Relapsing polychondritis (RP) is associated with other rheumatic or autoimmune disease in about 30% of cases; however, an association with malignancy is rare with the exception of myelodysplastic syndrome (MDS).
Herein we report the first case, to our knowledge, of RP following splenic non-Hodgkin lymphoma (NHL), and we have reviewed all the previous well-documented reports that described the cases of RP associated with malignant lymphoma (ML).
Our case and the review of reported cases showed that RP preceded ML in 2 cases, RP occurred after diagnosis and treatment of ML in 2 cases, and RP and ML occurred simultaneously in 1 case. The types of ML encountered were Hodgkin lymphoma, orbital mucosa associated lymphoid tissue type lymphoma, nodal NHL, and splenic NHL. From the frequent association of RP with MDS and, less frequently, with ML, we speculate that some RP cases may occur as a paraneoplastic condition of the concurrent hematological malignancies.