scholarly journals Case report: successful perioperative management of patients with haemophilia A using an extended half-life factor VIII (Efmoroctocog alfa) during neurosurgical procedures

2021 ◽  
Vol 12 ◽  
pp. 204062072199368
Author(s):  
Florian Kocher ◽  
Andreas Seeber ◽  
Johannes Kerschbaumer ◽  
Stefan Schmidt ◽  
Dominik Wolf ◽  
...  

Patients with haemophilia A (HA) undergoing neurosurgical procedures have a high risk of haemorrhage with potential fatal outcome. Here, we present a successful perioperative haemostatic concept applying an extended half-life factor VIII (EHL FVIII), Efmoroctocog alfa, in two patients with HA undergoing neurosurgery for paramedian right-sided disc herniation (case 1) and astrocytoma (case 2). After adequate EHL FVIII treatment the surgical procedures were performed without any bleeding complications despite the high-risk interventions. Laboratory measurements confirmed stable FVIII levels throughout the hospital stay. We suggest close interdisciplinary collaboration between involved clinicians as mandatory prerequisite for an optimized perioperative management in patients with HA. The presented cases indicate, that the increased stability, safety and fewer injections provide a rationale to use EHL FVIII products in HA patients undergoing surgical interventions with a very high bleeding risk.

Haemophilia ◽  
2018 ◽  
Vol 24 (4) ◽  
pp. e280-e283
Author(s):  
I. C. L. Kremer Hovinga ◽  
R. E. G. Schutgens ◽  
P. R. van der Valk ◽  
L. F. D. van Vulpen ◽  
E. P. Mauser-Bunschoten ◽  
...  

2011 ◽  
Vol 31 (S 01) ◽  
pp. S20-S23
Author(s):  
D. Mihailov ◽  
L. Pop ◽  
H. Ionita ◽  
E. Ursu ◽  
S. Talpos-Niculescu ◽  
...  

SummaryA number of 513 consecutive patients (494-haemophilia A and 19-haemophilia B) from eight haemophilia treatment centers have been investigated with Bethesda assay for the presence of factor VIII or IX inhibitors. The overall prevalence of inhibitors was 15.20%, 18.48% in severe, 5.60% in moderate and 12.24% in mild forms. The prevalence was higher than reported in most of the western countries. The age at start of substitution (p = 0.9775), the frequent switching of factor concentrates (p = 0.8931) were not relevant factors for the development of inhibitors. It is worth to be mentioned the unexpectedly occurrence of inhibitors in prior inhibitor negative (6/72) patients (during surgical interventions) probably due to their previous scarce substitution, occurrence which seems not being connected with the continuous infusion modality of factor VIII administration (p = 0.8341). In controversial situations, in the field of low titer (≤1 BU/ml) inhibitors for a reliable interpretation of the results the performance of recovery index and half-life time assessment of FVIII/IX was undertaken.


Haemophilia ◽  
2021 ◽  
Author(s):  
Alanna McEneny‐King ◽  
Pierre Chelle ◽  
Margaret H. Goggans ◽  
Patricia J. Barker ◽  
Timothy W. Jacobs ◽  
...  

2020 ◽  
Vol 40 (S 01) ◽  
pp. S15-S20
Author(s):  
Jens Müller ◽  
Georg Goldmann ◽  
Natascha Marquardt ◽  
Bernd Pötzsch ◽  
Johannes Oldenburg

AbstractDue to structural differences between extended half-life (EHL) factor VIII (FVIII) or FIX products and equivalent plasma wild-type molecules used for assay calibration, reagent-dependent discrepancies during monitoring of FVIII- and FIX-replacement therapies with EHL products have been described. To assess the performance of available one-stage clotting and chromogenic substrate assays on the Siemens Atellica COAG 360 analyzer, an in vitro study using spiked plasma samples was performed. The described results confirm previously described findings and allowed allocation of each EHL product to an appropriate assay. In addition, corresponding EHL product–specific analytes were defined within the order entry system of the University Hospital Bonn. The requirement of product-specific FVIII and FIX assays complicates patient monitoring and demonstrates the need for both continuous education and communication between treating physicians and the coagulation laboratory.


2015 ◽  
Vol 169 (6) ◽  
pp. 768-776 ◽  
Author(s):  
Ali J. Mahdi ◽  
Samya G. Obaji ◽  
Peter W. Collins

Haemophilia ◽  
2016 ◽  
Vol 22 (6) ◽  
pp. e552-e554 ◽  
Author(s):  
L. M. Malec ◽  
J. Journeycake ◽  
M. V. Ragni

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