Preview

Russian journal of hematology and transfusiology

Advanced search

The use of a growth factor inhibitor in the treatment of recurrent bleeding in von Willebrand disease

https://doi.org/10.35754/0234-5730-2022-68-4-511-519

Abstract

Introduction. Von Willebrand disease (vWD) is a hereditary disorder of the blood coagulation system caused by a quantitative and/or qualitative defect of Willebrand factor (vWF), the pathogenetic principle of treatment of which is substitution therapy with combined concentrates of factor III and vWF. When bleeding foci of gastrointestinal angiodysplasia appear, hemostatic replacement therapy may not be effective.
Aim: to present a clinical observation of the cessation of bleeding from gastrointestinal angiodysplasia after the use of a growth factor inhibitor in a vWD patient.
Main fundings. A clinical case of treatment of a type III vWD patient with recurrent bleeding from foci of gastrointestinal dysplasia is presented. In order to stop bleeding, a course of therapy with a recombinant humanized monoclonal antibody targeting vascular endothelial growth factor (bevacizumab) was performed. 6 injections of the drug were administered, with a single dose being 400 mg. The interval between injections was 2 weeks. After bevacizumab therapy for 12 months, there were no cases of bleeding, although no hemostatic drugs were administered.

About the Authors

S. A. Shutov
National Medical Research Center for Hematology
Russian Federation

Shutov Sergey Alexandrovich, Dr. Sci. (Med.), Surgeon, Leading Researcher

AuthorID: 163127

125167

Moscow



K. I. Danishyan
National Medical Research Center for Hematology
Russian Federation

Karen I. Danishyan, Dr. Sci. (Med.), Head of the Department of Surgery

125167

Moscow



N. I. Zozulya
National Medical Research Center for Hematology
Russian Federation

Nadezhda I. Zozulya, Dr. Sci. (Med.), Head of Clinical and Diagnostic Department of Hematology and Hemostasis Disorders

125167

Moscow



V. A. Novikov
National Medical Research Center for Hematology
Russian Federation

Vyacheslav A. Novikov, Cand. Sci. (Med.), Head of the Department of Anesthesiology-Ranimatology with the offi ce of general anesthesia

125167

Moscow



V. E. Salnikov
National Medical Research Center for Hematology
Russian Federation

Vladislav E. Salnikov, surgeon

125167

Moscow



A. I. Glebova
National Medical Research Center for Hematology
Russian Federation

Anastasia I. Glebova, surgeon

125167

Moscow



References

1. Swami A., Kaur V. von Willebrand Disease: A Concise Review and Update for the Practicing Physician. Clin Appl Thromb Hemost. 2017; 23(8): 900–10. DOI: 10.1177/1076029616675969.

2. Papayan L.P. Willebrand’s disease: etiology, pathogenesis, specifi c laboratory diagnostics. Vestnik Gematologii. 2021; 17(4): 32–42 (In Russian).

3. Gralnick H.R., Williams S.B., Shafer B.C., Corash L. Factor VIII/von Willebrand Factor Binding to von Willebrand’s Disease Platelets. Blood. 1982; 60(2): 328–32.

4. Fogarty Н., Dearbhla D., O’Donnell J.S. New developments in von Willebrand disease. Br J Haematol. 2020; 191(3): 329–39. DOI: 10.1111/bjh.16681.

5. James P.D., Connell N.T., Ameer B., et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021; 5(1): 280–300. DOI: 10.1182/bloodadvances.2020003265.

6. Leebeek F.W., Eikenboom J.C. Von Willebrand’s Disease. N Engl J Med. 2016; 375(21): 2067–80. DOI: 10.1056/NEJMra1601561.

7. Chernova E.V. Willebrand factor.Vestnik Severo-Zapadnogo gosudarstvennogo meditsinskogo universiteta im. I.I. Mechnikova. 2018; 10(4): 73–80 (In Russian).

8. Leebeek F.W.G. New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome. HemaSphere. 2021; 5(6): e586. DOI: 10.1097/HS9.0000000000000586.

9. Randi A.M., Smith K.E., Castaman G. von Willebrand factor regulation of blood vessel formation. Blood. 2018; 132(2): 132–40. DOI: 10.1182/ blood-2018-01-769018

10. Simone J.V., Cornet J.A., Abildgaard C.F. Acquired von Willebrand’s syndrome in systemic lupus erythematosus. Blood. 1968; 31(6): 806–12. DOI: 10.1182/ blood.V31.6.806.806.

11. Panholzer B., Bajorat Т., Haneya А., Kowalski D., et al. Acquired von Willebrand syndrome in ECMO patients: A 3-year cohort study. Blood Cells Mol Dis. 2021; 87: 102526. DOI: 10.1016/j.bcmd.2020.102526.

12. Manfredi E., van Zaane B., Gerdes V E A, et al. Hypothyroidism and acquired von Willebrand’s syndrome: a systematic review. Haemophilia. 2008;14(3):423-33. DOI: 10.1111/j.1365-2516.2007.01642.x.

13. Vincentelli A., Susen S., Le Tourneau T., et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med. 2003; 349(4): 343–9. DOI: 10.1056/NEJMoa022831.

14. Mondal S., Hollander K.N., Ibekwe S.O., et al. Heyde Syndrome Pathophysiology and Perioperative Implications. J Cardiothorac Vasc Anesth. 2021; 35: 3331–9. DOI: 10.1053/j.jvca.2020.10.003.

15. Theis S., Turner S. Heyde Syndrome. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023.

16. Belov D.V., Garbuzenko D.V., Lukin O.P., Anufrieva S.S. Heyde syndrome as a rare cause of gastrointestinal bleeding in patients with aortic stenosis. Kardiovasculyarnaya terapiya I profi laktika. 2021; 20(1): 59–64 (In Russian).

17. Song A.B., Sakhuja R., Gracin N.M. Systemic bevacizumab for refractory bleeding and transfusion-dependent anemia in Heyde syndrome. Blood Adv. 2021; 5(19): 3850–4. DOI: 10.1182/bloodadvances.2021004810.

18. Theis S.R., Turner S.D. Heyde Syndrome. Treasure Island (FL): StatPearls Publishing; 2022. Available at: https://www.ncbi.nlm.nih.gov/books/NBK551625

19. Heyde E.C. Gastrointestinal Bleeding in Aortic Stenosis. N Engl J Med. 1958; 259: 196. DOI: 10.1056/NEJM195807242590416.

20. Warkentin T.E., Moore J.C., Morgan D.G. Aortic stenosis and bleeding gastrointestinal angiodysplasia: is acquired von Willebrand’s disease the link? Lancet. 1992; 340(8810): 35–7. DOI: 10.1016/0140-6736(92)92434-h.

21. Ibrahim H., Rondina M.T., Kleiman N.S. Von Willebrand factor and the aortic valve: Concepts that are important in the transcatheter aortic valve replacement era. Thromb Res. 2018; 170: 20–7. DOI: 10.1016/j.thromres.2018.07.028.

22. Kashchenko V. A., Nakatis Ya. А. Lodygin A.V., et al. Bleeding from foci of angiodysplasias of the gastrointestinal tract: diagnosis and treatment. Vestnik hirurgii im. I.I Grekova. 2018; 177(2): 91–94 (In Russian). DOI: 10.24884/0042-4625-2018-177-2-91-94.

23. Sadler J.E., Budde U., Eikenboom J.C., et al. Update on the pathophysiology and classifi cation of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost. 2006; 4(10): 2103–14. DOI: 10.1111/j.1538-7836.2006.02146.x.

24. Biguzzi E., Siboni S.M.,Peyvandi F. How I treat gastrointestinal bleeding in congenital and acquired von Willebrand disease. Blood. 2020; 136(10): 1125– 33.

25. Jackson C.S., Strong R. Gastrointestinal angiodysplasia diagnosis and management. Gastrointest Endosc Clin N Am. 2017; 27(1): 51–62. DOI: 10.1016/j. giec.2016.08.012.

26. Avtaeva Y.N., Melnikov I.S., Vasiliev S.A., Gabbasov Z.A. Role of von Willebrand

27. factor in hemostasis pathology.Aterotrombos. 2022;12(2):79–102. (In Russian). DOI: 10.21518/2307-1109-2022-12-2-79-102.

28. Zozulya N.I., Kumskova M.A., Svirin P.V., Mamaev A.N. Clinical Recommendations Willebrand’s Disease. https://apicr.minzdrav.gov.ru/api.ashx?op=GetClinrecPdf&id=123_5 (In Russian).

29. Dimitrios E. Angioectasias on the major and accessory duodenal papillae; a unique cause of recurrent bleed in a patient with von Willebrand’s disease. BMJ Case Rep. 2015; 2015: bcr2015210781. DOI: 10.1136/bcr-2015-210781

30. Gerstein E. С. Modern ideas about the transfer mechanisms of growth factor signals as a basis for effective molecularly directed antitumour therapy.Voprosi biologicheskoi, medtsinskoi I farmatevticheskoi chimii. 2007; 5(1): 4–9 (In Russian).

31. Chekhonin V. P. The role of VEGF in the development of neoplastic angiogenesis. Vestnik RAMN. 2012; 2: 23–34 (In Russian).

32. Van-Custem E, Rutgeerts P, Vantrappen G. Treatment of bleeding gastrointestinal malformations with oestrogen–progesterone. Lancet. 1990; 335: 953–5.

33. Jameson J.J., Cave D.R. Hormonal and antihormonal therapy for epistaxis in hereditary hemorrhagic telangiectasia. Laryngoscope. 2004; 114: 750–9.

34. Yaniv E., Preis M., Hadar T., et al. Antiestrogen therapy for hereditary hemorrhagic telangiectasia: a double-blind placebo-controlled clinical trial. Laryngoscope. 2009; 119: 284–8.

35. Yaniv E., Preis M., Shevro J., et al. Anti-estrogen therapy for hereditary hemorrhagic telangiectasia — a long-term clinical trial. Rhinology. 2011; 49: 214–6.

36. Thachil J., Hay C.R., Campbell S. Tamoxifen for recurrent bleeds due to angiodysplasia in von Willebrand’s disease. Haemophilia. 2013; 19(5): e313–5.

37. Kovalenko E.I., Manzyuk L.V., Kononenko I.B., Grebennikova O.P. Side effects of tamoxifen treatment with hormone therapy. Onkoginekologiya. 2018: 3(27): 16-22 (In Russian).

38. Vladimirova L.Y., Storozhakova A.E., Kalabanova E.A., et al. Experience of bevacizumab use in adjuvant therapy in patients with ovarian cancer. Yugno-Rossijskiy onkologicheskij gurnak 2020; 1(3):67–74 (In Russian).

39. Mitchell A., Adams L.A., MacQuillan G., et al. Bevacizumab reverses need for liver transplantation in hereditary hemorrhagic telengiectasia. Liver Transpl. 2008; 14: 210–3.

40. Flieger D., Hainke S., Fischbach W. Dramatic improvement in hereditary hemorrhagic telangiectasia after treatment with the vascular endothelial growth factor (VEGF) antagonist bevacizumab. Ann Hematol. 2006; 85: 631–2.

41. Al-Samkari H., Kasthuri R.S., Parambil J.G., et al. An international, multicenter study of intravenous bevacizumab for bleeding in hereditary hemorrhagic telangiectasia: the InHIBIT-Bleed study. Haematologica. 2021; 106(8): 2161–9.


Review

For citations:


Shutov S.A., Danishyan K.I., Zozulya N.I., Novikov V.A., Salnikov V.E., Glebova A.I. The use of a growth factor inhibitor in the treatment of recurrent bleeding in von Willebrand disease. Russian journal of hematology and transfusiology. 2023;68(4):511-519. (In Russ.) https://doi.org/10.35754/0234-5730-2022-68-4-511-519

Views: 536


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 0234-5730 (Print)
ISSN 2411-3042 (Online)