Type 2M and Type 2A von Willebrand Disease: Similar but Different

Semin Thromb Hemost. 2016 Jul;42(5):483-97. doi: 10.1055/s-0036-1579641. Epub 2016 May 5.

Abstract

Analogous to the differentiation between hemophilia A and B, respectively, reflecting deficiency in factor VIII (FVIII) and FIX, and increasing being recognized as reflecting clinically different disorders, types 2A and 2M von Willebrand disease (VWD) can also be shown to express both similarities and differences in their prevalence, genetic defects, laboratory test results, clinical features, and treatment responses. In this narrative review, we explore these two "subtypes" of type 2 VWD, identifying parallels and dissimilarities in various aspects of their presentation to clinicians and to scientists/laboratories. This differential will become increasingly important as we strive to provide personalized approaches to future management of patients with VWD, particularly in the emerging landscape of recombinant von Willebrand factor.

Publication types

  • Review

MeSH terms

  • Humans
  • von Willebrand Disease, Type 2* / blood
  • von Willebrand Disease, Type 2* / classification
  • von Willebrand Disease, Type 2* / drug therapy
  • von Willebrand Disease, Type 2* / genetics
  • von Willebrand Factor* / genetics
  • von Willebrand Factor* / metabolism
  • von Willebrand Factor* / therapeutic use

Substances

  • von Willebrand Factor