Pregnant Women with Severe Factor VII Deficiency Undergoing Cesarean Section Managed with a Short-Term Regimen of Recombinant Factor VIIa

To editor:Factor VII(FVII)is a determining factor in activating the exogenous coagulation pathway;F7 gene mutation decreases the FVII number or function.Factor VII deficiency(FVIID)is characterized by an isolated prolongation of the prothrombin time(PT)with a normal activated partial thromboplastin time(aPTT).Factor assay and genotyping for FVII can be done to confirm the diagnosis.1,2 Patients may present with menorrhagia,and bleeding tendencies may vary from slight gingival bleeding,epistaxis,and ecchymosis,to severe bleeding,such as gastrointestinal and intracranial bleeding.

Recombinant activated factor Ⅶ in hemophagocytic lymphohistiocytosis with disseminated intravascular coagulation

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.