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von Willebrand disease type 2B

Eric McGinnis and Suzanne M. Vercauteren

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  • RE: Type 2B von Willebrand disease diagnosed from peripheral blood smear?
    • Karthik Bommannan B.K., D.M. Hematopathology senior resident Postgraduate Institute of Medical Education and Research, Chandigarh, India.

    Respected Editor,
    It was fascinating to read at the ‘Blood work’ published by Eric McGinnis and Suzanne M. Vercauteren. The authors have described a neonate with spurious thrombocytopenia resulting from platelet aggregation in peripheral blood film. A presumptive diagnosis of type 2B von Willebrand disease (vWD) was suggested as the neonate had a positive family history for this dominantly inherited disease. The authors’ claim that physiologic increase in high molecular weight von Willebrand multimers resulting in thrombocytopenia is well taken and it highlights the uniqueness of neonatal hemostasis. In the era of molecular diagnostics, the manuscript underlines the importance of clinical history and morphologic analysis.
    However, few questions remain unanswered in the manuscript: 1. whether the thrombocytopenia was persistent?, 2. whether common causes for platelet aggregation like sampling errors and EDTA induced platelet aggregation (prevalence ranging from 0.1-2% in hospitalized patients to 15-17% in outpatients evaluated for isolated thrombocytopenia) have been excluded ?[1]. Type 2B vWD, resulting from gain of function mutations in the A2 domain of vWF accounts for 5-8% of vWD [2]. The diagnosis requires a detailed workup for vWF antigen levels, vWF: Ristocetin cofactor (RCo) activity, vWF activity: antigen ratio of <0.6, low dose ristocetin induced platelet agglutination, vWF multimer analysis and mutational analysis for confirmation. It is importa...

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    Conflict of Interest:
    None declared.