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Condensed MCMDM-1 VWD

 
Condensed MCMDM-1 VWD
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Condensed Molecular and Clinical Markers for the Diagnosis and Management of Type 1 (MCMDM-1) VWD Bleeding Questionnaire

Authors/developers

Bowman M, Mundell G, Grabell J, Hopman WH, Rapson D, Lillicrap D, James PD

Questionnaire and scoring key

N.B.: These scores are posted with the permission of the developers.

  • Description
  • Utility
  • Administration
  • Psychometrics
  • References

The condensed version of the MCMDM-1 VWD bleeding questionnaire can be used in the clinical setting to identify those with normal vs. abnormal bleeding symptoms and to determine the severity of bleeding. The target population includes patients with known VWD and those being evaluated for a possible bleeding disorder.

Contact person:
PD James: jamesp@queensu.ca

Date of this review: August 19, 2013
Updated: April, 2014

Overall utility:

  • Very useful in the clinical setting to distinguish between various bleeding disorders, severity of specific bleeding disorders, and ruling out a bleeding disorder, particularly VWD
  • Quick and easy to use

Limitations: Requires a skilled professional to administer

  1. Time to complete: 5–10 minutes
  2. Equipment/space required: None
  3. Training required: Expert-administered; no training specific to the administration of the scoring tool is required
  4. Cost: None
  5. Scoring/scaling/interpretation of results:
  • Scale: 3 categories are scored –1 to 4 (dental extraction, post-op, and post-partum), 7 are scored 0 to 4, GI is scored 0 to 3 and bruising is scored 0 to 2 (details as for MCMDM-1 VWD Bleeding Questionnaire score)
  • Possible range of total score: –3 to 45
  • Differentiating features as for MCMDM-1 VWD Bleeding Questionnaire score
  • Abnormal bleeding score (BS): >4

Psychometric properties:

1.  Construct validity:

Convergent validity

  • Higher bleeding score (BS) being inversely correlated to VWF:Ag, FVIII:C and VWF:RCo levels, bleeding time.1,2,3 
  • Comparing the Condensed MCMDM-1 VWD Questionnaire to the full MCMDM-1VWD Questionnaire showed a Spearman’s rho of 0.97 and an r2 of 96.4.1

Divergent validity

  • In those with a negative BS (i.e. <4): when challenged with a surgical procedure, none had significant bleeding.1

Group differences

  • With increased BS being associated with type 3 > type 2 > type 1 VWD.
  • BS discriminated between cases with VWD, undefined bleeding disorders, or mild platelet dysfunction and controls.
  • With increased BS being associated with type 3 as compared to type 1 VWD, obligate carriers, and unaffected family members.2

2.  Criterion validity:

  • The BS was significantly different between those with laboratory confirmed VWD and those who bled without having VWD and those without bleeding.1

3.  Reliability:

  • In the original study, 24 subjects (of the total 259) were administered the questionnaire by two different observers three months apart with an inter-observer Spearman’s rho of 0.72 and an intra-class correlation (ICC) of 0.805 (reasonable inter-observer reliability).1

4.  Responsiveness/sensitivity:

  • Responsiveness was excellent with an ROC-AUC of 0.96 for distinguishing affected and unaffected individuals based on having a positive BS (defined as ≥4).1
  • For type 1 VWD, the sensitivity was 100%, specificity was 87%, positive predictive value (PPV) was 0.20, and negative predictive value (NPV) was 1.
  • For the diagnosis of a mild bleeding disorder, the PPV was 0.71–0.78 and the NPV was 99%. The ROC-AUC was 0.63. Inclusion of positive BS with elevated aPTT (ratio >1.2) improved the ROC-AUC to 0.74 (which is considered adequate).
  • In women with menorrhagia, using a BS cut-off of 3.5, the sensitivity was 85%, specificity was 90%, PPV was 0.88, and NPV was 0.89 with an ROC-AUC of 0.91.3

Languages studied: English

Groups tested with this measure:

  • Community controls1,4
  • Type 1, 2A, 2B, 2M, 3 VWD1,2,4
  • Subjects being evaluated to rule out VWD1
  • Undefined bleeding disorders4,5
  • Mild platelet dysfunction disorders
  • Mild hemophilia
  • Factor XI deficiency
  • Women with menorrhagia3

Age: Children and adults

  1. Bowman M et al. Generation and validation of the condensed MCMDM-1VWD bleeding questionnaire for von Willebrand disease. J Thromb Haemost 2008; 6: 2062-2066.
  2. Bowman M et al. The genetics of Canadian type 3 von Willebrand disease: further evidence for co-dominant inheritance of mutant alleles. J Thromb Haemost 2013; 11: 512-520.
  3. Azzam HAG et al. The condensed MCMDM-1 VWD bleeding questionnaire as a predictor of bleeding disorders in women with unexplained menorrhagia. Blood Coagul Fibrinolysis 2012; 23: 311-315.
  4. Jackson SC et al. Suspected collagen disorders in the bleeding disorder clinic: A case–control study.Haemophilia 2013; 19: 246-250.
  5. Tosetto A et al. Prospective evaluation of the clinical utility of quantitative bleeding severity assessment in patients referred for hemostatic evaluation. J Thromb Haemost 2011; 9: 1143-1148.
  6. Rydz N, James P The evolution and value of bleeding assessment tools. J Thromb Haemost 2012; 10: 2223-2229.