Increased Risk of Lymphoma in Sicca Syndrome

TLDR

The study evaluated cancer risk in 136 women with sicca syndrome followed at NIH. Seven patients developed non‑Hodgkin lymphoma (43.8‑fold higher than expected) and three Waldenström macroglobulinemia cases, while other cancers matched population rates; parotid enlargement, splenomegaly, and lymphadenopathy identified a subgroup with markedly increased lymphoma risk.

Abstract

The risk of cancer was ascertained in 136 women with sicca syndrome followed at the National Institutes of Health (NIH). Seven patients developed non-Hodgkin's lymphoma from 6 months to 13 years after their first admission to NIH. This was 43.8 times (P < 0.01) the incidence expected from the rates of cancer prevailing among women of the same age range in the general population during this time. In addition, three cases of Waldenström's macroglobulinemia occurred in this study group. Eight patients developed cancers other than lymphoma, similar to the number expected based on the rates prevailing in the general population. Patients with a history of parotid enlargement, splenomegaly, and lymphadenopathy had an increased risk of lymphoma. These clinical conditions did not appear to be early manifestations of undiagnosed lymphoma but rather seemed to identify a subgroup of patients with sicca syndrome with marked lymphoid reactivity, who had a particularly high risk of subsequently developing lymphoma.