As a part of this very special issue of Seminars in Thrombosis & Hemostasis, we have provided access through this Appendix to four articles previously published
in this journal.[1]
[2]
[3]
[4]
These articles hold special relevance to this issue of the journal, as extensively
highlighted within the Preface to this issue. Seminars in Thrombosis & Hemostasis began publishing 40 years ago in 1974 under the stewardship of Eberhard F. Mammen,
the founding Editor-in-Chief of the journal.
In a fitting tribute to Dr. Mammen, we began this celebratory issue with a new contribution
on the topic of “Sticky platelet syndrome”, as this topic was of major interest to
him, as evidenced by both the new contribution as well as by the reproduction of one
of his articles on this topic in this journal in 1999.[1]
Several contributions within this issue of the journal are related to von Willebrand
factor (VWF), Factor VIIII (FVIII), von Willebrand disease (VWD), and hemophilia,
and to various interrelationships. VWD is reportedly the most common bleeding disorder,
and arises as a deficiency or defect in VWF. Sufferers of severe VWD, particularly
type 3 VWD, also suffer from loss of FVIII, given that one role of VWF is to bind
to and protect FVIII from degradation. Classical hemophilia is caused by the lack
of specific coagulation factors, and notably hemophilia A is caused by a lack of FVIII.
The historical context to what we now know to be true regarding these disorders and
the proteins involved took several decades to evolve, and there was once considerable
confusion regarding the relationship between the linked but separate molecules FVIII
and VWF, and the respective disorders of hemophilia A and VWD. Some of this historical
context is therefore highlighted within the Appendix with several historical reproductions.[2]
[3]
Finally, we are now at cross-roads in hemophilia care, given the development of new
and improved therapies. Nevertheless, universal access to care still remains imperfect,
even today. Accordingly, the final historical piece within the Appendix[4] has been included to provide some historical context to life in the hemophilia world
of the 1990s.
I hope that the readership of this journal finds these reproductions of interest.