TY - JOUR
T1 - Type 2M and Type 2A von Willebrand disease
T2 - Similar but different
AU - Favaloro, Emmanuel J
AU - Pasalic, Leonardo
AU - Curnow, Jennifer
N1 - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
PY - 2016/7
Y1 - 2016/7
N2 - 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.
AB - 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.
KW - Humans
KW - von Willebrand Disease, Type 2/blood
KW - von Willebrand Factor/genetics
U2 - 10.1055/s-0036-1579641
DO - 10.1055/s-0036-1579641
M3 - Review article
C2 - 27148841
SN - 0094-6176
VL - 42
SP - 483
EP - 497
JO - Seminars in Thrombosis and Hemostasis
JF - Seminars in Thrombosis and Hemostasis
IS - 5
ER -