Evaluation of the MCMDM-1 VWD Bleeding Questionnaire score for the detection of coagulation abnormalities in an outpatient setting

Introduction The condensed MCMDM-1 Bleeding Questionnaire score has originally been designed to investigate for type 1 von Willebrand Disease (VWD). However, its diagnostic utility in detecting other hemorrhagic disorders is unknown. Since patients with a suspected bleeding diathesis usually undergo an extensive laboratory assessment including platelet function tests and measurement of coagulation factor activities, we wanted to assess the association between MCMDM-1 VWD bleeding score and other laboratory parameters.

Method We conducted a retrospective study at the University Medical Center Hamburg-Eppendorf, Germany. Patients presented to our department between January 2012 and December 2022 with a suspected bleeding diathesis. Patients receiving anticoagulant or antiplatelet therapy were excluded. Clinical and laboratory datawere retrieved fromelectronic patient charts. MCMDM-1 VWD bleeding score higher than three was defined as pathologic.

Results In total, 1806 patients (median age: 39 years) were analyzed. Seventy-nine percent (n=1431) were female. A bleeding score>3 was detected in 51% (n=929) of patients. An abnormal bleeding score was associated with lower levels of factor VIII (FVIII) activity (127.2% vs 137.7%, p<0.001), von Willebrand factor (VWF) activity (98.98% vs 105%, p<0.005) and VWF antigen (103.33% vs 111.8%, p<0.001). However, frequency of manifest VWD (VWF:Ag or VWF:Ac<50%), VWF activity to antigen ratio<0.7, or FVIII deficiency did not differ between both groups. In contrast, patients with a bleeding score>3 had longer median closure times (CTs) in the platelet function analyzer (PFA-100®​) using the collagen/ADP or the collagen/epinephrine cartridges (101 sec vs 97 sec, p<0.001, and 146 sec vs 139 sec, p<0.001, respectively) and were more frequently found to have abnormal CT prolongation above the upper limit of each test system (23% vs 17%, p<0.005, and 40% vs 31%, p<0.001, respectively). There were no differences in platelet aggregation by different platelet agonists as assessed by light transmittance aggregometry (LTA) but lower levels of factor VII activity (94.5% vs 100.25, p<0.01) in patients with a bleeding score<3.

Conclusion An MCMDM-1 VWD bleeding score>3 is associated with lower VWF and FVIII levels but not with a higher detection rate of VWD or FVIII deficiency in outpatients presenting to a tertiary center for the diagnostic work-up of a suspected bleeding disorder. In contrast, CTs in the PFA-100®​ were longer and findings of abnormal CT prolongation indicating impaired primary hemostasis were more frequent in patients with a bleeding score>3.

Publication History

Article published online:
26 February 2024