Abella E M, Artrip J, Schultz K, Ravindranath Y
Children's Hospital of Michigan, Department of Pediatrics, Wayne State University School of Medicine, Detroit 48201, USA.
J Pediatr. 1997 Mar;130(3):467-70. doi: 10.1016/s0022-3476(97)70212-9.
Familial erythrophagocytic lymphocytosis (FEL) is a rare, nonmalignant class II histiocytosis characterized by fever, irritability, hepatosplenomegaly, pancytopenia, and hemophagocytosis. Various chemotherapeutic regimens have had mixed success, with the only curative therapy being bone marrow transplantation. We report our experience with two children whose therapy with etoposide and steroids failed. They were successfully treated and had durable remissions with cyclosporine A (CSA). We propose that in FEL there may exist abnormal interactions between antigen-presenting cells and T-lymphocyte subsets, and that CSA may down-modulate this aberrant response. The use of a low-dose CSA regimen may represent a treatment option that should be further explored.
家族性噬血细胞性淋巴细胞增多症（FEL）是一种罕见的非恶性II类组织细胞增多症，其特征为发热、易激惹、肝脾肿大、全血细胞减少和噬血细胞现象。各种化疗方案的效果不一，唯一的治愈性疗法是骨髓移植。我们报告了两例使用依托泊苷和类固醇治疗失败的儿童的治疗经验。他们使用环孢素A（CSA）成功治疗并获得了持久缓解。我们提出，在FEL中，抗原呈递细胞与T淋巴细胞亚群之间可能存在异常相互作用，且CSA可能下调这种异常反应。低剂量CSA方案的使用可能代表一种应进一步探索的治疗选择。
