[成人斯蒂尔病中的高铁蛋白血症与反应性噬血细胞综合征]
[Hyperferritinemia in Still syndrome in the adult and reactive hemophagocytic syndrome].
作者信息
Zollner R C, Kern P, Steininger H, Kalden J R, Manger B
机构信息
Medizinische Abteilung, Klinikum Deggendorf.
出版信息
Med Klin (Munich). 1997 Aug 15;92(8):494-8. doi: 10.1007/BF03044919.
This report describes the fatal outcome of a case of adult onset Still's disease in a 46-year old man. The diagnosis was made according to the 1992 criteria, proposed by Yamaguchi. Nine months after the initial disease manifestations a rapid deterioration with progressive hepatosplenomegaly developed. In parallel, pancytopenia and marked hyperferritinemia could be detected. Transjugular liver biopsy revealed the presence of a hemophagocytic syndrome. The course of the disease was refractory to any form of treatment and the patient died from disseminated intravascular coagulation, hepatic and pulmonary failure. Pathogenetic mechanisms and possible associations between Still's disease and reactive hemophagocytic syndrome are discussed.
本报告描述了一名46岁男性成人斯蒂尔病患者的致命结局。诊断依据是山口于1992年提出的标准。在最初出现疾病症状9个月后，病情迅速恶化，出现进行性肝脾肿大。同时，可检测到全血细胞减少和显著的高铁蛋白血症。经颈静脉肝活检显示存在噬血细胞综合征。该疾病的病程对任何形式的治疗均无效，患者死于弥散性血管内凝血、肝衰竭和呼吸衰竭。文中讨论了斯蒂尔病与反应性噬血细胞综合征之间的发病机制及可能的关联。
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