Primary Sjoegren's syndrome presenting as unconsciousness associated with IgA-.LAMBDA. M-protein.

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  • ＩｇＡ‐λ型Ｍ蛋白血症を合併し，意識消失発作を呈した原発性シェーグレン症候群

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Abstract

We describe the case of a 43-year-old woman who presented with primary Sjögren's syndrome (SS) which manifestated as unconsciousness due to M protein. A diagnosis of SS was made based on a ten-year history of dryness, a Shirmer test, and the histological findings of labial biopsy. A rouleaux formation was observed and serum protein electrophoresis revealed a monoclonal spike of 4.0gm/dl in the γ-region, which was characterized as IgA-λ. Biopsy of the minor salivary glands showed marked polyclonal lymphoproliferation with lymphoid follicles, including both T cells and B cells as revealed by immunohistological staining. Therefore, the patient had a lymphoproliferative lesion of the minor salivary glands, which is also known as pseudolymphoma. We conclude that fainting associated with marked M protein may be manifestation of SS. Such cases should be followed carefully since the subsequent neoplastic transformation of pseudolymphomas have been previously reported.

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