Lymphoproliferative Disorders Associated with Sjoegren's Syndrome.
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Accession number;02A0208863
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| Title;Lymphoproliferative Disorders Associated with Sjoegren's Syndrome. |
| Author;
YANO TAKAHIRO
(Yokohamashiminbyoin Naika)
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Journal Title;Prog Med
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Journal Code:F0664B
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ISSN:0287-3648
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VOL.22;NO.1;PAGE.70-75(2002)
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| Figure&Table&Reference;FIG.1, TBL.1, REF.21 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;Patients with Sjoegren's syndrome are known to be 44 times more likely to develop malignant lymphoma (SS-ML) than a comparable normal population. The majority of SS-MLs are CD5-/CD10-/CD20+ marginal zone B-cell lymphoma of MALT type, typically occurring in the salivary gland, where local chronic stimulation by autoantigens such as IgG results in benign, but "prelymphomatous" lymphoepithelial lesions and consequently the establishment of polyclonal B cell populations secreting rheumatoid factor. These cells acquire several sequential molecular alterations to become monoclonal or oligoclonal, ending up to be SS-ML with systemic dissemination. Preferential usage of particular immunoglobulin VH gene segments by the SS-ML cells and on going somatic mutations in the segments strongly suggest that SS-ML may be derived from post germinal center B cells and the persistent stimulation of antigen receptors by autoantigens may be involved in the growth of salivary gland SS-ML. Recent cytogenetic analyses have revealed that the majority of SS-MLs have trisomy 3 and, less frequently, t (11;18) (q21;q21) which leads to the production of a MALT-lymphoma-specific novel chimeric protein c-IAP 2/MALT1. The chimeric protein is believed to exert its lymphomagenic activity through NF-.KAPPA.B activation or possibly by altering the programmed cell death mechanisms. Treatment of SS-MLs has not been established but anti-CD20 monoclonal antibody (rituximab), purine analogues or non-ablative stem cell transplant could be a new choice of treatment modalities, contributing to the eventual improvement of long-term clinical outcome of the patients. (author abst.) |
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