Suppr超能文献

类风湿关节炎、中性粒细胞减少症和脾肿大:费尔蒂综合征

[Rheumatoid arthritis, neutropenia and splenomegaly: the Felty syndrome].

作者信息

Hofbauer L C, Diebold J, Heufelder A E

机构信息

Medizinische Klinik, Klinikum Innenstadt, Ludwig-Maximilians-Universität München.

出版信息

Dtsch Med Wochenschr. 1995 Dec 8;120(49):1689-94. doi: 10.1055/s-2008-1055529.

Abstract

HISTORY AND FINDINGS

A 59-year-old asymptomatic man, first diagnosed to have rheumatoid arthritis 27 years ago, was admitted to hospital because of splenomegaly and neutropenia, first noted 2 years ago. Physical examination confirmed splenomegaly and also revealed pretibial hyperpigmentation, but no evidence of active rheumatoid arthritis.

EXAMINATIONS

Biochemical tests showed relative and absolute neutropenia (white blood cell count 2200/microliters; 1% neutrophils), thrombocytopenia and polyclonal hypergammaglobulinaemia. He also had increased erythrocyte sedimentation rate (38/92), a high titre of rheumatic factor (2128 IU/ml) and increased circulating immune-complexes (74%). Thoracic and abdominal computed tomography provided no evidence of malignant tumor. The spleen measured 15 x 7 x 10 cm. Bone-marrow biopsy from the iliac crest revealed abnormal maturation of granulopoiesis and marked lymphoid infiltration. The clinical triad of rheumatoid arthritis, splenomegaly and neutropenia are diagnostic of Felty's syndrome. As the patient was asymptomatic there was no indication for treatment.

CONCLUSION

Felty's syndrome is a rare condition demanding considerable effort in differential diagnosis.

摘要

病史与检查结果

一名59岁无症状男性,27年前首次被诊断为类风湿关节炎,因2年前首次发现的脾肿大和中性粒细胞减少症入院。体格检查证实有脾肿大,还发现胫前色素沉着,但无活动性类风湿关节炎的迹象。

检查

生化检查显示相对和绝对中性粒细胞减少(白细胞计数2200/微升;中性粒细胞1%)、血小板减少和多克隆高球蛋白血症。他还出现红细胞沉降率升高(38/92)、高滴度风湿因子(2128 IU/ml)和循环免疫复合物增加(74%)。胸部和腹部计算机断层扫描未发现恶性肿瘤迹象。脾脏大小为15×7×10厘米。髂嵴骨髓活检显示粒细胞生成异常成熟和明显的淋巴细胞浸润。类风湿关节炎、脾肿大和中性粒细胞减少三联征可诊断为费尔蒂综合征。由于患者无症状,故无需治疗。

结论

费尔蒂综合征是一种罕见疾病,鉴别诊断需要付出相当大的努力。

相似文献

7
Felty's syndrome.费尔蒂综合征。
Semin Hematol. 1971 Jul;8(3):216-20.
9
[Felty's syndrome].[费尔蒂综合征]
Tidsskr Nor Laegeforen. 2000 Mar 30;120(9):1038-40.
10

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验