Introduction Perioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half‐life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients. Aim To summarise Nordic real‐world surgical experiences with EHL recombinant factor VIII/IX Fc (rFVIIIFc/rFIXFc) fusion proteins using retrospective data from clinical records at four HTCs in Finland, Sweden and Norway. Methods Factor dosing and surgical outcomes were recorded from HA/HB patients who underwent surgery and were treated with rFVIIIFc/rFIXFc. Perioperative factor dosing regimens were clinician‐determined based on local practises. Results Twenty five surgeries were performed on 20 patients, all covered by bolus injections except one minor HA surgery; eight minor surgeries were in paediatric patients. Median preoperative rFVIIIFc dose for major HA surgeries (n = 8) was 48 IU/kg (range: 35–57), with total consumption up to Day 14 of 427 IU/kg (196–568). For the two major HB surgeries (in one patient), preoperative rFIXFc doses were 50 IU/kg and 20 IU/kg; total consumption up to Day 14 was 130 IU/kg and 40 IU/kg. Median preoperative rFVIIIFc/rFIXFc bolus doses for minor HA (n = 10) and HB (n = 4) surgeries were 50 IU/kg (24–79) and 47 IU/kg (40–71), with total consumption up to Day 5 of 138 IU/kg (49–404) and 100 IU/kg (43–125), respectively. Intraoperative and postoperative haemostatic responses were rated as at least good/excellent for 24/25 surgeries, with bleeding episodes reported in only three surgeries. Conclusion Nordic real‐world experiences suggest that EHL products can be used safely and effectively for peri‐operative haemostasis. Further research is required to develop local dosing guidelines for optimised treatment schedules.
Summary Acquired haemophilia A (AHA) is a rare bleeding disorder caused by acquired antibodies against coagulation factor VIII. In the Nordic countries, treatment and outcomes have not been studied in recent times. To collect retrospective data on patients diagnosed with AHA in the Nordic countries between 2006 and 2018 and compare demographic data and clinical outcomes with previously published reports, data were collected by six haemophilia centres: three Swedish, one Finnish, one Danish and one Estonian. The study included 181 patients. Median age at diagnosis was 76 (range 5–99) years, with even gender distribution. Type and severity of bleeding was comparable to that in the large European Acquired Haemophilia Registry study (EACH2). Bleedings were primarily treated with activated prothrombin complex concentrate (aPCC) with a high success rate (91%). For immunosuppressive therapy, corticosteroid monotherapy was used most frequently and this may be the cause of the overall lower clinical remission rate compared to the EACH2 study (57% vs. 72%). Survey data on 181 patients collected from four north European countries showed similar demographic and clinical features as in previous studies on AHA. aPCC was used more frequently than in the EACH2 study and the overall remission rate was lower.
ing protection against bleeds. In both the inhibitor and non-inhibitor patients the remarkable improvement is evidenced in the real-world settings after the extensive clinical phase 3 studies. [1][2][3][4] After stopping the coadministration with activated prothrombin complex concentrate (aPCC, Feiba R ), only a small number of adverse events have been encountered, associated with thrombosis and some breakthrough bleeds. The immediate management option for possible breakthrough bleeds or trauma either with recombinant FVIIa (rFVIIa, Novo Seven R ) or factor VIII concentrate (non-inhibitor patients) should be available for all patients. In our centre we have also advised the patients to immediately contact us (24/7) in case they would need rFVIIa or a replacement therapy.Recent WFH publication reports that prophylactic haemophilia care is not provided globally in 85% of patients. 5 This is mostly related to the
Introduction Majority of haemophilia A patients in our comprehensive care centre have switched from standard half‐life (SHL) to extended half‐life (EHL) FVIII products in a short time. Aim We compared the clinical and laboratory outcomes between SHL and EHL FVIII prophylaxis in product switchers. Methods This is a retrospective inception cohort of all adult haemophilia A patients switched to EHL (rFVIIIFc or rFVIII‐PEG) prophylaxis in our centre. Dosing, product utilization, annualized bleed rates (ABR), treatment regimen and pharmacokinetics by Web Accessible Population Pharmacokinetic Service (WAPPS)‐Hemo were compared between SHL and EHL. Results We included 38 patients, whose median age was 38 years (range 17–75). Median FVIII dose was 23 IU/kg for SHL versus 25 IU/kg for EHL. After switching, weekly infusions decreased by 29% from median 2.8 (every 2.5 days) to 2.0 (every 3.5 days) (P = <.001) and factor consumption for prophylaxis by 17% from 60 to 50 IU/kg/week (P = <.001). Weekly infusions decreased in 71% and FVIII utilization in 55% of patients. ABR remained low (1.0 for SHL and .5 for EHL, respectively). In pharmacokinetics, the half‐life of FVIII increased from median 13 to 21 h after switching. Times above .01 and .03 IU/ml improved from 85 to 131 h and from 65 to 106 h. Half‐lives of the SHL products and von Willebrand factor levels predicted half‐lives with the EHL products. Conclusions Our cohort study confirms the successful experience of switching to EHL FVIII products, with decreased infusion frequency, factor consumption and excellent clinical efficacy.
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