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Splenic marginal zone B-cell lymphoma associated with primary Sjögren’s syndrome

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Abstract

We describe a 67-year-old woman with primary Sjögren’s syndrome who developed a splenic marginal zone B-cell lymphoma, and complained of a dry mouth and Raynaud’s phenomenon. She had splenomegaly, swollen lymph nodes and monoclonal IgM-κ cryoglobulin. After splenectomy, Raynaud’s phenomenon had improved with the decrease of cyroglobulin. This is the first case of primary Sjögren’s syndrome with splenic marginal zone B-cell lymphoma to be reported.

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Abbreviations

MALT:

Marginal zone lymphoma arising in the mucosa-associated lymphoid tissue

MZL:

Marginal zone lymphoma

SMZL:

Splenic marginal zone lymphoma

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Acknowledgements

We thank Dr Kenji Notohara (Department of Pathology, Okayama University Medical School) for critical work of immunostaining and pathological diagnosis. We also thank Dr Susumu Sugai (Hematology Immunology Section, Internal Medicine, Kanazawa Medical University), Dr Masami Nagai (First Department of Internal Medicine, Kagawa Medical University) and Dr Shuji Hamazaki (Department of Pathology, Okayama University Hospital) for useful advice.

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Correspondence to Susumu Nishiyama.

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Nishiyama, S., Miyawaki, S. Splenic marginal zone B-cell lymphoma associated with primary Sjögren’s syndrome. Clin Rheumatol 23, 242–245 (2004). https://doi.org/10.1007/s10067-003-0838-7

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  • DOI: https://doi.org/10.1007/s10067-003-0838-7

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