PMID- 15029273
OWN - NLM
STAT- MEDLINE
DCOM- 20040422
LR  - 20191108
IS  - 0720-9355 (Print)
IS  - 0720-9355 (Linking)
VI  - 24
IP  - 1
DP  - 2004 Feb
TI  - Acquired von Willebrand syndrome 2004: International Registry--diagnosis and 
      management from online to bedside.
PG  - 50-5
AB  - The acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with 
      laboratory findings similar to those for congenital von Willebrand disease. 
      Unlike the congenital form, AVWS usually occurs in individuals with no personal 
      or family history of bleeding. Large studies on AVWS are not available, diagnosis 
      remains difficult and treatment empirical. Acquired von Willebrand syndrome is 
      especially frequent in lympho- or myeloproliferative disorders. It is associated 
      with solid tumours, immunological and cardiovascular disorders as well as other 
      miscellaneous conditions. Diagnosis of AVWS is based on assays measuring 
      ristocetin cofactor activity or collagen binding, which are usually abnormally 
      low, while factor VIII coagulant activity is some-times within the reference 
      range. FVIII/VWF inhibiting activities are found in only a minority of cases. 
      Bleeding episodes in patients with AVWS are mostly of the mucocutaneous type and 
      can be managed with desmopressin, plasma-derived factor VIII/von Willebrand 
      factor (FVIII/VWF) concentrates and intravenous immunoglobulin. Recombinant 
      activated factor VII can be useful in cases unresponsive to standard therapy. In 
      conclusion, the AVWS, although rare, is certainly underestimated in clinical 
      practice: The actual clinical impact of AVWS should be evaluated by prospective 
      studies. The authors are co-ordinating an updated version of the International 
      Registry on AVWS that will allow data to be entered directly online.
FAU - Federici, A B
AU  - Federici AB
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal 
      Medicine and Dermatology, IRCCS Maggiore Hospital and University of Milan, Via 
      Pace 9, 20122 Milano, Italy. Augusto.Federici@unimi.it
FAU - Budde, U
AU  - Budde U
FAU - Rand, J H
AU  - Rand JH
LA  - eng
PT  - Journal Article
PT  - Review
PL  - Germany
TA  - Hamostaseologie
JT  - Hamostaseologie
JID - 8204531
SB  - IM
MH  - Cardiovascular Diseases/complications
MH  - Diagnosis, Differential
MH  - Humans
MH  - Immune System Diseases/complications
MH  - Myeloproliferative Disorders/complications
MH  - Registries
MH  - von Willebrand Diseases/complications/diagnosis/*epidemiology/therapy
RF  - 62
EDAT- 2004/03/19 05:00
MHDA- 2004/04/23 05:00
CRDT- 2004/03/19 05:00
PHST- 2004/03/19 05:00 [pubmed]
PHST- 2004/04/23 05:00 [medline]
PHST- 2004/03/19 05:00 [entrez]
AID - 04010050 [pii]
AID - 10.1267/hamo04010050 [doi]
PST - ppublish
SO  - Hamostaseologie. 2004 Feb;24(1):50-5. doi: 10.1267/hamo04010050.