Sibilia J, Javier R M, Albert A, Cazenave J P, Kuntz J L
Service de Rhumatologie, CHU de Hautepierre and Etablissement de Transfusion Sanguine de Strasbourg, France.
J Rheumatol. 1998 Jun;25(6):1218-20.
Hemophagocytic syndrome is an exceptional cause of pancytopenia. Its etiologies are most commonly viral or bacterial infections, lymphoproliferative syndromes, acquired or congenital immunodeficiencies, systemic diseases, or immunomodulatory treatment. We describe a patient with rheumatoid arthritis (RA) treated with methotrexate (MTX), sulfasalazine, and low dose corticosteroids, whose case was seriously complicated by the occurrence of acute febrile pancytopenia. The pancytopenia appeared secondary to hemophagocytic syndrome triggered by Escherichia coli septicemia. The evolution was marked by severe aggravation of RA, probably due to release of cytokines from macrophages (tumor necrosis factor-alpha, interleukin 6). Reintroduction of MTX (without sulfasalazine) resulted in partial remission and there was no reappearance of new hematological anomalies after 16 month followup. A knowledge of this syndrome is particularly important, since it mimics drug toxicity and other complications such as lymphoproliferative diseases.
噬血细胞综合征是全血细胞减少的一种罕见病因。其病因最常见的是病毒或细菌感染、淋巴增殖性综合征、获得性或先天性免疫缺陷、全身性疾病或免疫调节治疗。我们描述了一名类风湿关节炎（RA）患者，该患者接受甲氨蝶呤（MTX）、柳氮磺胺吡啶和低剂量皮质类固醇治疗，其病情因急性发热性全血细胞减少的发生而严重复杂化。全血细胞减少继发于大肠杆菌败血症引发的噬血细胞综合征。病情演变的特征是RA严重加重，可能是由于巨噬细胞释放细胞因子（肿瘤坏死因子-α、白细胞介素6）所致。重新使用MTX（不使用柳氮磺胺吡啶）导致部分缓解，随访16个月后未再次出现新的血液学异常。了解这种综合征尤为重要，因为它可模仿药物毒性和其他并发症，如淋巴增殖性疾病。
