Local Recurrence as Immunoglobulin G4 (IgG4)-Related Disease 10 Years after Radiotherapy to Ocular Adnexal Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue

  • Matsuo Toshihiko
    Departments of Ophthalmology, Okayama University Medical School, and Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
  • Ichimura Kouichi
    Departments of Pathology, Okayama University Medical School, and Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
  • Yoshino Tadashi
    Departments of Pathology, Okayama University Medical School, and Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences

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In 2000, a 48-year-old woman developed a left orbital mass with lacrimal gland involvement and then, in 2003, a right orbital mass with lacrimal gland involvement, both of which were diagnosed as extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). She underwent 30 Gy external beam radiation to bilateral orbital lesions. The lymphoma cells in both lesions did not share the same clonality, as shown by amplification by polymerase chain reaction of the immunoglobulin heavy chain gene. Immunoglobulin light chain analysis by immunohistochemistry and messenger RNA in situ hybridization showed λ chain monotype in the left orbital lesion but κ chain monotype in the right orbital lesion. She developed recurrent left orbital mass with high uptake on fluorodeoxyglucose positron emission tomography fused with computed tomography in 2010, and excisional biopsy disclosed the formation of follicles and infiltration with immunoglobulin G4 (IgG4)-positive plasma cells mainly in interfollicular areas. The immunoglobulin light chain analysis showed the λ chain and κ chain bitype. With the immunohistopathological diagnosis of IgG4-related disease, the serum IgG4 level was found to show elevation at 376 mg/dL, and the patient chose observation. This is the first reported case of development of IgG4-related disease after bilataral orbital MALT lymphoma with external beam radiotherapy. [J Clin Exp Hematopathol 51(2) : 125-133, 2011]

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