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China began hemophilia registration in the 1990s.
In 2009, the former Ministry of Health decided to establish a national hemophilia case information management system and designated the Chinese Academy of Medical Sciences Hematology Hospital as the national hemophilia case information management center.
As of 2019, a total of 17,779 patients with hemophilia A (HA) have been registered.
The production of inhibitors is the most serious complication of exogenous factor VIII (FVIII) concentrate replacement therapy in HA patients.
On February 22, the team of Professor Yang Renchi from the Hospital of Hematology of the Chinese Academy of Medical Sciences officially published in the British Journal of Haematology the results of a real-world study on Chinese patients with hemophilia A and inhibitors, revealing the current status of patients with HA and inhibitors in China.
The study retrospectively analyzed the data of 493 HA patients with inhibitors (466 severe HA and 27 non-severe HA) from 30 provincial hemophilia treatment centers (HTC) from 1995 to 2020.
The results of the study among all registered 17779 HA patients, 757 HA patients had inhibitors (Figure A below).
In the retrospective analysis of 493 patients with inhibitors, the proportions of patients in different age groups are as shown in Figure B; the median follow-up time is 3 (2-6) years; the median age at which inhibitors are diagnosed in severe and non-severe patients are respectively They were 13 (5-28) years old and 24 (10.
5-39.
5) years old.
The diagnosis age of non-severe patients was later than that of severe patients (OR 1.
027, 95% CI 1.
004-1.
051; P=0.
023).
The proportion of high-titer inhibitors is higher than that of low-titer inhibitors (Figure C below), and has nothing to do with the severity of hemophilia, family history, and treatment strategies before the inhibitors are produced.
An analysis of the follow-up data of 361 patients with HA with inhibitors from May 2019 to April 2020 (as shown in the figure below) found that only 19 (5.
3%) patients had no bleeding, and the main bleeding sites were joints (87.
5%) and muscles.
(43.
2%), mucosa (42.
9%).
The treatment status of 493 patients with inhibitor HA is shown in the figure below.
Prothrombin complex (PCC) is the most commonly used bypass hemostatic agent, followed by recombinant human coagulation factor VIIa (rFVIIa).
A small number of patients started Eimexal beads.
Monoclonal antibody treatment.
Only 22.
3% of patients received Immune Induction Tolerance (ITI), with a success rate of 64.
9%.
Among patients who did not undergo ITI, 17.
7% of patients with inhibitor titers continued to turn negative spontaneously, and inhibitors in the low-titer group continued spontaneously.
The proportion of patients who turned negative was much higher than that of the high-titer group.
Research conclusions This study is the largest cohort of patients with HA and inhibitors in China so far.
Patients with HA combined with inhibitors have high bleeding frequency and limited therapeutic drugs, and only a small proportion of patients receive ITI treatment.
Iimerizumab has brought better benefits to Chinese patients with HA combined with inhibitors, but the cost of treatment is still a huge obstacle.
This study provides data support for further exploring the diagnosis and treatment of patients with HA combined inhibitors and promoting the management of hemophilia in China.
Literature source: Xueqing Dou, Wei Liu, Man-Chiu Poon, et al.
Patients with haemophilia A with inhibitors in China: a national real-world analysis and follow-up.
British Journal of Haematology, 2021, 192, 900–908.
doi: 10.
1111/bjh.
17322.
Stamp "read the original", we will make progress together
In 2009, the former Ministry of Health decided to establish a national hemophilia case information management system and designated the Chinese Academy of Medical Sciences Hematology Hospital as the national hemophilia case information management center.
As of 2019, a total of 17,779 patients with hemophilia A (HA) have been registered.
The production of inhibitors is the most serious complication of exogenous factor VIII (FVIII) concentrate replacement therapy in HA patients.
On February 22, the team of Professor Yang Renchi from the Hospital of Hematology of the Chinese Academy of Medical Sciences officially published in the British Journal of Haematology the results of a real-world study on Chinese patients with hemophilia A and inhibitors, revealing the current status of patients with HA and inhibitors in China.
The study retrospectively analyzed the data of 493 HA patients with inhibitors (466 severe HA and 27 non-severe HA) from 30 provincial hemophilia treatment centers (HTC) from 1995 to 2020.
The results of the study among all registered 17779 HA patients, 757 HA patients had inhibitors (Figure A below).
In the retrospective analysis of 493 patients with inhibitors, the proportions of patients in different age groups are as shown in Figure B; the median follow-up time is 3 (2-6) years; the median age at which inhibitors are diagnosed in severe and non-severe patients are respectively They were 13 (5-28) years old and 24 (10.
5-39.
5) years old.
The diagnosis age of non-severe patients was later than that of severe patients (OR 1.
027, 95% CI 1.
004-1.
051; P=0.
023).
The proportion of high-titer inhibitors is higher than that of low-titer inhibitors (Figure C below), and has nothing to do with the severity of hemophilia, family history, and treatment strategies before the inhibitors are produced.
An analysis of the follow-up data of 361 patients with HA with inhibitors from May 2019 to April 2020 (as shown in the figure below) found that only 19 (5.
3%) patients had no bleeding, and the main bleeding sites were joints (87.
5%) and muscles.
(43.
2%), mucosa (42.
9%).
The treatment status of 493 patients with inhibitor HA is shown in the figure below.
Prothrombin complex (PCC) is the most commonly used bypass hemostatic agent, followed by recombinant human coagulation factor VIIa (rFVIIa).
A small number of patients started Eimexal beads.
Monoclonal antibody treatment.
Only 22.
3% of patients received Immune Induction Tolerance (ITI), with a success rate of 64.
9%.
Among patients who did not undergo ITI, 17.
7% of patients with inhibitor titers continued to turn negative spontaneously, and inhibitors in the low-titer group continued spontaneously.
The proportion of patients who turned negative was much higher than that of the high-titer group.
Research conclusions This study is the largest cohort of patients with HA and inhibitors in China so far.
Patients with HA combined with inhibitors have high bleeding frequency and limited therapeutic drugs, and only a small proportion of patients receive ITI treatment.
Iimerizumab has brought better benefits to Chinese patients with HA combined with inhibitors, but the cost of treatment is still a huge obstacle.
This study provides data support for further exploring the diagnosis and treatment of patients with HA combined inhibitors and promoting the management of hemophilia in China.
Literature source: Xueqing Dou, Wei Liu, Man-Chiu Poon, et al.
Patients with haemophilia A with inhibitors in China: a national real-world analysis and follow-up.
British Journal of Haematology, 2021, 192, 900–908.
doi: 10.
1111/bjh.
17322.
Stamp "read the original", we will make progress together
