基础医学与临床 ›› 2017, Vol. 37 ›› Issue (2): 234-237.

• 临床园地 • 上一篇    下一篇

多发性肌炎/皮肌炎合并淋巴瘤的临床特点分析

杨华夏1,郭静波2,肖欣悦1,张烜3   

  1. 1. 北京协和医院
    2. 中国人民解放军256医院
    3. 中国医学科学院 北京协和医学院 北京协和医院
  • 收稿日期:2015-12-31 修回日期:2016-04-03 出版日期:2017-02-05 发布日期:2017-01-16
  • 通讯作者: 张烜 E-mail:zxpumch2003@hotmail.com
  • 基金资助:
    国家自然科学基金面上项目

Clinical characteristics of polymyositis and dermatomyositiscomplicated with lymphoma

  • Received:2015-12-31 Revised:2016-04-03 Online:2017-02-05 Published:2017-01-16
  • Supported by:
    the National Natural Science Foundation of China

摘要: 目的 探讨多发性肌炎(PM)/皮肌炎(DM)合并淋巴瘤的临床特点。方法 回顾性分析2000年至2015年北京协和医院收治的PM/DM合并淋巴瘤患者的资料,分析临床特点和预后,并以“多发性肌炎、皮肌炎、淋巴瘤”为关键词在PubMed检索并文献复习。结果 PM/DM合并胸腺瘤住院患者共10例,女性6例,男性4例。PM/DM的平均确诊年龄44.5岁,淋巴瘤的平均确诊年龄44.9岁。PM/DM进展至胸腺瘤的平均病程为4.7月。10例患者中6例为皮肌炎,4例为多发性肌炎,3例有肺间质病变,2例有关节炎,抗Jo-1抗体均阴性。合并淋巴瘤时前3位表现:淋巴结肿大(9例)、发热(8例)和脾大(5例)。淋巴瘤的病理确诊前3位组织来源:淋巴结(4例)、皮肤活检(2例)和局部肿物(2例)。10例患者均为非霍奇金淋巴瘤,其中T细胞来源4例,B细胞来源2例,4例分型不清。临床分期为:IIIB期4例,IVB期6例。10例患者在确诊后均使用免疫抑制治疗,确诊淋巴瘤后随访,5例死亡,4例化疗,1例失访。结论 PM/DM可以合并淋巴瘤,病程较短,死亡率高,值得引起临床医师重视。

关键词: 多发性肌炎, 皮肌炎, 淋巴瘤

Abstract: Objective To investigate the clinical features of polymyositis and dermatomyositis(PM/DM) with lymphoma. Methods The clinical data, treatment and prognosis ofPM/DM with lymphoma from 2000 to 2015 in Peking Union Medical College Hospital were retrospectively collected and analyzed. Results Ten cases of PM/DM patients complicated with lymphoma were recruited.6 were female and 4 was male. The mean ages of the diagnosis of PM/DM and lymphoma were 44.5 and 44.9 years old respectively. The average disease course from PM/DM to lymphoma was 4.7months. 6 cases were dermatomyositis and 4 cases were polymyositis. 3 cases had pulmonary fibrosis and 2 cases had arthritis. Nocasehad a positive result of anti-Jo-1 antibody. The most frequent symptoms complicated with lymphoma included as following: enlargement of lymph nodes (9 cases), fever (8 cases), splenomegaly (5 cases). Thetop 3 lymphoma biopsy specimenincluded lymph node(4 cases), skin(2 cases)andlocalmass(2 cases).All the 10 patients were diagnosed as non-Hodgkin lymphoma. 4 cases were derived from T cell, 2 cases from B cell, and 4 cases with unknown origin. The clinical staging showed III B (4 cases) and IV B (6 cases). Immunosuppressant agents were given to treat PM/DM. During thefollow-up period, 5 patients died, 4 patients received chemotherapy and 1 patient was lost. Conclusions The coincidence of PM/DM and lymphoma is not rare. The course between PM/DM and lymphoma is short, and the mortality is high, which should arise the attention of clinicians.

Key words: dermatomyositis, polymyositis, lymphoma

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