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Multicenter Study
. 2019 Dec;187(5):653-665.
doi: 10.1111/bjh.16128. Epub 2019 Aug 2.

Outcome of CARE: a 6-year national registry of acquired haemophilia A in China

Boyang Sun  1 , Feng Xue  1 , Ying Feng  2 , Jing Sun  3 , Ziqiang Yu  4 , Ming Hou  5 , Jingyu Zhang  6 , Xiaojing Zeng  7 , Yongqiang Zhao  8 , Shimei Lian  9 , Meijuan Huang  10 , Ting Niu  11 , Zhongguang Cui  12 , Jingsheng Wu  13 , Renchi Yang  1
Affiliations
Multicenter Study

Outcome of CARE: a 6-year national registry of acquired haemophilia A in China

Boyang Sun et al. Br J Haematol. 2019 Dec.

Abstract

Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by autoantibodies directed against the functional epitopes of coagulation factor VIII (FVIII). Its management relies on prompt diagnosis, control of bleeding and eradication of the inhibitor by immunosuppression. China Acquired Hemophilia Registry (CARE), a nationwide multicentre registry, was intended to survey the status of AHA and standardize its diagnosis and therapy in China. One hundred and eighty-seven registered patients had an average age of 52 years. Diagnosis was delayed in 46·5% patients. There was a significant delay from diagnosis to immunosuppressive therapy in 68·3% patients. Bleeding control was significantly higher in patients treated with prothrombin complex concentrate (PCC) versus FVIII replacement therapy (84·6% vs. 34·4%; P < 0·001). Inhibitor eradication with a combination of steroids and cyclophosphamide showed a higher partial remission (PR) rate (92·2% vs. 70·3%) and stable complete remission (CR) rate (82·8% vs. 48·6%) than with steroids alone. Logistic regression model showed age and malignancy were significantly related to survival at final follow-up. The mean age for the survivors [51 years (IQR, 35-65 years)] was significantly lower than that of the non-survivors [79 years (IQR, 67-86 years)] (P < 0·001). Overall survival was higher in non-malignancy group than malignancy group (94·9% vs. 70%) (OR = 1·313; 95% CI, 0·913-1·889, P = 0·015).

Keywords: acquired haemophilia A; diagnosis; haemostatic treatment; immunosuppression; outcome; registry.

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