Recombinant activated factor Ⅶ in hemophagocytic lymphohistiocytosis with disseminated intravascular coagulation
10.3760/cma.j.issn.0366-6999.2011.19.043
- Author:
Jun-Ling ZHUANG
1
;
Qing-Wei JIANG
;
Ying XU
;
Shu-Jie WANG
Author Information
1. Department of Hematology Peking Union Medical College Hospital, Beijing 100730, China
- Keywords:
recombinant factor Vlla;
hemaphagocytic lymphohistiocytosis;
disseminated intravascular coagulation
- From:
Chinese Medical Journal
2011;125(19):3189-3191
- CountryChina
- Language:Chinese
-
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.
