Rheumatology: Current Research
Open Access

Abstract

Risk of Lymphoproliferative Disorders in Spondyloarthritis during Treatment with Methotrexate: A Case Report and Literature Review

Akihiro Nakamura, Tomoya Miyamura, Brian Wub and Eiichi Suematsu

Unlike rheumatoid arthritis, risks of lymphoproliferative disorders (LPDs) in spondyloarthritis (SpA) are not well established. Recent studies suggest that the overall risk of lymphoma does not seem to be increased compared to the general population, however, SpA patients taking disease-modifying anti-rheumatic drugs including methotrexate (MTX) may link to higher occurrence of LPDs. We herein report a 40-year-old man diagnosed with non-radiographic axial SpA according to the Assessment of Spondyloarthritis International Society (ASAS) classification criteria. While initiation of oral MTX and celecoxib treatments appeared to gradually improve symptoms, cervical lymphadenopathy appeared accompanied by fever and deteriorated arthritis following 13 months. Laboratory results showed increased serum levels of inflammatory markers, liver transaminases, soluble interleukin-2 receptor and ferritin as well as thrombocytopenia in combination with hepatosplenomegaly detected by Computed Tomography. Furthermore, hemophagocytosis also existed in bone marrow. Diagnostic lymph node biopsy pathologically revealed diffuse large B-cell lymphoma (DLBCL), resulting in a diagnosis of DLBCL concomitant with lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH). Since the DLBCL persisted following four weeks of MTX discontinuation, chemotherapy was initiated and finally resulted in remission. To our knowledge, there is no report on LPDs concomitant with LA-HLH in a patient with SpA during MTX treatment. This suggestive case supports the recent estimation in which the risk of LPDs in SpA patients is increased and also demonstrates the importance of chemotherapeutic treatment for LPDs concomitant with LA-HLH.