滑膜肉瘤患者发生获得性噬血细胞综合征：一例报告

Acquired hemophagocytic syndrome in a patient with synovial sarcoma: a case report.

作者信息

Ciccarese Chiara, Ferrara Roberto, Fantinel Emanuela, Zecchetto Camilla, Simionato Francesca, Grego Elisabetta, Ortolani Silvia, Caccese Mario, Bimbatti Davide, Cingarlini Sara, Brunelli Matteo, Andreini Angelo, Tortora Giampaolo, Massari Francesco

机构信息

Medical Oncology, Azienda Ospedaliera Universitaria Integrata (A.O.U.I.), University of Verona, P.le L.A Scuro 10, 37134 Verona, Italy; Medical Oncology, Azienda Ospedaliera Universitaria Integrata (A.O.U.I.), University of Verona, P.le L.A Scuro 10, 37134 Verona, Italy.

Department of Pathology & Diagnostic, Azienda Ospedaliera Universitaria Integrata (A.O.U.I.), University of Verona, Verona, Italy; Department of Pathology & Diagnostic, Azienda Ospedaliera Universitaria Integrata (A.O.U.I.), University of Verona, Verona, Italy.

出版信息

Future Sci OA. 2015 Nov 1;1(4):FSO29. doi: 10.4155/fso.15.27. eCollection 2015 Nov.

DOI:10.4155/fso.15.27

PMID:28031902

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5138004/

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia and low fibrinogen. Hemophagocytosis in the bone marrow is a hallmark of this syndrome. Based on the pathogenetic mechanism, it can be classified into primary (inherited) or secondary (acquired) HLH. We report, to our knowledge, the first case of acquired hemophagocytic syndrome that arose in a 20-year-old man affected by synovial sarcoma as a complication during chemotherapy.

摘要

噬血细胞性淋巴组织细胞增生症（HLH）是一种由于对不同触发因素产生压倒性无效免疫反应而导致严重炎症反应的综合征。最重要的症状是发热、肝脾肿大和血细胞减少。生化指标包括铁蛋白升高、高甘油三酯血症和纤维蛋白原降低。骨髓中的噬血细胞现象是该综合征的一个标志。根据发病机制，它可分为原发性（遗传性）或继发性（获得性）HLH。据我们所知，我们报告了首例在一名20岁滑膜肉瘤患者化疗期间作为并发症出现的获得性噬血细胞综合征病例。