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Semin Thromb Hemost 2016; 42(05): 483-497
DOI: 10.1055/s-0036-1579641
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

Emmanuel J. Favaloro
1   Departments of Clinical and Laboratory Haematology, Institute of Clinical Pathology and Medical Research and Westmead Hospital, Sydney Centres for Thrombosis and Haemostasis, Westmead, New South Wales, Australia
2   Pathology West, NSW Health Pathology, New South Wales, Australia
,
Leonardo Pasalic
1   Departments of Clinical and Laboratory Haematology, Institute of Clinical Pathology and Medical Research and Westmead Hospital, Sydney Centres for Thrombosis and Haemostasis, Westmead, New South Wales, Australia
2   Pathology West, NSW Health Pathology, New South Wales, Australia
,
Jennifer Curnow
1   Departments of Clinical and Laboratory Haematology, Institute of Clinical Pathology and Medical Research and Westmead Hospital, Sydney Centres for Thrombosis and Haemostasis, Westmead, New South Wales, Australia
› Author Affiliations
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Publication History

Publication Date:
05 May 2016 (online)

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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.

Keywords

von Willebrand disease - 2A VWD - 2M VWD - von Willebrand factor
 

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