Malignant lymphoma in patients with systemic rheumatic disease (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and dermatomyositis): a clinicopathologic study of 24 Japanese cases.
We conducted clinicopathologic and immunohistochemical analyses of the prevalence of Epstein-Barr virus (EBV) among 24 patients with malignant lymphoma complicating systemic rheumatic diseases. (SRD) These 24 patients included 17 with rheumatoid arthritis (RA), 3 with systemic lupus erythematosus (SLE), 2 with systemic sclerosis (SS), and 2 with dermatomyositis (DM). There were 2 men and 22 women ranging in age from 30 to 86 years (mean: 64 years). The interval between the onset of rheumatic disease and that of malignant lymphomas ranged from 3 months to 35 years (mean: 142 months). The use of immunosuppressive drugs before the onset of malignant lymphoma was recorded in 15 patients. Among them, 5 patients received methotrexate (MTX) therapy. Malignant lymphomas were found at extranodal sites in 9 patients, and the disease was in the advanced stage in 17 patients. Histologic and immunohistochemical studies demonstrated that 18 cases (75%) were B-cell lymphoma (RA=12, SLE=2, SS=2, DM=2), 3 (12.5%) were peripheral T-cell lymphoma (RA=3), and 3 (12.5%) were classical Hodgkin lymphoma (RA=2, SLE=1). As in previous reports, there was an increased frequency of diffuse large B-cell lymphoma (50%) in the present series. Moreover, a majority of the diffuse large B-cell lymphomas exhibited activated B-cell phenotype. EBV-encoded small RNAs (Epstein-Barr early region [EBER]-) and/or LMP-1+tumor cells were identified in only 3 cases of classical Hodgkin lymphomas. Our findings suggested EBV-associated lymphoma comprised only a small fraction of all non-Hodgkin's lymphomas in the general SRD patient population.
Kojima
Masaru
M
Department of Pathology and Clinical Laboratories, Gunma Cancer Center Hospital, Ohta, Dokkyo University School of Medicine, Mibu, Japan.
Itoh
Hideaki
H
Shimizu
Kazuhiko
K
Saruki
Nobuhiro
N
Murayama
Kayoko
K
Higuchi
Keiko
K
Tamaki
Yoshio
Y
Matsumoto
Morio
M
Hirabayashi
Kaoru
K
Igarishi
Seiji
S
Masawa
Nobuhide
N
Nakamura
Shigeo
S
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018951:Antigens, CD20 / Q000032:analysis; D001172:Arthritis, Rheumatoid / Q000150:complications / Q000473:pathology; D003882:Dermatomyositis / Q000150:complications / Q000473:pathology; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics; D006689:Hodgkin Disease / Q000150:complications / Q000473:pathology / Q000821:virology; D006801:Humans; D007150:Immunohistochemistry; D007564:Japan; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000473:pathology; D008223:Lymphoma / Q000150:complications* / Q000473:pathology / Q000821:virology; D016393:Lymphoma, B-Cell / Q000150:complications / Q000473:pathology / Q000821:virology; D016399:Lymphoma, T-Cell / Q000150:complications / Q000473:pathology / Q000821:virology; D008297:Male; D008875:Middle Aged; D012367:RNA, Viral / Q000032:analysis; D012216:Rheumatic Diseases / Q000150:complications* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications / Q000473:pathology
D018951:Antigens, CD20; D012367:RNA, Viral