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Semin Thromb Hemost 2017; 43(05): 530-539
DOI: 10.1055/s-0036-1597289
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Bleeding Scores for the Diagnosis of von Willebrand Disease

Mackenzie L. Bowman
1   Department of Medicine, Queen's University, Kingston, Ontario, Canada
,
Paula D. James
1   Department of Medicine, Queen's University, Kingston, Ontario, Canada
› Author Affiliations
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Publication History

Publication Date:
14 February 2017 (online)

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Abstract

Obtaining a personal history of bleeding is a critical component to the diagnosis of von Willebrand disease (VWD). The collection of this information can be challenging for physicians, however, as the reporting and interpretation of bleeding symptoms is subjective. The need for more precise quantification of bleeding symptoms was recognized and the Vicenza Bleeding Questionnaire was developed in 2005. This questionnaire collects data regarding the presence and severity of bleeding symptoms and generates a bleeding score by summing the severity of all symptoms reported by a patient. Several subsequent bleeding assessment tools (BATs) have been developed based from this original questionnaire and there has been a surge in the use of BATs in various clinical settings for the diagnosis and evaluation of VWD. This review will discuss the evolution of BATs over the past decade, as well as their use and validation in various settings for the diagnosis and evaluation of VWD. Additionally, we will discuss the clinical utility of BATs, the limitations of these tools, and future directions.

Keywords

bleeding questionnaire - bleeding score - von Willebrand disease
 

  • References

  • 1 Sadler JE. Von Willebrand disease type 1: a diagnosis in search of a disease. Blood 2003; 101 (06) 2089-2093
    CrossrefPubMedGoogle Scholar
  • 2 Mauer AC, Khazanov NA, Levenkova N. , et al. Impact of sex, age, race, ethnicity and aspirin use on bleeding symptoms in healthy adults. J Thromb Haemost 2011; 9 (01) 100-108
    CrossrefPubMedGoogle Scholar
  • 3 Srámek A, Eikenboom JC, Briët E, Vandenbroucke JP, Rosendaal FR. Usefulness of patient interview in bleeding disorders. Arch Intern Med 1995; 155 (13) 1409-1415
    CrossrefPubMedGoogle Scholar
  • 4 Sadler JE, Rodeghiero F. ; ISTH SSC Subcommittee on von Willebrand Factor. Provisional criteria for the diagnosis of VWD type 1. J Thromb Haemost 2005; 3 (04) 775-777
    CrossrefPubMedGoogle Scholar
  • 5 Rodeghiero F, Castaman G, Tosetto A. , et al. The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study. J Thromb Haemost 2005; 3 (12) 2619-2626
    CrossrefPubMedGoogle Scholar
  • 6 Tosetto A, Rodeghiero F, Castaman G. , et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4 (04) 766-773
    CrossrefPubMedGoogle Scholar
  • 7 Bowman M, Mundell G, Grabell J. , et al. Generation and validation of the Condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease. J Thromb Haemost 2008; 6 (12) 2062-2066
    CrossrefPubMedGoogle Scholar
  • 8 Tosetto A, Castaman G, Plug I, Rodeghiero F, Eikenboom J. Prospective evaluation of the clinical utility of quantitative bleeding severity assessment in patients referred for hemostatic evaluation. J Thromb Haemost 2011; 9 (06) 1143-1148
    CrossrefPubMedGoogle Scholar
  • 9 Azzam HA, Goneim HR, El-Saddik AM, Azmy E, Hassan M, El-Sharawy S. The condensed MCMDM-1 VWD bleeding questionnaire as a predictor of bleeding disorders in women with unexplained menorrhagia. Blood Coagul Fibrinolysis 2012; 23 (04) 311-315
    CrossrefPubMedGoogle Scholar
  • 10 Rodeghiero F, Tosetto A, Abshire T. , et al; ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost 2010; 8 (09) 2063-2065
    CrossrefPubMedGoogle Scholar
  • 11 Elbatarny M, Mollah S, Grabell J. , et al; Zimmerman Program Investigators. Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project. Haemophilia 2014; 20 (06) 831-835
    CrossrefPubMedGoogle Scholar
  • 12 Lowe GC, Lordkipanidzé M, Watson SP. ; UK GAPP study group. Utility of the ISTH bleeding assessment tool in predicting platelet defects in participants with suspected inherited platelet function disorders. J Thromb Haemost 2013; 11 (09) 1663-1668
    CrossrefPubMedGoogle Scholar
  • 13 Kaur H, Borhany M, Azzam H, Costa-Lima C, Ozelo M, Othman M. The utility of International Society on Thrombosis and Haemostasis-Bleeding Assessment Tool and other bleeding questionnaires in assessing the bleeding phenotype in two platelet function defects. Blood Coagul Fibrinolysis 2016; 27 (05) 589-593
    CrossrefPubMedGoogle Scholar
  • 14 Deforest M, Grabell J, Albert S. , et al. Generation and optimization of the self-administered bleeding assessment tool and its validation as a screening test for von Willebrand disease. Haemophilia 2015; 21 (05) e384-e388
    CrossrefPubMedGoogle Scholar
  • 15 Katsanis E, Luke KH, Hsu E, Li M, Lillicrap D. Prevalence and significance of mild bleeding disorders in children with recurrent epistaxis. J Pediatr 1988; 113 (1, Pt 1): 73-76
    CrossrefPubMedGoogle Scholar
  • 16 Dean JA, Blanchette VS, Carcao MD. , et al. von Willebrand disease in a pediatric-based population--comparison of type 1 diagnostic criteria and use of the PFA-100 and a von Willebrand factor/collagen-binding assay. Thromb Haemost 2000; 84 (03) 401-409
    Article in Thieme ConnectPubMedGoogle Scholar
  • 17 Hedlund-Treutiger I, Revel-Vilk S, Blanchette VS, Curtin JA, Lillicrap D, Rand ML. Reliability and reproducibility of classification of children as “bleeders” versus “non-bleeders” using a questionnaire for significant mucocutaneous bleeding. J Pediatr Hematol Oncol 2004; 26 (08) 488-491
    CrossrefPubMedGoogle Scholar
  • 18 Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD. Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost 2009; 7 (08) 1418-1421
    CrossrefPubMedGoogle Scholar
  • 19 Biss TT, Blanchette VS, Clark DS. , et al. Quantitation of bleeding symptoms in children with von Willebrand disease: use of a standardized pediatric bleeding questionnaire. J Thromb Haemost 2010; 8 (05) 950-956
    CrossrefPubMedGoogle Scholar
  • 20 Marcus PD, Nire KG, Grooms L, Klima J, O'Brien SH. The power of a standardized bleeding score in diagnosing paediatric type 1 von Willebrand's disease and platelet function defects. Haemophilia 2011; 17 (02) 223-227
    CrossrefPubMedGoogle Scholar
  • 21 Bidlingmaier C, Grote V, Budde U, Olivieri M, Kurnik K. Prospective evaluation of a pediatric bleeding questionnaire and the ISTH bleeding assessment tool in children and parents in routine clinical practice. J Thromb Haemost 2012; 10 (07) 1335-1341
    CrossrefPubMedGoogle Scholar
  • 22 Mittal N, Naridze R, James P, Shott S, Valentino LA. Utility of a Paediatric Bleeding Questionnaire as a screening tool for von Willebrand disease in apparently healthy children. Haemophilia 2015; 21 (06) 806-811
    CrossrefPubMedGoogle Scholar
  • 23 Sanders YV, Fijnvandraat K, Boender J. , et al; WiN Study Group. Bleeding spectrum in children with moderate or severe von Willebrand disease: relevance of pediatric-specific bleeding. Am J Hematol 2015; 90 (12) 1142-1148
    CrossrefPubMedGoogle Scholar
  • 24 Bujnicki HC, Sidonio RF, Kempton C. , et al. Screening for von Willebrand disease in children: a case-control study. J Thromb Haemost 2011; 9 (05) 1086-1089
    CrossrefPubMedGoogle Scholar
  • 25 Malec LM, Moore CG, Bennett CM. , et al. Validation study of the composite score to identify Von Willebrand disease in children. J Pediatr Hematol Oncol 2016; 38 (02) 139-142
    CrossrefPubMedGoogle Scholar
  • 26 Federici AB, Bucciarelli P, Castaman G. , et al. The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease. Blood 2014; 123 (26) 4037-4044
    CrossrefPubMedGoogle Scholar
  • 27 Trossaert M, Lienhart A, Nougier C. , et al. Diagnosis and management challenges in patients with mild haemophilia A and discrepant FVIII measurements. Haemophilia 2014; 20 (04) 550-558
    CrossrefPubMedGoogle Scholar
  • 28 Lotta LA, Maino A, Tuana G. , et al. Prevalence of disease and relationships between laboratory phenotype and bleeding severity in platelet primary secretion defects. PLoS One 2013; 8 (04) e60396
    CrossrefPubMedGoogle Scholar
  • 29 Olsson A, Hellgren M, Berntorp E, Ljung R, Baghaei F. Clotting factor level is not a good predictor of bleeding in carriers of haemophilia A and B. Blood Coagul Fibrinolysis 2014; 25 (05) 471-475
    CrossrefPubMedGoogle Scholar
  • 30 James PD, Mahlangu J, Bidlingmaier C. , et al; Global Emerging Hemostasis Experts Panel (GEHEP). Evaluation of the utility of the ISTH-BAT in haemophilia carriers: a multinational study. Haemophilia 2016; 22 (06) 912-918
    CrossrefPubMedGoogle Scholar
  • 31 Bowman M, Tuttle A, Notley C. , et al; Association of Hemophilia Clinic Directors of Canada. The genetics of Canadian type 3 von Willebrand disease: further evidence for co-dominant inheritance of mutant alleles. J Thromb Haemost 2013; 11 (03) 512-520
    CrossrefPubMedGoogle Scholar