PB1215 - Sequential combined bypassing therapy in congenital haemophilia patients with inhibitors to VIII or IX factors: Surgical experience

Tuesday, June 25, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Hu Zhou, MD

Chief Physician
Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)

Co-Author(s)

Bingjie Ding, MD

Attending doctor
Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
XS

Xuewen Song

attending doctor
Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
PZ

Peng Zhang

Chief Physician
Department of Orthopaedic and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
LL

Liu Liu

Chief Physician
Department of Hematology, First Affiliated Hospital of Zhengzhou University
Zhengzhou, Henan, China (People's Republic)
XN

Xiaoying Niu

attending doctor
Department of Orthopaedic and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
ML

Mengjuan Li

attending doctor
Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
YZ

Yuanyuan Zhang

resident doctor
Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
AX

Ao Xia

attending doctor
Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
JL

Jingyuan Liu

attending doctor
Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
PL

Po Li

attending doctor
Department of Orthopaedic and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
FZ

Fan Zhang

attending doctor
Department of Orthopaedic and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
GL

Guancong Liu

attending doctor
Department of Orthopaedic and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)
ZL

Zhehuang Li

attending doctor
Department of Orthopaedic and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital
Zhengzhou, Henan, China (People's Republic)

Background: Some research is now examining the efficacy of Sequential combination bypass therapy (SCBT) regimens in individuals with hemophilia and exploring different inhibitor dosages and intervals.

Aims: Aimed to evaluate these surgical interventions' safety, efficacy.

Methods: The surgical data of 16 hemophilia patients with inhibitors was analysed .The main goal was to gather and outline the treatment plans employed in conjunction with bypass medications. The secondary aims were to evaluate the safety and efficacy of various SCBT regimens in hemophilia with inhibitors during the perioperative period. For Hemophilia patients with high titer inhibitors: Regimen 1 and Regimen 2 were shown in Figure 1. For Hemophilia patients with low titer inhibitors: Refer to Table 1.

Results: A total of 16 patients underwent 15 major surgeries and one minor surgery. (1) Four patients with high levels of inhibitors in regimen 1: The median dosage of rFVIIa on the day of operation was 728.22 μg/kg (705.88-800.00 μg/kg), resulting in a total consumption of 3143.59 μg/kg (2,823.53-4,900 μg/kg) of rFVIIa. The hemostasis rate achieved during surgery was 75% and 50% after operation. (2) Of the ten patients with regimen 2, the median rFVIIa on the day of surgery was 356.82 μg/kg (331.03-400.00μg/kg), and the total consumption was 2,140.91μg/kg. The total PCC consumption was 1443.42 IU/kg, ranging from 1,200.00 to 1,920.00 IU/kg. The hemostasis rate was 90% during and after surgery. The factor level increased to 1.321±0.118 IU/ml on the first day following the operation and gradually decreased to their lowest levels. 7.14%(1/14) patients with high levels of inhibitors had venous thrombosis. The FVIII inhibitor of one patient with a low titer inhibitor was found to have increased.43.75% (7/16/) patients had D-Di levels exceeding 5 mg/L.

Conclusion(s): Modifying SCBT regimens for perioperative hemophilia with inhibitors treatment is a secure and efficient approach for patients.