Abstract :
An association between rheumatic disorders and the occurence of lymphoproliferative diseases was proposed many years ago (Hench PK 1962; Lea AJ 1964; Miller DG 1967). Lymphoid neoplasms occuring in rheumatisms resemble those in immunocompromised patients and are almost all of B-lymphocyte lineage. Neoplastic cells nearly always contain Epstein-Barr virus (EBV). An impairment of the immune system occuring in rheumatic patients could underlie this association. It could depend on the rheumatic disorder or the treatment given, or a combination of these factors. At present it is difficult to demonstrate whether rheumatic patients are at increased risk of developing certain types of lymphoma. This results from the wide spectrum of lymphoproliferative disorders which may be detected and from the numerous precipitating factors which may be involved in the development of these neoplasms. In fact, apart from Sjogrenʹs Syndrome, which associates with malignant lymphomas of salivary glands and neighboring lymphnodes there has not been a specific type of lymphoid neoplasm occurring in a specific rheumatic disorder. In Sjogrenʹs Syndrome the relative risk of developing lymphomas seems to be 40 times greater than the matched control population (Fox R. I. 1992). Immunopathological studies underline a high frequency of low grade salivary gland B cell lymphomas (Weiss L.M. 1991; Shin S.S. 1991; Ortiz-Hidalgo C 1992). On the other hand, recent reports (Ellmon M. H. 1991; Zijlmans J. M. J. M. 1992; Kamel O. W.1993; Kamel O. W. 1994) have demonstrated that some patients with rheumatic disease may show a subset of lymphoma which resembles features of immunosuppression associated lymphoproliferative disorders. Most of these patients suffer from long-standing rheumatoid arthritis. Lymphoid neoplasms may develop spontaneously or during immunosuppressive therapy (Shiroky J. B. 1991; Kingsmore SF 1992; Ellmon M. H.1991), sometimes they spontaneously regress with therapy withdrawal (Shivoky J. B. 1991; Kamel O. W. 1993), in some cases show the features of lymphoproliferations occuring in immunocompromised patients (Kamel O W 1994). Thus, further researches could better define the steps involved in the phases of lymphoma development. Moreover, the early detection of rheumatic patients at the risk of immunosoppression-associated lymphoma could allow a careful monitoring of these possible complications described during immunosuppressive therapies.
