken for malignancy, especially by clinical imaging analyses, unifies these features. In the present report, we describe a case of IgG4-producing mucosa-associated lymphoid tissue lymphoma mimicking IgG4-related disease. The patient was a 55-year-old male who was being followed for right orbital tumor over 1.5 years. The lesion had recently increased in size, so a biopsy was performed. Histologically, the lesion was consistent with IgG4-related disease ; however, IgG4+ plasma cells showed immunoglobulin light-chain restriction and immunoglobulin heavy chain gene rearrangement was detected in the lesion. Therefore, the lesion was diagnosed as IgG4-producing mucosa-associated lymphoid tissue lymphoma. In conclusion, in histological diagnosis of IgG4-related disease, it is important to examine not only IgG4-immunostain but also immunoglobulin light-chain restriction. [J Clin Exp Hematopathol 52(1) : 51-55, 2012]

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© 2012 by The Japanese Society for Lymphoreticular Tissue Research
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