IgG4-producing marginal zone B-cell lymphoma

Our plain-English summary of IgG4-producing marginal zone B-cell lymphoma, by Y. Sato, K. Takata, K. Ichimura, et al (International Journal of Hematology, 2008).

The study

• Authors: Y. Sato, K. Takata, K. Ichimura, T. Tanaka, T. Morito, M. Tamura, T. Yoshino
• Journal: International Journal of Hematology
• Published: 2008;88(4):428-433
• Type of article: Case report
• Cited by: 73 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

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. Little is known about lymphomagenesis in the context of IgG4-related disease, we recently first reported the ocular adnexal marginal zone B-cell lymphomas arising from IgG4-related disease. To the best of our knowledge, no existing study has ever established the neoplastic potential of IgG4-producing cells. In the present report, we describe the first IgG4-producing lymphoma. The patient was a 72-year-old male who was being followed for an asbestos-related pleural plaque. During follow-up, computed tomography revealed bilateral renal masses and multiple swollen retroperitoneal lymph nodes. A retroperitoneal lymph node biopsy was performed. Histologically, the interfollicular areas were expanded by medium to large plasmacytoid cells. These plasmacytoid cells showed nuclear pleomorphism and had prominent Russell bodies. Immunohistochemistry and double immunofluorescence staining of these cells revealed IgG4 positivity and monotypic lambda-light chain predominance. A portion of these cells were partially positive for CD20, negative for CD3, and somewhat faintly positive for CD138. In addition, serum IgG4 was elevated. Southern blot analysis of the lymph node specimen detected immunoglobulin heavy chain gene rearrangement. The present study indicates that, not only can malignant lymphomas occur in the setting of IgG4-related disease, but IgG4-producing cells can also be neoplastic.

In plain English

This case report from the Department of Pathology at Okayama University, Japan, described a 72-year-old Japanese man who had been followed for 8 years for a right upper lobe lung nodule and an asbestos-related pleural plaque. During follow-up, computed tomography showed masses in both kidneys and multiple enlarged retroperitoneal lymph nodes. In a lymph node biopsy, IgG4 was detected in most of the medium to large plasmacytoid cells. These cells showed only lambda light chains, and a Southern blot test found a monoclonal immunoglobulin heavy chain gene rearrangement. His serum IgG4 was 453 mg/dL (reference range 4.8 to 105), 18% of total IgG (normal less than 6%). The authors diagnosed an IgG4-producing marginal zone B-cell lymphoma, which they described as the first IgG4-producing lymphoma reported. Separately, none of 32 IgG-type multiple myeloma samples they examined was positive for IgG4. The authors concluded that IgG4-producing cells can be neoplastic. The article mentioned asbestos only as part of the patient's history and did not examine any link between asbestos and the lymphoma.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One 72-year-old Japanese man, followed for 8 years for a right upper lobe lung nodule and an asbestos-related pleural plaque; studied at Okayama University, Japan
• Exposure: No asbestos exposure is described (no fiber type, job or dose). The article states only that the patient had an asbestos-related pleural plaque, which had been followed for 8 years. The study's focus was IgG4-producing cells, not asbestos.
• Main results:
  • On follow-up computed tomography, the patient had bilateral kidney masses and multiple enlarged retroperitoneal lymph nodes.
  • The patient's serum IgG4 was 453 mg/dL (reference range 4.8 to 105).
  • In the patient, IgG4 made up 18% of the total IgG fraction (normal, less than 6%).
  • In the patient's lymph node biopsy, IgG4 was detected in most of the plasmacytoid cells.
  • In the biopsy, a lambda light chain monotype was shown, and the Ki-67 labeling index was approximately 30%.
  • In the biopsy specimen, Southern blot analysis detected a monoclonal immunoglobulin heavy chain gene rearrangement.
  • The patient's findings were diagnosed as an IgG4-producing marginal zone B-cell lymphoma.
  • None of the 32 IgG-type multiple myeloma samples the authors examined was positive for IgG4 (data not shown).
  • In the patient, an M-bow was not clearly detected by immunoelectrophoresis, and the result was equivocal.
• The authors' conclusion: The authors concluded that the IgG4-producing cells in this case were neoplastic, and that IgG4-producing cells can be neoplastic.
• Limitations the authors state:
  • The authors stated that the clinicopathological features of this abnormality still need to be clarified by collecting and evaluating more such cases.

Topics

Medical terms in this study, explained in our Lexicon: Fibrosis, Pleural Plaques, Bilateral, Biopsy, Nuclear Pleomorphism, Immunohistochemistry.

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