[Primary Sjögren's syndrome presenting as unconsciousness associated with IgA-lambda M-protein].
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 4.0 gm/dl in the gamma-region, which was characterized as IgA-lambda. 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.
Nagasaki
M
M
First Department of Internal Medicine, Nara Medical University.
Fujimoto
T
T
Umemura
Y
Y
Nakamura
S
S
Dohi
K
K
D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007146:Immunoglobulin lambda-Chains / Q000097:blood*; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications*; D019310:Pseudolymphoma / Q000150:complications; D012466:Salivary Gland Diseases / Q000150:complications; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis; D014474:Unconsciousness / Q000209:etiology*
D007070:Immunoglobulin A; D007146:Immunoglobulin lambda-Chains