Abstract
The case of a 49-year-old man with peripheral T-cell lymphoma arising in Behçet disease (BD) is reported. A diagnosis of incomplete BD was made, and the patient was treated with immunosuppressive agents for 9 months. A left perirenal mass emerged, and a computed tomography-guided needle biopsy of the tumor revealed the infiltration of small- and medium-sized lymphoma cells.The cells were positive for CD3, CD8, CD45RO, CD43, granzyme B, and T-cell intracellular antigen-1.A diagnosis of non-Hodgkin’s lymphoma (diffuse medium, T-cell) was made.A left orbital mass also appeared. Standard combination chemotherapy diminished the perirenal and orbital lesions.Lymphoma cell infiltration in the esophagus was detected after chemotherapy, and the patient died of massive bleeding from the gastrointestinal tract. Non-Hodgkin’s lymphoma is rarely associated with BD, and only 7 cases have been reported in the literature.We have summarized the published case reports of malignant lymphoma arising in BD.To our knowledge, this case report is the first to describe cytotoxic T-cell lymphoma arising in Behçet disease.
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Sakane T, Takeno M, Suzuki N, Inaba G. Behçet’s disease. N Engl J Med. 1999;341:1284–1291.
Direskeneli H. Behçet’s disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum Dis. 2001;60:996–1002.
Harris NL, Jaffe ES, Diebold J, et al. The World Health Organization classification of hematological malignancies report of the Clinical Advisory Committee Meeting, Airlie House,Virginia, November 1997. Mod Pathol. 2000;13:193–207.
Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84:1361–1392.
Jaffe ES, Krenacs L, Kumar S, Kingma DW, Raffeld M. Extranodal peripheral T-cell and NK-cell neoplasms. Am J Clin Pathol. 1999; 111(suppl 1):S46-S55.
Mizushima Y. Recent research into Behçet’s disease in Japan. Int J Tissue React. 1988;10:59–65.
Cengiz M, Altundag MK, Zorlu AF, Gullu IH, Ozyar E, Atahan IL. Malignancy in Behçet’s disease: a report of 13 cases and a review of the literature. Clin Rheumatol. 2001;20:239–244.
Black KA, Zilko PJ, Dawkins RL, Armstrong BK, Mastaglia GL. Cancer in connective tissue disease. Arthritis Rheum. 1982;25:1130–11333.
Barnes BE, Mawr B. Dermatomyositis and malignancy: a review of the literature. Ann Intern Med. 1976;84:68–76.
Canoso JJ, Cohen AS. Malignancy in a series of 70 patients with systemic lupus erythematosus. Arthritis Rheum. 1974;17:383–390.
Hamza MH. Cancer complicating Behçet’s disease treated with chlorambucil. Ann Rheum Dis. 1986;45:789.
Celik I, Altundag K, Erman M, Baltali E. Cyclophosphamideassociated carcinoma of the urinary bladder in Behçet’s disease. Nephron. 1999;81:239.
Harada K, Ohtsuru K, Nakayama K, Takagi S, Sugita Y, Torigoe R. Intracranial primary malignant lymphoma following Behçet’s disease: case report [in Japanese]. No To Shinkei. 1992;44:1029–1033.
Muramatsu M, Kotake S, Yoshikawa K, Sasamoto Y, Matsuda H, Yamawaki S. The development of malignant rhabdoid tumor in a patient with Behçet’s disease treated with ciclosporin. Graefes Arch Clin Exp Ophthalmol. 1998;236:798–799.
Sakane T, Kotani H, Takada S, Tsunematsu T. Functional aberration of T cell subsets in patients with Behçet’s disease. Arthritis Rheum. 1982;25:1343–1351.
Kanavaros P, Boulland ML, Petit B, Arnulf B, Gaulard P. Expression of cytotoxic proteins in peripheral T-cell and natural killer-cell (NK) lymphomas: association with extranodal site, NK or Tgammadelta phenotype, anaplastic morphology and CD30 expression. Leuk Lymphoma. 2000;38:317–326.
Yamashita Y, Yatabe Y, Tsuzuki T, et al. Perforin and granzyme expression in cytotoxic T-cell lymphomas.Mod Pathol. 1998;11:313–323.
Ranheim EA, Jones C, Zehnder JL, Warnke R, Yuen A. Spontaneously relapsing clonal, mucosal cytotoxic T-cell lymphoproliferative disorder: case report and review of the literature. Am J Surg Pathol. 2000;24:296–301.
Egawa N, Fukayama M, Kawaguchi K, et al. Relapsing oral and colonic ulcers with monoclonal T-cell infiltration: a low grade mucosal T-lymphoproliferative disease of the digestive tract. Cancer. 1995;75:1728–1733.
Ueda M, Kobayashi Y, Yoshimori K, et al. Epstein-Barr virusinfected T-cell malignancy in an adult patient with Behçet’s disease- like symptoms [in Japanese]. Rinsho Ketsueki. 1997;38:657–6622.
Kaneko H, Hojo Y, Nakajima H, Okamura A, Fukase M. Behçet syndrome associated with nasal malignant lymphoma: report of an autopsy case. Acta Pathol Jpn. 1974;24:141–150.
Moulonguet-Michau I, Blanc F, Cavelier-Balloy B, Brice P, Flageul B, Civatte J. Angiocentric lymphoma in Behçet’s disease [in French]. Ann Dermatol Venereol. 1990;117:885–887.
Kawamoto S, Terada H, Niikura H, Kumasaka T, Saiki S. Hodgkin’s disease associated with Behçet’s disease [in Japanese]. Rinsho Ketsueki. 1992;33:211–215.
Abe T, Yachi A, Yabana T, et al. Gastric non-Hodgkin’s lymphoma associated with Behçet’s disease. Intern Med. 1993;32:663–667.
Yamamoto T, Tamura M, Hamauzu T, et al. Intestinal Behçet’s disease associated with non-Hodgkin’s lymphoma. J Gastroenterol. 1997;32:241–245.
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Katsura, Y., Suzukawa, K., Kojima, H. et al. Cytotoxic T-Cell Lymphoma Arising in Behçet Disease. Int J Hematol 77, 282–285 (2003). https://doi.org/10.1007/BF02983787
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DOI: https://doi.org/10.1007/BF02983787