Lymphomatous Polyarthritis in Patients with Peripheral T-Cell Lymphoma

Abstract

Direct involvement of the joints is a rare complication of malignant lymphoma and lymphoma cells in synovium or synovial fluid have been characterized in only a very few cases. We report two cases of CD4-positive, HTLV-I-negative peripheral T-cell lymphomas that manifested polyarthritis infiltrated with lymphoma cells which we further characterized. Patient 1, with a prior 7-year history of cutaneous T-cell lymphoma (mycosis fungoides) and polyarthralgia, developed pain and swelling in the right knee joint and right femoral region. Patient 2 was initially diagnosed with immunoblastic lymphadenopathy, later rediagnosed as the prodromal stage of T zone lymphoma. Seven years later she developed skin eruptions, cervical lymph node swelling, polyarthritis, and pleural effusion. Synovial fluid analysis in both cases showed predominant CD3+ or cytoplasmic CD3+, CD4+, and CD8- atypical lymphoid cell infiltration. In both cases the T-cell receptor beta and gamma chains were rearranged in the synovial fluid mononuclear cells. Analysis of these two cases and a review of the literature suggest that lymphoma cell infiltration of synovium occurs preferentially in patients with CD4+ peripheral T-cell lymphoma.

Clonal identity between skin and synovial tissue in a case of mycosis fungoides with polyarthritis

“…Molecular analysis of synovial fluid cells of patients with MF and polyarthritis has been performed and revealed monoclonal TCR gene rearrangements. 6,7,12 As such, the finding of an (oligo)clonal T-cell population in the synovial tissue of patients with RA or other inflammatory rheumatic diseases even in absence of a T-cell malignancy can occur. [20][21][22] Tachibana et al 12 reported the detection of an identical clone in synovial fluid and LN by SB analysis in a patient with T-zone lymphoma.…”

“…24 In the presence of MF, direct invasion of the synovium by malignant clonal CD4 + cells has been described to result in a reactive cellular inflammatory response by the synovial tissue, in which the patients had a symmetric nonerosive polyarthritis. 6,7,12 Paraneoplastic arthritis is rare, of unknown origin, and has been reported in different types of neoplasms, including acute lymphoblastic leukemia. [25][26][27] Seleznick et al 5 suggest that reactive phenomena directed to neoplastic T-cell proliferation may play a role in MF-associated polyarthritis.…”

“…In late-stage MF, extracutaneous dissemination of monoclonal cell populations can be easily detected by molecular T-cell receptor (TCR) gene rearrangement analysis. 1,2 Polyarthritis in the presence of malignant cutaneous T-cell lymphoma has been described before, [3][4][5][6][7][8][9][10][11][12] but this combination of entities still is a rare phenomenon. However, defining the possible cause of the arthritis or polyarthritis is important, even when the patient already has MF.…”

“…If infiltration of malignant clonal T lymphocytes into the synovium was responsible for the arthritis, the staging of MF and, thereby, the treatment would be altered. 3,7,12 Typically, such polyarthritis is rheumatoid factorenegative, nonerosive, symmetric, and mimics rheumatoid arthritis (RA). 9,11 If RA has developed in the absence of extracutaneous localization of MF, both disease entities can be treated separately.…”

Paraneoplastic arthritis in a patient with Sézary syndrome

“…The exact etiology is unknown. In some cases, malignant T cells were detected in joints and bones, while in other cases there is no evidence of direct invasion . There is also a possibility that polyarthritis develops independently of CTCL.…”