ISTH Congress Abstracts

Official abstracts site for the ISTH Congress

MENU 
  • Home
  • Congress Archive
    • ISTH 2021 Congress
    • ISTH 2020 Congress
  • Resources
  • Search

Real-world Experience of rIX-FP Prophylaxis at Dosing Intervals of up to 14 Days in a Pediatric Patient with Hemophilia B during the COVID-19 Pandemic

B.L. Díaz Jordan1, T. Cebanu2, M.T. Álvarez Román2

1Servicio de Hematología, Hospital General de Valdepeñas, Ciudad Real, Spain, 2Unidad de Hemostasia, Servicio de Hematología, Hospital Universitario La Paz, Madrid, Spain

Abstract Number: PB0645

Meeting: ISTH 2021 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: rIX-FP, a recombinant factor IX (rFIX) linked with recombinant human albumin, has demonstrated efficacy in prophylactic regimens up to every 21 days in patients with hemophilia B.

Aims: To describe real world experience for a rural pediatric patient during the COVID-19 pandemic.

Methods: Demographic, clinical (HEAD-US and Hemophilia Joint Health Score) and pharmacokinetic (PK) data were collected for up to one year following a switch from rFIX to rIX-FP.

Results: In May 2018, a 3-year-old patient (moderate hemophilia B, FIX: 2.7 IU/dL) experienced hemarthrosis in the left knee and initiated prophylaxis with rFIX (40 IU/kg 2×/week). In March 2020, the patient switched to weekly rIX-FP prophylaxis (40 IU/kg), due to difficulties with venous access and accessing the clinic during the pandemic. Trough levels of 7.7% two weeks post-infusion and 5.1% 20 days post-infusion were maintained. Functional and joint assessment on day 90 of rIX-FP treatment showed no change, and no FIX inhibitors developed. On day 95 of treatment, a post-traumatic otorrhagia subsided after a single 40 IU/kg dose of rIX-FP. On day 120, the treatment interval was extended to 10 days without complications. After 160 days, the patient was diagnosed with an asymptomatic SARS-CoV-2 infection and self‐isolated at home for 14 days with no changes to prophylactic treatment. On day 200, after a new PK study (trough levels of 6.9% at 14 days post-infusion), the prophylaxis interval was prolonged to 70 IU/kg every 14 days without incident. With 14-day dose intervals, monthly consumption reduced by 56.2% and the number of infusions by 75% compared with the initial rFIX treatment, without affecting efficacy or adherence.

Conclusions: Switching to rIX-FP in this pediatric patient reduced the number of administrations and clinic visits without affecting efficacy or adherence, a fact that is relevant in the current context of the pandemic.

To cite this abstract in AMA style:

Díaz Jordan BL, Cebanu T, Álvarez Román MT. Real-world Experience of rIX-FP Prophylaxis at Dosing Intervals of up to 14 Days in a Pediatric Patient with Hemophilia B during the COVID-19 Pandemic [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/real-world-experience-of-rix-fp-prophylaxis-at-dosing-intervals-of-up-to-14-days-in-a-pediatric-patient-with-hemophilia-b-during-the-covid-19-pandemic/. Accessed May 20, 2022.

« Back to ISTH 2021 Congress

ISTH Congress Abstracts - https://abstracts.isth.org/abstract/real-world-experience-of-rix-fp-prophylaxis-at-dosing-intervals-of-up-to-14-days-in-a-pediatric-patient-with-hemophilia-b-during-the-covid-19-pandemic/

Simple Search

Supported By:

Bristol Myers Squibb and Pfizer

ISTH 2021 Congress site

Visit the official web site for the ISTH 2021 Virtual Congress »

  • Help & Support
  • About Us
  • Cookies & Privacy
  • Wiley Job Network
  • Terms & Conditions
  • Advertisers & Agents
Copyright © 2022 John Wiley & Sons, Inc. All Rights Reserved.
Wiley