Cognitive Dysfunction as an Initial Manifestation of Rheumatoid Arthritis-associated Intravascular Large B-cell Lymphoma.

A 58-year-old Japanese woman with rheumatoid arthritis (RA) presented with the sudden onset of cognitive dysfunction. A random skin biopsy revealed intravascular large B-cell lymphoma (IVLBCL), which resolved spontaneously with methotrexate withdrawal. However, four months later, the disease relapsed with liver injury. After completion of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone therapy, both RA and IVLBCL remained in remission for two years. Among the pathological subtypes of RA-associated lymphoproliferative diseases, reports on IVLBCL are limited, and little is known about its clinical course. Our literature review summarizes the clinical course and mortality of 11 patients with RA-IVLBCL.