Ocular Adnexal IgG4-Producing Mucosa-Associated Lymphoid Tissue Lymphoma Mimicking IgG4-Related Disease

Sato, Yasuharu; Ohshima, Koh Ichi; Takata, Katsuyoshi; Huang, Xingang; Cui, Wei; Ohno, Kyotaro; Yoshino, Tadashi

doi:10.3960/jslrt.52.51

Cited by 49 publications

(42 citation statements)

References 8 publications

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“…Although IgG4 expression has been described in a single case of a nodal marginal zone lymphoma, 21 few cases of ocular adnexae marginal zone lymphomas, [17][18][19]22 as well as in a subset of marginal zone lymphomas of the dura, 16 the rate of 39% IgG4-positive cases among our primary cutaneous marginal zone lymphomas is striking. Only two IgG4-rearranged primary cutaneous marginal zone lymphomas, identified by RT-PCR, were previously reported.…”

Section: Discussionmentioning

confidence: 81%

“…This scenario raises the question of a secondary skin involvement by an originally noncutaneous marginal zone lymphoma. 2,6 Given some recent reports that particular subsets of marginal zone lymphomas, specifically epidural and ocular marginal zone lymphomas, [16][17][18][19] show increased frequencies of IgG4 expression and based on the known fact that primary cutaneous marginal zone lymphomas predominantly express IgG in contrast to other marginal zone lymphomas, we were interested to study the prevalence of IgG4 expression in primary cutaneous marginal zone lymphomas in comparison with various noncutaneous marginal zone lymphomas. The concept of IgG4-related diseases has attracted major attention in recent years comprising an ever increasing amount of manifestations of various organs (autoimmune pancreatitis type 1, Mikulicz's disease, Kuttner's tumor, and retroperitoneal fibrosis).…”

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confidence: 99%

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Primary cutaneous marginal zone lymphomas with plasmacytic differentiation show frequent IgG4 expression

et al. 2013

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The expression of IgG4 in malignant B-cell lymphomas has only partially been studied. Recent reports described single cases of marginal zone lymphomas arising in the ocular adnexae that express IgG4. Moreover, a subset of dura-associated marginal zone lymphomas appear to express IgG4 as well. We investigated IgG4 expression in a more systematic manner in a large cohort of marginal zone lymphoma specimens derived from the archive of our institute. Overall, we examined 169 marginal zone lymphomas of various primary sites that displayed a distinct plasmacytic differentiation and light chain restriction, allowing for a detailed investigation of the immunoglobulin heavy chain expression in these tumors by immunohistochemistry. Unexpectedly, primary cutaneous marginal zone lymphomas showed frequent IgG4 expression. Although only 1 out of 120 noncutaneous marginal zone lymphomas, located in the ocular adnexae, expressed IgG4, 19 of 49 (39%) primary cutaneous marginal zone lymphomas showed this feature, constituting the highest expression rate of IgG4 reported to date in any B-cell lymphoma. None of the IgG4-positive cutaneous marginal zone lymphomas with available clinical data showed evidence of a preexisting systemic IgG4-related disease, suggesting a localized immunologic IgG4-driven pathogenetic process at early stages of the disease. IgG4-positive and IgG4-negative primary cutaneous marginal zone lymphomas did not significantly differ in architectural features of the infiltrate or the composition of the reactive T-cell infiltrate as determined by analysis of T-cell content, CD4/CD8 ratio, and content of FOXP3-and PD1-positive T cells. Although the pathogenetic role of IgG4 expression in a significant subset of primary cutaneous marginal zone lymphomas with plasmacytic differentiation remains unclear at present, the demonstration of IgG4 expression in a marginal zone lymphoma involving the skin might be a helpful clue in the routine diagnostic setting, as these tumors will almost invariably be of primary cutaneous origin with an extremely low risk of spread to noncutaneous sites and an excellent prognosis.

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Section: Discussionmentioning

confidence: 81%

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confidence: 99%

Primary cutaneous marginal zone lymphomas with plasmacytic differentiation show frequent IgG4 expression

et al. 2013

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“…In such cases, numerous non-neoplastic, IgG4-positive cells can be found among the malignant tumor cells. In contrast, we previously described marginal zone B cell lymphomas in which the tumor cells produced IgG4, which suggests that the infiltrating IgG4-positive cells observed in IgG4-RD can become malignant [10].…”

Section: Introductionmentioning

confidence: 78%

IgG4-producing lymphoma arising in a patient with IgG4-related disease

et al. 2016

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We herein report a case in which an IgG4-producing lymphoma arose in a patient with a previous diagnosis consistent with an IgG4-related disease. A 43-year-old man presented with enlarged cervical lymph nodes and was treated with steroids and radiation for what was initially assumed to be Kimura's disease, although the lesions were later histologically re-diagnosed as IgG4-related lymphadenopathy. Fourteen years later, when the patient was 58-years-old, he presented with retroperitoneal fibrosis and swollen lymph nodes. The suspicious lesions were not histologically examined as the patient did not give consent. However, the serum IgG4 concentration was high (1400 mg/dL) and he was clinically diagnosed with systemic IgG4-related disease. Although steroid administration reduced the size of the lesions, tapering the dose finally resulted in systemic, prominently enlarged lymph nodes. Analysis of the biopsy specimen revealed that these multiple lymph node lesions were marginal zone B cell lymphomas that themselves expressed IgG4. Complete remission was achieved after a total of six courses of chemotherapy including rituximab. This case suggests that the infiltrating IgG4-expressing cells observed in IgG4-related disease can clonally expand to malignant lymphomas.

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“…Therefore, symptomatic therapy, such as the appropriate administration of eye drops, in the management of IgG4-related ocular adnexal disease may be appropriate, unless progressive deterioration of eye symptoms is observed. A case of ocular adnexal malignant lymphoma developing in a patient with IgG4-RD during the follow-up period has been reported (28). Therefore, conducting periodical physical examinations, laboratory tests and imaging tests is warranted.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related Ocular Adnexal Disease Mimicking Thyroid-associated Orbitopathy

et al. 2013

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A 72-year-old man presented with bilateral eyelid swelling and redness. An orbital CT scan showed bilateral proptosis, extraocular muscle enlargement and swollen lacrimal glands, mimicking thyroid-associated orbitopathy (TAO) with Hashimoto's thyroiditis (HT). During the patient's clinical course, spontaneous remission of lung consolidation (35×26 mm) was seen. A diagnosis of IgG4-related disease (IgG4-RD) was made based on an elevated serum IgG4 level (1,020 mg/dL; normal, 4-108), predominance of IgG4-positive plasma cells (IgG4/IgG: 35/70 in HPF) in the lacrimal glands and typical features of Mikulicz's disease. This report provides a novel description of this unusual disease entity among HT, TAO and IgG4-RD.

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Ocular Adnexal IgG4-Producing Mucosa-Associated Lymphoid Tissue Lymphoma Mimicking IgG4-Related Disease

Cited by 49 publications

References 8 publications

Primary cutaneous marginal zone lymphomas with plasmacytic differentiation show frequent IgG4 expression

Primary cutaneous marginal zone lymphomas with plasmacytic differentiation show frequent IgG4 expression

IgG4-producing lymphoma arising in a patient with IgG4-related disease

IgG4-related Ocular Adnexal Disease Mimicking Thyroid-associated Orbitopathy

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