Grosbois B, Jego P, Leblay R
Service de médecine interne G, Hôpital Sud, Rennes, France.
Rev Med Interne. 1998 May;19(5):319-24. doi: 10.1016/s0248-8663(98)80101-x.
This review is aimed at defining the frequency, anatomical and clinical presentation, pathogenesis, predictive factors and treatment of malignant lymphoproliferative diseases occurring in the course of Sjögren's syndrome.
The frequency of non-Hodgkin's lymphoma (NHL) is estimated to be about 7%. Other malignant lymphoproliferative diseases (Waldenstrom's macroglobulinemia, multiple myeloma, Hodgkin's disease) are rarely observed. NHL is most frequently extranodal (affecting the salivary glands, stomach, lung, etc) in low grade malignancy (MALT lymphoma [mucosa associated lymphoid tissue]). The pathogenesis of NHL in Sjögren's syndrome is a multi-step process, including B cell monoclonal proliferation, oncogenic and/or infectious agents, and/or cytokines. Various predictive factors such as persistent enlargment of parotid glands, adenopathy, splenomegaly, mixed cryoglobulinemia, monoclonal gammopathy, suggest potential lymphoma evolution. The treatment of Sjögren's syndrome-associated NHL depends on the type of lymphoma. Moreover, in patients with low-grade lymphoma therapeutical strategies varies according to the stage and evolution of the disease.
Future prospective longitudinal studies should permit to define the best criteria for malignant transformation and to improve therapeutical strategies.
本综述旨在明确干燥综合征病程中发生的恶性淋巴增殖性疾病的发生率、解剖学及临床表现、发病机制、预测因素及治疗方法。
非霍奇金淋巴瘤（NHL）的发生率估计约为7%。其他恶性淋巴增殖性疾病（华氏巨球蛋白血症、多发性骨髓瘤、霍奇金病）则很少见。NHL在低级别恶性肿瘤（黏膜相关淋巴组织淋巴瘤 [MALT淋巴瘤]）中最常发生于结外（累及唾液腺、胃、肺等）。干燥综合征中NHL的发病机制是一个多步骤过程，包括B细胞单克隆增殖、致癌和/或感染因子以及细胞因子。各种预测因素，如腮腺持续肿大、淋巴结病、脾肿大、混合性冷球蛋白血症、单克隆丙种球蛋白病，提示可能发生淋巴瘤演变。干燥综合征相关NHL的治疗取决于淋巴瘤的类型。此外，对于低级别淋巴瘤患者，治疗策略会根据疾病的阶段和演变而有所不同。
未来的前瞻性纵向研究应有助于确定恶性转化的最佳标准并改进治疗策略。
