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[与原发性干燥综合征相关的恶性淋巴瘤]

[Malignant lymphoma associated with primary Sjögren's syndrome].

作者信息

Wang Li, Zhao Yan, Zhang Feng-chun

机构信息

Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 100032, China.

出版信息

Zhonghua Yi Xue Za Zhi. 2010 Oct 26;90(39):2773-5.

PMID:21162916
Abstract

OBJECTIVE

To address the clinical features of malignant lymphoma (ML) occurring in primary Sjögren's syndrome (pSS), understand their similarities and differences and explore their risk factors.

METHODS

Medical records of 17 cases of pSS/ML inpatients admitted to our hospital from January 1980 to August 2010 were systemically reviewed. And 163 cases were randomly selected as controls from 4485 pSS/nML inpatients at our hospital during the same period.

RESULTS

There were 14 females and 3 m ales with the diagnostic age of pSS at (53 ± 13) years old. And the disease duration was (8 ± 9) years. There was no significant difference between two groups in three general aspects (P > 0.05). The gap between pSS and ML was (4 ± 4) years. Several significant differences existed between two groups (P < 0.05) in clinical features and laboratory findings: lymphadenopathy, parothydomegalia and leucopenia. The pathologic categories of ML in pSS/ML included NHL (n = 15) (14 cases of B-cell origin), HL (n = 1) and lymphosarcoma (n = 1).

CONCLUSION

During the course of pSS, the occurrence of such manifestations as lymphadenopathy, parothydomegalia and leucopenia, etc.tends to have a complication of ML with a dominance of NHL. And a worse prognosis is expected.

摘要

目的

探讨原发性干燥综合征(pSS)合并恶性淋巴瘤(ML)的临床特点,了解两者异同并探索其危险因素。

方法

系统回顾1980年1月至2010年8月我院收治的17例pSS/ML住院患者的病历资料。同期从我院4485例pSS/非ML住院患者中随机选取163例作为对照。

结果

pSS诊断年龄为(53±13)岁,共14例女性、3例男性,病程为(8±9)年。两组在三个总体方面无显著差异(P>0.05)。pSS与ML的间隔时间为(4±4)年。两组在临床特征和实验室检查结果方面存在一些显著差异(P<0.05):淋巴结病、腮腺肿大和白细胞减少。pSS/ML中ML的病理类型包括非霍奇金淋巴瘤(NHL,n=15)(14例为B细胞起源)、霍奇金淋巴瘤(HL,n=1)和淋巴肉瘤(n=1)。

结论

在pSS病程中,出现淋巴结病、腮腺肿大和白细胞减少等表现时,易合并ML,且以NHL为主,预后较差。

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