Burns B F, Walley V M, Davies R A, Auclair F, Bormanis J
Anatomical Pathology, Department of Laboratory Medicine, Department of Medicine, The University of Ottawa Heart Institute and The Ottawa Civic Hospital, Ottawa, Ontario, Canada.
Cardiology, Department of Medicine, The University of Ottawa Heart Institute and The Ottawa Civic Hospital, Ottawa, Ontario, Canada.
Cardiovasc Pathol. 1998 Jan-Feb;7(1):47-50. doi: 10.1016/S1054-8807(97)00060-4.
Infection-associated hemophagocytic syndrome is one of the hemophagocytic disorders, and is most often seen in the pediatric population, typically in the setting of immunosuppression. We present the case of a 33-year-old man who had been well for more than 3 years following cardiac transplantation until he developed the infection-associated hemophagocytic syndrome. The patient had a fulminant downhill course, dying in shock 10 weeks after his first presentation. Serologic studies for Epstein-Barr virus suggested a remote infection; other viral and microbiologic studies were negative. The only previous report of infection-associated hemophagocytic syndrome complicating cardiac transplant appears to be that of a pediatric patient. The case presented illustrates the difficulties in antemortem diagnosis of this disorder, and in its treatment.
感染相关噬血细胞综合征是噬血细胞疾病之一，最常见于儿童群体，通常发生在免疫抑制的情况下。我们报告一例33岁男性病例，该患者心脏移植后3年多情况良好，直至发生感染相关噬血细胞综合征。患者病情急转直下，首次就诊10周后死于休克。针对EB病毒的血清学研究提示既往感染；其他病毒学和微生物学研究均为阴性。既往唯一一篇关于心脏移植并发感染相关噬血细胞综合征的报道似乎是一名儿科患者的病例。本文所呈现的病例说明了该疾病生前诊断及治疗的困难。
