Intravascular large cell lymphoma mimicking central nervous system vasculitis in a patient with rheumatoid arthritis (RCD code: VI-2C.2)
Increased incidence of lymphomas in patients with rheumatoid arthritis has been reported and may be associated with several factors, including genetic factors, chronic inflammation, and immunosuppressive treatment. Intravascular large cell lymphoma (ILCL) is a rare subtype of large cell lymphoma with undetectable lymphoma cells in peripheral blood and without any extravascular localisation. Diag- nosis is often delayed because bone marrow, spleen, and cerebrovascular fluid may not be involved and there are no specific laboratory studies for intravascular large cell lymphoma. We report the case of a 63-year-old woman with a 10-year history of seropositive rheumatoid arthritis and intravascular large cell lymphoma recognised at autopsy. The patient presented with progressive central nervous system-related symptoms mimicking vasculitis without lymphadenopathy or bone marrow involvement. Malignant lymphoproliferative disorders should be considered in RA patients with varying clinical presentation, even in the absence of lymphadenopathy and bone marrow involvement. JRCD 2018; 4 (1):
Eriksson JK, Neovius M, Ernestam S, et al. Incidence of rheumatoid arthritis in Sweden: A nationwide population-based assessment of incidence, its determinants, and treatment penetration. Arthritis care res 2013: 65: 870–878.
Doran MF, Pond GR, Crowson CS, et al. Trends in incidence and mortality in rheumatoid arthritis in Rochester, Minnesota, over a forty-year period. Arthritis Rheum 2002; 46: 625–631.
Turesson C, O’Fallon WM, Crowson CS, et al. Extra-articular disease manifestations in rheumatoid arthritis: incidence trends and factors over 46 years. Ann Rheum Dis 2003: 62: 722–727.
Medina LD, Hirshberg L, Taylor MJ, et al. Rates of neuropsychological dysfunction in fibromyalgia and rheumatoid arthritis: An automated clinical rating approach. J Clin Rheumatol 2018; doi:10.1097/RHU.0 000 000 000 000 837;
[Epub ahead of print].
Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthrutus rheum 1988; 31: 315–324.
Franklin J, Lunt M, Bunn D, et al. Incidence of lymphoma in a large primary care derived cohort of cases of inflammatory polyarthritis. Ann Reum Dis 2006: 65: 617–622.
Fragkioudaki S, Mavragani CP, Moutsopoulos HM. Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use. Medicine (Baltimore). 2016; 95:e3766.
Ferreri AJ, Campo E, Seymour JF, et al. Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the “cutaneous variant”. Br J Haematol 2004;127:173–183.
Shimada K, Matsue K, Yamamoto K, et al. Retrospective analysis of intravascular large B-cell lymphoma treated with rituximab-containing chemotherapy as reported by the IVL study group in Japan. J Clin Oncol 2008; 26: 3189–3195.
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