中华医学杂志（英文版）2011，Vol.125Issue(19)：3189-3191,3.DOI:10.3760/cma.j.issn.0366-6999.2011.19.043
Recombinant activated factor Ⅶ in hemophagocytic lymphohistiocytosis with disseminated intravascular coagulation
Recombinant activated factor Ⅶ in hemophagocytic lymphohistiocytosis with disseminated intravascular coagulation
ZHUANG Jun-ling 1JIANG Qing-wei 2XU Ying 1WANG Shu-jie3
作者信息
- 1. Department of Hematology Peking Union Medical College Hospital, Beijing 100730, China
- 2. Department of Internal Medicine Peking Union Medical College Hospital, Beijing 100730, China
- 3. Department of Hematology Peking Union Medical College Hospital, Beijing 100730, China
- 折叠
摘要
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening disorder due to hyperinflammation resulting in infiltration of different organs with extensive hemophagocytosis. Severe coagulopathy was one of the main reasons for death in HLH. Over secretion of plasminogen activator by activated macrophages leads to hyperfibrinolysis. We reported a 36-year-old woman who was diagnosed as HLH probably secondary to lymphoma. Massive bleeding from gut and retroperitoneal area were not able to be controlled by conventional hemostatic treatments. This patient received one dose recombinant activated factor Ⅶ (rFVlla) 3.6 mg (70 μg/kg). Hemostatic effect was achieved in 0.5 hour and lasted 24 hours. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were quickly corrected to normal ranges.Fibrinogen level elevated from 0.5 g/L before using rFVIla to 1.8 g/L 20 hours after. Although dexamethasone and etopside were administrated to treat HLH, this patient died from septic shock after persistent neutropenia. This suggests that rFVlla may be effective in the management of intractable hemorrhage in patients with HLH.关键词
recombinant factor Vlla/hemaphagocytic lymphohistiocytosis/disseminated intravascular coagulationKey words
recombinant factor Vlla/hemaphagocytic lymphohistiocytosis/disseminated intravascular coagulation引用本文复制引用
ZHUANG Jun-ling,JIANG Qing-wei,XU Ying,WANG Shu-jie..Recombinant activated factor Ⅶ in hemophagocytic lymphohistiocytosis with disseminated intravascular coagulation[J].中华医学杂志（英文版）,2011,125(19):3189-3191,3.
