PB0573 - Monthly prophylactic human fibrinogen concentrate (HFC) supplementation in Afibrinogenemia: Experience from a tertiary care centre in North India

Monday, June 24, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Co-Author(s)

MV

Mona Vijayaran

Assistant Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences Lucknow India
Lucknow, Uttar Pradesh, India
PN

Prisha Nankana

Senior Resident, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India
RK

Rajesh Kashyap

Professor & Head, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India

Presenting Author(s)

Dinesh Chandra, MD, DM

Associate Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India

Co-Author(s)

SY

Sanjeev Yadav

Associate Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India
RG

Ruchi Gupta

Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India
KR

Khaliqur Rahman

Additional Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India
MS

Manish Kumar Singh

Associate Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India

Background: An estimated prevalence of afibrinogenemia is approximately 1–2 per million [1]. Prevalence data from India are sparse [2,3]. As per Peyvandi et al [4] and Casini et al [5], 19% and 35% Afibrinogenemia patients were on fibrinogen prophylaxis respectively. They concluded that the optimal strategy (prophylactic vs. on-demand) for fibrinogen supplementation needs to be determined. So, with the availability of lower cost HFC (Fibrogen-I) in India, we planned for monthly prophylactic HFC replacement in Afibrinogenemia.

Aims: To assess the bleeding rates, cost effectiveness and feasibility of monthly prophylactic HFC supplementation in 2 paediatric Afibrinogenemia patients at SGPGIMS, Lucknow, India over a period of 6 months from June to November 2023

Methods: A total of 7 patients (2015 to 2023) with afibrinogenemia have been diagnosed at our centre. Fibrinogen concentrate (Fibrogen-I TM (50ml/1000mg) Intas Pharmaceuticals Limited, Ahmedabad, India-Image 1) is available in hospital pharmacy. Two patients (6year/Male, 8 day/Male; severe bleeding phenotype) received HFC supplementation monthly for 6 months. They were called on the same days and 1 vial of fibrinogen was divided between them, 6yr old received at 30 mg/kg , 750 mg/month; 8 days old at 60 mg/kg, 250 mg/month administered at the rate of 5ml/min (Figure 1)

Results: Both patients had no breakthrough bleeding on monthly prophylaxis. Looking at the cost effectiveness - 1 vial of Fibrogen-I costs 16,445 INR/1 g (200 USD). As the vial was shared between the 2 patients, it costed the government approximately 1 lakh INR (1200 USD) for a period of 6 months. No episode of thrombosis, allergic or hypersensitivity reaction was observed.

Conclusion(s): The management (prophylactic vs. on-demand) of Afibrinogenemia in resource constrained setting like India largely depends on availability and cost of the fibrinogen product (FFP vs. Cryoprecipitate vs. HFC). Monthly prophylactic HFC supplementation appears feasible but needs further validation with multicentric collaborations.