[Characteristics of patients with primary Sjögren's syndrome and non-Hodgkin's lymphoma: analysis of 9 cases].
OBJECTIVE
To characterize the clinical patterns of expression, laboratory serologic parameters and lymphomatous histological characteristics in patients with primary Sjögren's syndrome (pSS) who subsequently developed non-Hodgkin's lymphoma (NHL).


METHODS
The authors analyzed 9 pSS patients (8 females, 1 male) who developed NHL. Five patients had received glucocorticoids, four of whom had received at least one immunosuppressive drugs (methotrexate, glucosidorum tripterygll totorum, cyclophosphamide and imuran). A protocol form was used to record the main characteristics of pSS and NHL.


RESULTS
Eight patients fulfilled the American-European Consensus Criteria (AECC). The main SS manifestations were painless parotid enlargement (n = 7), six of whom were unilateral; the main immunologic features were positive rheumatoid factor (RF) in all examined patients and hyperimmunoglobulinemia (n = 7). The main manifestations of NHL were splenomegaly (n = 7) and lymphadenopathy (n = 5). The main histological subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma (n = 4) and diffuse large B cell lymphoma (n = 2). None of the patients with MALT lymphoma had a nodal primary location. Eight patients had an extranodal primary location, most frequently in salivary gland (n = 4) and lung (n = 4).


CONCLUSIONS
Patients with pSS and NHL are clinically characterized by a high frequency of painless unilateral parotid enlargement, splenomegaly, lymphadenopathy, an immunologic pattern dominated by the presence of high-titer RF and hyperimmunoglobulinemia, a predominance of MALT lymphomas and an elevated frequency of primary extranodal involvement.
Department of Medical Oncology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Ning
N
Jia
Fu-Lin
FL
Tang
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000150:complications / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications / Q000473:pathology*; D055815:Young Adult