sjogren病:淋巴瘤和骨髓瘤的风险

Gaetane Nocturne
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引用次数: 0

摘要

Sjögren病(pSS)患者发生淋巴样血病的风险增加。淋巴瘤的发展是SJS最严重的并发症。5%到10%的患者会出现这种情况。pSS的淋巴瘤通常是结外边缘区非霍奇金b细胞淋巴瘤,起源于粘膜相关淋巴组织。它们经常发生在唾液腺,唾液腺是这种自身免疫性疾病的靶器官。pSS淋巴瘤的发展是一个多步骤的过程,与B细胞的慢性抗原刺激有关。本文回顾了pSS淋巴瘤的主要预测因素以及实际的诊断和治疗管理。此外,它回顾了pSS中骨髓瘤的风险,这是一种鲜为人知的并发症,几乎和淋巴瘤一样常见。
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Maladie de Sjögren : risque de lymphome et de myélome

Patients with Sjögren's disease (pSS) are at increased risk of lymphoid hemopathy. The development of lymphoma is the most serious complication of SJS. It occurs in 5 to 10% of the patients. Lymphomas in pSS are most often extra-nodal marginal zone non-Hodgkin's B-cell lymphomas that develop from the mucosa associated lymphoid tissue. They frequently occur in the salivary glands, which are the target organ of this autoimmune disease. The development of lymphoma in pSS is a multistep process related to chronic antigenic stimulation of B cells. This update reviews the main predictive factors for lymphoma in pSS and also the practical diagnostic and therapeutic management. In addition, it reviews the risk of myeloma in pSS, a much lesser known complication that is almost as common as lymphoma.

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