[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.
Jia
Ning
N
Department of Medical Oncology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Tang
Fu-Lin
FL
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