原發性乾燥症與罹患淋巴癌的風險－個案報告及文獻回顧

Primary Sjögren's Syndrome and the Risk of Sjögren's Syndrome Related Lymphoma - A Case Report and Literature Review

葉宏明(Horng-Ming Yeh)

《台灣老年醫學暨老年學會雜誌》 14卷4期 (2019/11) Pp. 243-250

https://doi.org/10.29461/TGGa.201911_14(4).0005

摘要

口乾、眼乾為老年人常見症狀，老化、藥物、病毒感染等，都會造成。張女士，66歲，眼睛常有反覆性異物感，常覺口渴，沒有反覆或持續性唾液腺體腫大，無雷諾氏症，無皮膚血管炎，肝脾無腫大，也無淋巴結腫大。實驗室檢查發現：抗核抗體陽性，類風濕因子陰性，抗SSA抗體陽性，餘正常。淚腺分泌測試異常，唾液腺核醫掃描陽性。張女士經診斷為原發性乾燥症後，擔心罹患淋巴癌的機會高而焦慮。乾燥症是常見的自體免疫疾病，部分原發性乾燥症病患會演變成淋巴癌，而造成較高死亡率。原發性乾燥症病患發生淋巴癌的危險因子包含唾液腺腫大，淋巴結腫大，雷諾氏症，抗SSA或抗SSB抗體陽性，類風濕因子陽性，單株免疫球蛋白升高，第四補體過低等。

關鍵字

乾燥症 ； 淋巴癌

並列摘要

Dry eye and dry mouth are the frequent complaints of many old people. Sjögren's syndrome can affect any age but the onset occurs most commonly in middle or older age. A 66-year old woman was diagnosed with primary Sjögren's syndrome due to dry eye, dry mouth, positive Schirmer test, and positive anti-SSA antibody. Primary Sjӧgren's syndrome is considered a chronic benign autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands, which leads to gradual destruction and consequent oral and ocular dryness. However, in the setting of Sjögren's syndrome, lymphoma is the most detrimental complication, affecting 5-10% of these patients. Salivary gland enlargement, lymphadenopathy, Raynaud phenomenon, anti-Ro/SSA or/and anti-La/SSB positivity, rheumatoid factor positivity, monoclonol gammopathy, C4 hypocomplementemia are risk factors of developing lymphoma in patients with primary Sjögren's syndrome. Efforts should focus on the identification of predictors that helps facilitate appropriate therapeutic decisions.

並列關鍵字

Sjögren's syndrome ； lymphoma