Primary Sj?gren syndrome complicated with lymphoma: a report of 10 cases with literature review

VernacularTitle:原发性干燥综合征合并淋巴瘤十例临床分析
Author: Dan MA ¹ ; Baoqi YANG ; Liyun ZHANG
Author Information

1. 山西白求恩医院风湿免疫科，太原　030032
Keywords: Sj?gren syndrome; Lymphoma
From: Chinese Journal of General Practitioners 2021;20(11):1166-1170
CountryChina
Language:Chinese
Abstract: Clinical characteristics, treatments and prognosis of 10 primary Sj?gren syndrome (pSS) patients with lymphoma were retrospectively analyzed. All 10 patients were female. Dry mouth, dry eyes and lymph node enlargement were common. Laboratory tests showed positive antinuclear antibody(ANA), anti-SSA antibody, anti-SSB antibody and rheumatoid factor(RF); lower lymphocyte counts and high IgG level. All patients were not treated regularly after pSS was diagnosed; 9 cases of non-Hodgkin lymphoma (NHL) and 1 case of Hodgkin lymphoma (HL) were finally diagnosed. The main histological types of NHL were mucosa-associated lymphoid tissue marginal zone lymphoma (MALT) and diffuse large B cell lymphoma (DLBCL). Both intranodal and extranodal involvement was common. Patients were followed up for 1-144 months, 8 patients were treated with R-CHOP regime, CHOP regime or rituximab; 5 patients were stable, 4 patients died, and 1 case lost to follow up. Patients with pSS are closely associated with an enhanced risk of lymphoma, especially NHL. The clinical manifestations were complicated and varied.