title: Clinical characteristics of patients with lymphoproliferative neoplasms in the setting of systemic autoimmune diseases.
abstract: Clinical features of 40 lymphoproliferative neoplasm patients in the setting of systemic autoimmune diseases managed in the Clinic of Hematology during 1994-2006 were analyzed retrospectively. The classification of systemic autoimmune disease patients was as follows: 15 systemic lupus erythematosus--SLE, 11 rheumatoid arthritis--RA, 12 Sjögren's syndrome--SS, 1 scleroderma, and 1 dermatomyositis. Patients comprised 31 women and 9 men of mean age 55 years (range 33-76). Systemic autoimmune diseases preceeded the development of lymphoproliferative neoplasms in 37/40 (92.5%) patients. Mean latency period between the onset of systemic autoimmune diseases and lymphoproliferative neoplasms occurrence was significantly longer in RA (113 months) than in SLE (75 months) and SS patients (65 months)--P < 0.05. The most frequent lymphoproliferative neoplasms were non-Hodgkin's lymphoma--NHL (35/40; 88%), diffuse large B-cell lymphoma (DBCL)--12 (34%), follicular lymphoma (FC)--7 (20%), small lymphocytic (SL), and marginal zone lymphoma (MZL)--5 (14%) each. The primary site of NHL was extranodal in 18/35 (51.5%) cases. Advanced disease on diagnosis (III + IV clinical stages), constitutional symptoms, and bulky disease were diagnosed in 27/35 (77%), 26/35 (74%), and 3/35 (8.5%) patients, respectively. The overall survival (OS) was as follows (months): DBCL-12, FC-63, SLL-60, and MZL-48. There was no association between the lymphoproliferative neoplasm histological subtype and the systemic autoimmune diseases type or antirheumatic treatment P > 0.05. Our findings are in line with earlier reports showing a high proportion of patients with advanced disease, constitutional symptoms, extranodal manifestations, high grade histology, and low OS in the systemic autoimmune diseases setting.
authors.affiliation: Clinic of Hematology, Clinical Center of Serbia, Dr. Koste Todorovica 2, 11000 Belgrade, Republic Serbia. nvukovic@eunet.rs
authors.forename: Nada
authors.initials: N
authors.lastname: Suvajdzic
authors.forename: Predrag
authors.initials: P
authors.lastname: Djurdjevic
authors.forename: Milena
authors.initials: M
authors.lastname: Todorovic
authors.forename: Maja
authors.initials: M
authors.lastname: Perunicic
authors.forename: Roksanda
authors.initials: R
authors.lastname: Stojanović
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Novkovic
authors.forename: Biljana
authors.initials: B
authors.lastname: Mihaljevic
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications* / Q000401:mortality / Q000473:pathology*; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies