title: Recombinant activated factor VII in hemophagocytic lymphohistiocytosis with disseminated intravascular coagulation.
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 (rFVIIa) 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 rFVIIa 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 rFVIIa may be effective in the management of intractable hemorrhage in patients with HLH.
authors: Department of Hematology, Peking Union Medical College Hospital, Beijing 100730, China.
authors: Jun-Ling
authors: JL
authors: Zhuang
authors: Qing-Wei
authors: QW
authors: Jiang
authors: Ying
authors: Y
authors: Xu
authors: Shu-Jie
authors: SJ
authors: Wang
mesh_terms: D000328:Adult; D004211:Disseminated Intravascular Coagulation / Q000150:complications*; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D011994:Recombinant Proteins; D015942:Factor VIIa