Hofbauer L C, Diebold J, Heufelder A E
Medizinische Klinik, Klinikum Innenstadt, Ludwig-Maximilians-Universität München.
Dtsch Med Wochenschr. 1995 Dec 8;120(49):1689-94. doi: 10.1055/s-2008-1055529.
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.
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.
Felty's syndrome is a rare condition demanding considerable effort in differential diagnosis.
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。
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。
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。