Combination of FVIII and low-dose rFVIIa improves haemostasis in acquired haemophilia A patients: a collaborative controlled study.
BACKGROUND
Acquired haemophilia A (AHA) is an autoimmune disease that potentially leads to severe bleeding and has a high rate of mortality. This collaborative study aimed to assess the efficacy of the co-administration of FVIII and low-dose rFVIIa in patients with AHA.


METHODS
This study retrospectively compared the combined FVIII/low-dose rFVIIa therapy (initial dose range of 25-55μg/Kg) with the combined FVIII/PCC therapy and low-dose rFVIIa monotherapy. Adverse drug reactions and recurrent bleeding episodes were also monitored. Crude comparisons and the exact conditional logistic regression were performed to compare the outcomes between three treatment groups.


RESULTS
First bleeding episodes of 56 consecutive patients from 5 centres were analyzed, and 37 bleeding episodes (66.1%) were determined to be severe. Specifically, the rate of bleeding control was significantly higher with the FVIII/low-dose rFVIIa therapy compared to that of the low-dose rFVIIa alone therapy or the FVIII/PCC therapy (58.3% vs. 41.7% vs. 95.0%, respectively). Analyzing of total 236 bleeding episodes showed a clear positive association between the early initiation of haemostatic treatment and efficacy. No therapy-related adverse events in which thrombosis predominated were reported.


CONCLUSIONS
The combination of FVIII and low-dose rFVIIa offers an ideal haemostatic cover and may be promoted as a feasible and safe therapy protocol for patients with AHA.
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.. Electronic address: xiaohuizhang987@163.com.
Xiao-Hui
XH
Zhang
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.
Xiao-Lu
XL
Zhu
Department of haematology, West China Hospital, West China School of Clinical Medicine, Sichuan University, Chengdu, Sichuan, P.R.C.
Ting
T
Niu
Department of haematology, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong, P.R.C.
Jing
J
Sun
Department of haematology, Beijing Hospital, Beijing, P.R.C.
Hui
H
Liu
Department of haematology, Beijing Hospital, Beijing, P.R.C.
Ru
R
Feng
Department of haematology, the Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, P.R.C.
Lin-Hua
LH
Yang
Department of haematology, West China Hospital, West China School of Clinical Medicine, Sichuan University, Chengdu, Sichuan, P.R.C.
Qing
Q
Wei
Department of haematology, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong, P.R.C.
Qing-Hui
QH
Ma
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.
Qing-Ming
QM
Wang
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.
Fei-Er
FE
Feng
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.
Hai-Xia
HX
Fu
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.
Xiao-Dong
XD
Mo
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.
Meng
M
Lv
Peking University People's Hospital, Peking University Institute of Haematology, Beijing, P.R.C.. Electronic address: huangxiaojun@bjmu.edu.cn.
Xiao-Jun
XJ
Huang
D000208:Acute Disease; D001327:Autoimmune Diseases / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D001779:Blood Coagulation Factors / Q000009:adverse effects / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D061345:Early Medical Intervention; D005169:Factor VIII / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D015942:Factor VIIa / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D006467:Hemophilia A / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D006470:Hemorrhage / Q000517:prevention & control; D006487:Hemostasis / Q000187:drug effects*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010314:Partial Thromboplastin Time; D011994:Recombinant Proteins / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use; D012189:Retrospective Studies; D016896:Treatment Outcome
D001779:Blood Coagulation Factors; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa
Acquired hemophiliaA; Bleeding disorder; FVIII; PCC; rFVIIa