Spivak J L
Johns Hopkins Med J. 1977 Sep;141(3):156-62.
The clinical and laboratory features of 72 patients with Felty's syndrome described within the last ten years have been compared with Felty's five original patients. Felty's syndrome appears to be a variant of rheumatoid arthritis with extra-articular manifestations in which leukopenia (usually due to neutropenia) and splenomegaly occur, although not always at the same time. Both are manifestations of the underlying disease process and are not necessarily otherwise related. The mechanism of the leukopenia is complex and abnormalities in leukocyte function appear to be as important as the leukopenia in predisposing patients with Felty's syndrome to infection. Functional abnormalities of the leukocytes in this syndrome are due in part to immune complex formation. Hypocomplementemia associated with this process may be another cause for the increased susceptibility to infection. It is proposed, therefore, that therapy in Felty's syndrome be directed at the underlying disease process, and gold salts and penicillamine should be considered for this purpose. Splenectomy should be reserved for specific situations, such as hemolytic anemia, severe thrombocytopenia, leg ulcers, and infections associated with profound leukopenia that are not responsive to medical therapy.
对过去十年间描述的72例费尔蒂综合征患者的临床和实验室特征与费尔蒂最初的5例患者进行了比较。费尔蒂综合征似乎是类风湿关节炎的一种变异型，伴有白细胞减少（通常由于中性粒细胞减少）和脾肿大等关节外表现，尽管并非总是同时出现。两者都是潜在疾病过程的表现，不一定有其他关联。白细胞减少的机制很复杂，白细胞功能异常在使费尔蒂综合征患者易发生感染方面似乎与白细胞减少同样重要。该综合征中白细胞的功能异常部分归因于免疫复合物的形成。与此过程相关的补体减少可能是易感性增加的另一个原因。因此，建议对费尔蒂综合征的治疗应针对潜在的疾病过程，为此可考虑使用金盐和青霉胺。脾切除术应保留用于特定情况，如溶血性贫血、严重血小板减少、腿部溃疡以及对药物治疗无反应的与严重白细胞减少相关的感染。
