A ten-year experience with the diagnosis of von Willebrand disease in Mexico based on a cost-effective strategy

Background

Von Willebrand disease (VWD), is the most common inherited bleeding disorder worldwide, but its diagnosis is complicated, expensive, and poorly evaluated in several countries.

Objective

To report our long-term experience with the diagnosis of VWD based on a cost-effective strategy.

Methods

We studied 802 Mexican patients, men and women, children, and adults, with clinical suspicion of VWD. The following tests were performed: blood count, bleeding time, prothrombin time, activated partial thromboplastin time, fibrinogen concentration, VWF antigen, ristocetin cofactor activity, collagen binding assay, ristocetin-induced platelet aggregation, FVIII activity, and VWF multimers analysis.

Results

VWD was diagnosed in 639 patients; 582 had type 1 VWD (91.1%). Type 2 VWD was found in 52 patients (8.1%). Type 2A was present in 25 cases (48.1%), while types 2B and 2 M accounted for 21 (40.4) and six (11.5%) cases, respectively. Type 3 VWD was present in five patients (0.8%). The mean age of patients with VWD was 25.3 years (range: 2–71) for males and 22.1 (range: 1–54) for females. The diagnosis was inconclusive in 40 cases (5.0%) and was discarded in 123 (15.3%). Blood group O was the most common among patients with VWD.

Conclusion

Using a low-cost diagnostic strategy, we confirmed that VWD is as common in Mexico as in other countries. Review of the patient's history is mandatory when VWD is suspected, although laboratory confirmation may be difficult and expensive. The consequences of a lack of accurate and timely diagnosis affect the promptness and quality of treatment.