Semin Thromb Hemost 2011; 37(5): 568-575
DOI: 10.1055/s-0031-1281044
© Thieme Medical Publishers

Diagnosis and Management of von Willebrand Disease in a Single Institution of Argentina

Adriana I. Woods1 , 2 , Analía Sánchez-Luceros1 , 2 , Susana S. Meschengieser1 , Ana C. Kempfer1 , 2 , Alicia N. Blanco1 , María A. Lazzari1 , 2
  • 1Instituto de Investigaciones Hematológicas “Mariano R Castex,” Academia Nacional de Medicina de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
  • 2Consejo Nacional de Investigaciones (CONICET), Argentina
Further Information

Publication History

Publication Date:
18 November 2011 (online)

ABSTRACT

von Willebrand disease (VWD) is a bleeding disorder with variable clinical expression. In this article we describe types, clinical features, genetic testing when needed, genotype/phenotype relationships, and the response to desmopressin (DDAVP) testing, according to our experience. Our findings are possible type 1, 69.6%; type 1, 13.5%; severe type 1, 0 .35%; type 3, 0.55%; type 2A, 9.5%; probable 2B, 0.6%; type 2M, 2.5%; and probable type 2N, 3.4%. The most frequent symptoms are ecchymoses-hematomas and epistaxis, and, in females >over 13 years also menorrhagia. In pregnant patients, assessment of laboratory parameters in months 7 and 8 is recommended to plan the need for prophylaxis at term. DDAVP merits to be considered as the first-choice therapy, including pregnant women and children, and no patient showed significant unwanted effects. Because this is a safe, effective, and affordable therapy, we hope to encourage clinicians, mainly pediatricians and obstetricians, to a wider use of DDAVP, especially in developing countries. We also report two patients with prophylactic treatment.

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Adriana Inés WoodsM.S. 

Instituto de Investigaciones Hematológicas “Mariano R Castex,” Academia Nacional de Medicina de Buenos Aires

Pacheco de Melo 3081, Ciudad Autónoma de Buenos Aires, (C1425AUM), Argentina

Email: aiwoods@hematologia.anm.edu.ar