PMID- 32759569
OWN - NLM
STAT- MEDLINE
DCOM- 20200924
LR  - 20200924
IS  - 0485-1439 (Print)
IS  - 0485-1439 (Linking)
VI  - 61
IP  - 7
DP  - 2020
TI  - [Acquired von Willebrand syndrome].
PG  - 809-817
LID - 10.11406/rinketsu.61.809 [doi]
AB  - Von Willebrand disease (VWD) is among the most common inherited bleeding 
      disorders. Interestingly, acquired von Willebrand syndrome (AVWS) is diagnosed 
      much less frequently, but can be identified in association with a substantial 
      number of medical conditions and diseases, including lymphoproliferative (48%), 
      cardiovascular (21%), myeloproliferative (15%), other neoplastic (5%), and 
      autoimmune disorders (2%). Most recently, AVWS has been diagnosed in patients 
      with aortic valve stenosis (AS, 79%) and continuous-flow left ventricular assist 
      devices (LVAD, up to 100%).1) Immune mechanisms mediated reduction of VWF 
      activityAutoantibodies to VWF have been identified in association with monoclonal 
      gammopathies, lymphoid, neoplasms, and autoimmune diseases. Some autoantibodies 
      have higher affinity to high molecular weight-VWF multimers (HMW-VWFMs); 
      clearance of HMW-VWFMs leads to bleeding.2) Non-immune mechanisms induced 
      reduction of VWF activityOne of the mechanisms is VWF adsorption onto malignant 
      cells and paraproteins (i.e., as in multiple myeloma) and thereby removed from 
      the blood circulation. VWF-linked proteolysis can be induced by shear stress. 
      According to high levels of shear stress in AS and LVAD, HM-VWFMs are more 
      susceptible to cleavage by ADAMTS13. We will find a large number of AVWS cases 
      related to cardiovascular diseases.
FAU - Hayakawa, Masaki
AU  - Hayakawa M
AD  - Department of Transfusion Medicine, Nara Medical University.
FAU - Matsumoto, Masanori
AU  - Matsumoto M
AD  - Department of Transfusion Medicine, Nara Medical University.
LA  - jpn
PT  - Journal Article
PL  - Japan
TA  - Rinsho Ketsueki
JT  - [Rinsho ketsueki] The Japanese journal of clinical hematology
JID - 2984782R
RN  - 0 (von Willebrand Factor)
SB  - IM
MH  - Hemorrhage
MH  - Humans
MH  - Multiple Myeloma
MH  - Paraproteinemias
MH  - *von Willebrand Diseases
MH  - von Willebrand Factor
OTO - NOTNLM
OT  - ADAMTS13
OT  - Aortic stenosis
OT  - Polycythemia vera (PV)
OT  - von Willebrand factor
EDAT- 2020/08/08 06:00
MHDA- 2020/09/25 06:00
CRDT- 2020/08/08 06:00
PHST- 2020/08/08 06:00 [entrez]
PHST- 2020/08/08 06:00 [pubmed]
PHST- 2020/09/25 06:00 [medline]
AID - 10.11406/rinketsu.61.809 [doi]
PST - ppublish
SO  - Rinsho Ketsueki. 2020;61(7):809-817. doi: 10.11406/rinketsu.61.809.