Cornet Alexander D, Thielen Noortje, Kramer Mark H, Nanayakkara Prabath W, Kooter Albertus J
VU medisch centrum, afd. Interne Geneeskunde, Amsterdam, the Netherlands.
Ned Tijdschr Geneeskd. 2010;154:A2528.
A 49-year-old woman with a history of adult-onset Still's disease (AOSD) presented with fever, general malaise and a rash. Laboratory blood testing revealed an extremely high level of serum ferritin, essentially restricting the differential diagnosis to either haemophagocytic syndrome as a complication of AOSD, or a flare-up of the latter. Haemophagocytosis as a complication of AOSD was diagnosed in our patient. After treatment with prednisone, she fully recovered and the serum ferritin returned to a normal level. Haemophagocytic syndrome is a rare but potentially life-threatening complication of lymphoproliferative and autoimmune diseases, as well as of viral infections. It is characterised by high fever, hepatosplenomegaly, cytopenia and extremely high levels of serum ferritin. Activation of macrophages and histiocytes induces phagocytosis of erythrocytes in the bone marrow and other parts of the reticuloendothelial system. The fact that haemophagocytic syndrome and AOSD are often described together, and coincide with extremely elevated serum ferritin levels characteristic to both entities, suggests a related pathogenesis.
一名患有成人斯蒂尔病（AOSD）的49岁女性出现发热、全身不适和皮疹。实验室血液检测显示血清铁蛋白水平极高，这基本上将鉴别诊断局限于AOSD并发症噬血细胞综合征或AOSD病情复发。我们的患者被诊断为AOSD并发症噬血细胞综合征。经泼尼松治疗后，她完全康复，血清铁蛋白恢复到正常水平。噬血细胞综合征是淋巴增殖性疾病、自身免疫性疾病以及病毒感染的一种罕见但可能危及生命的并发症。其特征为高热、肝脾肿大、血细胞减少和血清铁蛋白水平极高。巨噬细胞和组织细胞的激活诱导骨髓及网状内皮系统其他部位的红细胞被吞噬。噬血细胞综合征和AOSD常被一同描述，且与两者均具有的血清铁蛋白水平极度升高同时出现，这一事实提示了相关的发病机制。
