Original article
Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma with Polyclonal Hypergammaglobulinemia

https://doi.org/10.1016/j.ajo.2008.01.006Get rights and content

Purpose

To determine the characteristics of patients with primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma associated with polyclonal hypergammaglobulinemia.

Design

Case series study.

Methods

Among 81 Japanese patients with primary ocular adnexal MALT lymphoma, seven patients (9%) were diagnosed with polyclonal hypergammaglobulinemia. Patient clinical data included a history of autoimmune disease and dissemination. Peripheral blood collected from all patients was analyzed for serum levels of rheumatoid factor, soluble interleukin-2 receptor (sIL-2R), and immunoglobulins at the time of diagnosis and after each treatment.

Results

Seven patients with polyclonal hypergammaglobulinemia had elevated serum levels of rheumatoid factor, sIL-2R, immunoglobulin G (IgG), and immunoglobulin E (IgE) at the time of diagnosis. One patient had Sjogren syndrome. Six patients (86%) had a dissemination of the MALT lymphoma or lymphadenopathy at the time of diagnosis. Histopathologic examination of the patients with lymphadenopathy revealed not only MALT lymphoma but also secondary follicles. None of the seven patients showed improvement in serum levels of IgG, rheumatoid factor, or sIL-2R in spite of complete regression of the ocular lesions after radiotherapy. After administration of cyclophosphamide/doxorubicin/vincristine/prednisone and/or rituximab to three patients, all three showed improved serum levels of IgG, rheumatoid factor, and sIL-2R.

Conclusions

Patients with ocular adnexal MALT lymphoma and polyclonal hypergammaglobulinemia have elevated serum levels of rheumatoid factor, sIL-2R, and IgE, and high dissemination or lymphadenopathy. These unique characteristics may correlate with the systemic immunologic imbalances.

Section snippets

Patients

The medical records of 81 patients with primary ocular adnexal MALT lymphoma who were examined between April 4, 2001 and August 30, 2007 were evaluated. Patients with a history of extraocular lymphoma were excluded. A complete medical history and laboratory data that included gammaglobulin levels were obtained for each patient, and patients with gammaglobulin levels of 2.0 g/dl or higher were classified as having hypergammaglobulinemia. Of the 81 patients, eight were found to have

Patient Clinical Data

Table 1 shows age, gender, lesion location, clinical stage, treatment, and outcome for all patients included in this study. Age and gender of the patients with ocular adnexal MALT lymphoma were not significantly different from those of previously described ocular adnexal MALT lymphoma patients.2 The anatomic lesions were bilateral in five patients (71%), and four patients (57%) had bilateral diffuse orbital or lacrimal gland lesions (Figure). One patient had Sjogren syndrome.

Six patients (86%)

Discussion

Patients with polyclonal hypergammaglobulinemia have different degrees of elevated rheumatoid factor, sIL-2R, and IgE. Although different diseases are associated with polyclonal hypergammaglobulinemia,5 we hypothesize that the common etiology of these serologic immunologic abnormalities may be systemic immunologic imbalances induced by activated Th2 cells. CD4+ helper T cells include Th1 cells and Th2 cells, which regulate each other;6 the immunologic imbalances induced by activated Th2 cells

Toshinobu Kubota, MD, has worked at National Hospital Organization, Nagoya Medical Center, a medical referral center in the middle of Japan. He graduated from the Mie University School of Medicine in 1992 and completed his residency in Ophthalmology at the Nagoya University School of Medicine in 1997. Dr Kubota has been engaged in research on ocular oncology, orbital diseases, and tumors.

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Toshinobu Kubota, MD, has worked at National Hospital Organization, Nagoya Medical Center, a medical referral center in the middle of Japan. He graduated from the Mie University School of Medicine in 1992 and completed his residency in Ophthalmology at the Nagoya University School of Medicine in 1997. Dr Kubota has been engaged in research on ocular oncology, orbital diseases, and tumors.

See accompanying Perspective on page 941.

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