Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients

Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients Publisher Pubmed

Lissitchkov T¹ ; Madan B² ; Djambas Khayat C³ ; Zozulya N⁴ ; Ross C⁵ ; Karimi M⁶ ; Kavakli K⁷ ; De Angulo GR⁸ ; Almomen A⁹ ; Subramanian K¹⁰ ; Dsouza F⁵ ; Viswabandya A¹¹ ; Hoorfar H¹² ; Schwartz BA¹³ Show All Authors

Lissitchkov T¹
Madan B²
Djambas Khayat C³
Zozulya N⁴
Ross C⁵
Karimi M⁶
Kavakli K⁷
De Angulo GR⁸
Almomen A⁹
Subramanian K¹⁰
Dsouza F⁵
Viswabandya A¹¹
Hoorfar H¹²
Schwartz BA¹³
Solomon C¹⁴
Knaub S¹⁴
Peyvandi F^{15, 16}

Show Affiliations

1. Department of Hemorrhagic Diathesis and Anemia, Specialized Hospital for Active Treatment (SHAT) “Joan Pavel”, Sofia, Bulgaria
2. Centre for Haemostasis & Thrombosis, Guy's & St Thomas' NHS Foundation Trust, London, United Kingdom
3. Hotel Dieu De France, Beirut, Lebanon
4. Federal State-Funded Institution “National Research Center for Hematology” of the Ministry of Healthcare of the Russian Federation, Moscow, Russian Federation
5. Department of Hematology, St. John's Medical College & Hospital, Bangalore, India
6. Dastgheib Hospital, Shiraz, Iran
7. Children's Hospital, Ege University, Izmir, Turkey
8. Miami Children's Hospital, Miami, FL, United States
9. Centre of Excellence in Thrombosis & Hemostasis, College of Medicine and King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia
10. Sahyadri Specialty Hospital, Pune, India
11. Christian Medical College, Vellore, India
12. Seyed Al Shohada Hospital, Isfahan, Iran
13. Clinical Research & Development, Octapharma, Hoboken, NJ, United States
14. Research & Development Department, Octapharma, Lachen, Switzerland
15. Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
16. Department of Pathophysiology and Transplantation, Universita degli Studi di Milano, Milan, Italy

Source: Journal of Thrombosis and Haemostasis Published:2020

Abstract

Background: Congenital fibrinogen deficiency is an ultra-rare disorder in which patients can experience severe and/or frequent bleeding episodes (BEs). Here, we present the largest prospective study to date on the treatment of this disorder. Methods: Hemostatic efficacy of human fibrinogen concentrate (HFC; FIBRYGA®, Octapharma AG) for treatment of bleeding or surgical prophylaxis was assessed by investigators and adjudicated by an independent data monitoring and endpoint adjudication committee (IDMEAC) according to a four-point scale, using objective criteria. Thromboelastometry maximum clot firmness (MCF) was also determined. Results: Twenty-five afibrinogenemia patients were treated with HFC: 24 for on-demand treatment of 89 BEs, and nine as prophylaxis for 12 surgeries. For BEs, treatment success (rating of excellent or good) evaluated by investigators was 96.6% (90% confidence interval [CI], 0.92-0.99; two missing ratings, classified as failures) and by the IDMEAC was 98.9% (90% CI, 0.95-0.999). Mean ± standard deviation (SD) increase in MCF was 5.8 ± 2.5 mm one hour after the first HFC infusion (mean ± SD dose, 61.88 ± 11.73 mg/kg). For the 12 surgeries (median [range] HFC dose/surgery, 85.80 mg/kg [34.09-225.36]), intraoperative and postoperative treatment success were both rated 100% (90% CI, 0.82-1.00) by investigators and the IDMEAC. Three adverse events were possibly treatment related, including a moderate case of thrombosis. There were no deaths, no severe allergic or hypersensitivity reactions, and no clinical evidence of neutralizing antifibrinogen antibodies. Conclusions: Human fibrinogen concentrate was efficacious for on-demand treatment of bleeding and as surgical prophylaxis, with a favorable safety profile, in patients with congenital afibrinogenemia. © 2020 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals, Inc. on behalf of International Society on Thrombosis and Haemostasis

2. Fibrinolytic Therapy in Thrombosis of Mechanical Valves: Outcomes and Complications, ARYA Atherosclerosis (2023)

3. Comparison of Changes in Serum Fibrinogen Level in Primary Intracranial Hemorrhage (Ich) and Ischemic Stroke, ARYA Atherosclerosis (2012)

4. Carotid Intima-Media Thickness and Plasma Fibrinogen Among Subjects With Metabolic Syndrome: Isfahan Cohort Study, ARYA Atherosclerosis (2014)

5. Comparing the Efficacy of Synthetic Fibrinogen Plus Albumin to Fresh Frozen Plasma in the Prevention of Coagulation Disorders in Pediatric Heart Surgery, Journal of Isfahan Medical School (2016)

6. Evaluation of Plasma Fibrinogen Levels Before and After Coronary Artery Bypass Graft Surgery and Its Association With the Need for Blood Products, Advanced Biomedical Research (2022)

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Style	Citing Format
MLA	Lissitchkov T, et al.. "Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients." Journal of Thrombosis and Haemostasis, vol. 18, no. 4, 2020, pp. 815-824.
APA	Lissitchkov T, Madan B, Djambas Khayat C, Zozulya N, Ross C, Karimi M, Kavakli K, De Angulo GR, Almomen A, Subramanian K, Dsouza F, Viswabandya A, Hoorfar H, Schwartz BA, Solomon C, Knaub S, Peyvandi F (2020). Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients. Journal of Thrombosis and Haemostasis, 18(4), 815-824.
Chicago	Lissitchkov T, Madan B, Djambas Khayat C, Zozulya N, Ross C, Karimi M, Kavakli K, et al.. "Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients." Journal of Thrombosis and Haemostasis 18, no. 4 (2020): 815-824.
Harvard	Lissitchkov T et al. (2020) 'Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients', Journal of Thrombosis and Haemostasis, 18(4), pp. 815-824.
Vancouver	Lissitchkov T, Madan B, Djambas Khayat C, Zozulya N, Ross C, Karimi M, et al.. Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients. Journal of Thrombosis and Haemostasis. 2020;18(4):815-824.
BibTex	@article{ author = {Lissitchkov T and Madan B and Djambas Khayat C and Zozulya N and Ross C and Karimi M and Kavakli K and De Angulo GR and Almomen A and Subramanian K and Dsouza F and Viswabandya A and Hoorfar H and Schwartz BA and Solomon C and Knaub S and Peyvandi F}, title = {Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients}, journal = {Journal of Thrombosis and Haemostasis}, volume = {18}, number = {4}, pages = {815-824}, year = {2020} }
RIS	TY - JOUR AU - Lissitchkov T AU - Madan B AU - Djambas Khayat C AU - Zozulya N AU - Ross C AU - Karimi M AU - Kavakli K AU - De Angulo GR AU - Almomen A AU - Subramanian K AU - Dsouza F AU - Viswabandya A AU - Hoorfar H AU - Schwartz BA AU - Solomon C AU - Knaub S AU - Peyvandi F TI - Fibrinogen Concentrate for Treatment of Bleeding and Surgical Prophylaxis in Congenital Fibrinogen Deficiency Patients JO - Journal of Thrombosis and Haemostasis VL - 18 IS - 4 SP - 815 EP - 824 PY - 2020 ER -

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