A qualitative review of the literature regarding managing Von willebrand disease in obstetrics and gynaecology
DOI:
https://doi.org/10.18597/rcog.284Keywords:
von Willebrand disease, antifibrinolytics, tranexamic acid, epsilon aminocaproic acid, desmopressin, FVW/FVIII concentrate, obstetric and gynaecological haemorrhagesAbstract
Introduction: Von Willebrand disease (vWD) is a disorder of the coagulation, being more prevalent in fertile females and is thus the most frequently occurring genetic haemostasis disorder in obstetric and gynaecological practice.
Objective: reviewing the literature concerning vWD in obstetric and gynaecology patients, placing special emphasis on the disease’s physiopathology, diagnosis and treatment.
Methodology: a bibliographic search was made of MEDLINE electronic databases via pubmed, Elsevier, Interscience, EBSCO, Scopus and SciELO from 1980 to 2009. The Cochrane Pregnancy and Childbirth Group (September 30th 2009) and printed texts and books were also consulted.
Results: diagnosis was based on careful anamnesis and detection and confirmatory laboratory tests. Correct identification of different types and subtypes is important from a therapeutic point of view. Treatment requires specific medication such as desmopressin, factor VIII concentrates and coadjuvant therapies. No evidence was found to contraindicate vaginal birth route; however, each particular case must be taken individually.
Conclusion: females suffering from coagulation disorders present a greater risk of bleeding during menarche, pregnancy, giving birth and the puerperium. This means that suitable evaluation and multidisciplinary management must be mandatory during pregnancy.
Author Biography
Franklin J. Espitia-De La Hoz
References
Nilsson IM. Von Willebrand's disease from 1926-1983. Scand J Haematol 1984;33:S21-43.
Von Willebrand EA. Hereditar pseudohemofili. Finska Lakarsallskapets Handl 1926;67:7-112.
Nichols WC, Ginsburg D. Reviews in molecular medicine: Von Willebrand disease. Medicine 1997;76:1-20.
Ruggeri ZM. New insights into the mechanism of platelet adhesion and aggregation. Semin Hematol 1994;31:229-39.
Milaskievicz RM, Holcroft A, Letsky E. Epidural anaesthesia and von Willebrand's disease. Anaesthesia 1990;45:462-4.
Duerbeck NB, Chaffin DG, Coney P. Platelet and hemorragic disorders associated with pregnancy: a review. Part II. Obstet Gynecol Surv 1997;52:585-96.
Duflos-Cohade C, Bernier MO, Thibaud E. Métrorragies pubertaires et troubles de la coagulation. Rev Int Pédiatr 2001;32:15-8.
Kadir RA, Lukes AS, Kouides PA, Fernandez H, Goudemand J. Management of excessive menstrual bleeding in women with hemostatic disorders. Fertil Steril 2005;84:1352-9.
Furlan M, Robles R, Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vitro proteolysis. Blood 1997;87:4223-39.
Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996;87:4235-44.
Perutelli P, Biglino P, Mori PG. von Willebrand factor: biological function and molecular defects. Pediatr Hemat Oncol 1997;14:499-512.
Ruggeri ZM. Perspectives Series: cell adhesion in vascular biology. J Clin Invest 1997;99:559-64.
Cauwenberghs N, Schlammadinger A, Vauterin S, Cooper S, Descheemaeker G, Tornai I, et al. Fcreceptor dependent platelet aggregation induced by monoclonal antibodies against platelet glycoprotein Ib or von Willebrand factor. Thromb Haemostasis 2001;85:679-85.
Bloom AL. von Willebrand factor: clinical features of inherited and acquired disorders. Mayo Clin Proc 1991;66:743-51.
Gill JC, Endes-Brooks J, Bauer PJ, Marks WJ Jr, Montgomery RR. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987;69:1691-5.
Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood 1987;69:454-9.
Triplett DA. Laboratory diagnosis of von Willebrand's disease. Mayo Clin Proc 1991;66:832-40.
Favaloro EJ, Facey D, Grispo L.Laboratory assessment of von Willebrand factor. AJCP 1995;104:264-271.
Kouides PA. Obstetric and gynaecological aspects of von Willebrand disease. Best Pract Res Clin Haematol 2001;14:381-99.
Jones BP, Bell EA, Maroof M. Epidural labor analgesia in a parturient with von Willebrand´s disease type IIA and severe preeclampsia. Anesthesiology 1999;90:1219-20.
Kujovich JL. von Willebrand disease and pregnancy. J Thromb Haemost 2005;3:246-53.
Abildgaard CF, Suzuki Z, Harrison J, Jefcoat K, Zimmerman TS. Serial studies in von Willebrand's disease: variability versus "variants". Blood 1980;56:712-6.
Nichols WC, Ginsburg D. Von Willebrand disease. Medicine (Baltimore) 1997;76:1-20.
Werner EJ, Abshire TC, Giroux DS, Tucker EL, Broxson EH. Relative value of diagnostic studies for von Willebrand disease. J Pediatr 1992;21:34-8.
Nieuwenhuis HK, Akermann JW, Sixma JJ. Patients with a prolonged bleeding time and normal aggregation tests may have storage pool deficiency: studies on one hundred six patients. Blood 1987;70:620-3.
Quiroga T, Pérez M, Pereira J, Mezzano D. Diagnóstico de los subtipos de Enfermedad de Von Willebrand, mediante análisis de la composición multimérica del factor Von Willebrand plasmático. Rev. Médica Chile 1993, 121:987-93.
Sadler JE. A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1994;71:520-5.
Fressinaud E, Veyradier A, Truchaud F, Martin I, Boyer-Neuman C, Trossaert M, et al. Screening for von Willebrand disease with a new analyser using high shear stress: a study of 60 cases. Blood 1998;91:1325-31.
Blombäck M, Eneroth P, Andersson O, Anvret M. On laboratory problems in diagnosing mild von Willebrand's disease. Am J Hematol 1992;40:117-20.
George JN, Nurden AT, Phillips DR. Molecular defects in interactions of platelets with the vessel wall. N Eng J Med 1984;311:1084-98.
Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand factor. J Thromb Haemost 2006;4:2103-14.
Petrovitch CT, Drummond JC. Hemoterapiaehemostasia. En: Barash PG, Cullen BF, Stoelting RK. Anestesia clínica, 4a. ed, São Paulo: Manole; 2004. p. 201-38.
Sadler J. A revised classification of von Willebrand disease. Thromb Haemost 1994;71:520-5.
Sadler JE, Matshushita T, Dong Z, Tuley EA, Westfield LA. Molecular mechanism and classification of Von Willebrand disease. Thromb Haemost 1995;74:161-6.
Hoyer LW. Pseudo-von Willebrand's disease. N Eng J Med 1982;306:360-2.
Ruggeri ZM, Manucci PM, Lombardi R, Federici AB, Zimmerman TS. Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand`s disease subtypes. Blood 1982;59:1272-8.
Mannucci PM. How I treat patients with von Willebrand disease. Blood 2001; 97:1915-9.
Mannucci PM, Levi M. Prevention and treatment of major blood loss. N Engl J Med 2007;356:2301-11.
Tobias J. Strategies for minimizing blood loss in orthopedic surgery. Semin Hematol 2004;41:S145-56.
Ness PM. Pharmacologic alternatives to transfusion. Vox Sang 2002;83:S3-6.
Hickey M, Higham J, Fraser IS. Progestogens versus estrogens and progestogens for irregular uterine bleeding associated with anovulation (Cochrane review). Cochrane Library, Issue 3. Chichester, UK: John Wiley Sons; 2004.
Lyer V, Farquhar C, Jepson R. Oral contraceptive pills for heavy menstrual bleeding (Cochrane Review). En: The Cochrane Library. 2003;2. Oxford: Update Software.
Royal College of Obstetricians and Gynecologists. The initial management of menorrhagia. Evidence-based clinical guidelines No. 1. London: RCOG; October, 1998.
Beers MH, Berkow R. Anomalías de la Función Plaquetaria. En: Beers MH, Berkow R, editores. Manual Merck de Diagnóstico y Tratamiento. Madrid: Elsevier Science 1999; p. 931-2.
Bertholini DM, Butler CS. Severe hiponatraemia secondary to desmopressin therapy in von Willebrand´s disease. Anaesth Intensive Care 2000;28:199-201.
Lethagen S. Desmopressin (DDAVP) and hemostasis. Ann Hematol 1994; 69:173-80.
Mannucci PM. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years. Blood 1997;90:2515-21.
Mannucci PM. Desmopressin: a nontransfusional hemostatic agent. Annu Rev Med 1990;41:55-64.
Kessler CM. Current and future challenges of antithrombotic agents and anticoagulants: strategies for reversal of hemorrhagic complications. Semin Hematol 2004;41:S44-50.
Abshire TC. Dose optimization of recombinant factor VIIa for control of mild to moderate bleeds in inhibitor patients: improved efficacy with higher dosing. Seminars in Hematology 2004;41:S 3-7.
Kadir RA, Lukes AS, Kouides PA, Fernandez H, Goudemand J. Management of excessive menstrual bleeding in women with hemostatic disorders. Fertil Steril 2005; 84:1352-9.
Kadir RA, Lee CA, Sabin CA, Pollard D, Economides DL. Pregnancy in women with von Willebrand's disease or factor XI deficiency. Br J Obstet Gynaecol 1998;105:314-21.
Nitu-Whalley IC, Griffioen A, Harrington C, Lee CA. Retrospective review of the management of elective surgery with desmopressin and clotting factor concentrates in patients with von Willebrand disease. Am J Hematol 2001; 66:280-4.
Schmalenberger KP, Mandell GL, Golebiewski KA, Brett SH. Von Willebrand disease and regional anesthesia in the parturient. Reg Anesth Pain Med 2003;28:A2.
Flordal PA, Ljungström KG, Svensson J, Ekman B, Neander J. Effects on coagulation and fibrinolysis of desmopressin in patients undergoing total hip replacement. Thromb Haemost 1991;66:652-6.
Slaughter TF, Greenberg CS. Practice guidelines and treatment of patients with von Willebrand´s disease. Anesthesiology 1996;85:441-2.
Lillicrap D, Poon MC, Walker I, Xie F, Schwartz BA; Association of Hemophilia Clinic Directors of Canada. Efficacy and safety of the factor VIII/von Willebrand factor concentrate, haemate-P/humate-P: ristocetin cofactor unit dosing in patients with von Willebrand disease. Thromb Haemost 2002;87:224-30.
Berntorp E, Ekman M, Gunnarsson M, Nilsson IM. Variation in factor VIII inhibitory reactivity with different commercial factor VIII preparations. Haemophilia 1996;2:95-9.
Altisent C. Coagulopatías congénitas y gestación. Cap 8. En: Ramón Salinas I Argente (eds). Abordaje práctico de las alteraciones hematológicas en la mujer embarazada y en el neonato. 1a. ed. Madrid, España: Acción Médica SA; 2004. p. 115-32.
How to Cite
Downloads
Downloads
Published
Issue
Section
License
Copyright (c) 2010 Revista Colombiana de Obstetricia y Ginecología
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
| Article metrics | |
|---|---|
| Abstract views | |
| Galley vies | |
| PDF Views | |
| HTML views | |
| Other views | |
