PMID- 7578899
OWN - NLM
STAT- MEDLINE
DCOM- 19951228
LR  - 20190913
IS  - 0957-5235 (Print)
IS  - 0957-5235 (Linking)
VI  - 6
IP  - 6
DP  - 1995 Sep
TI  - Acquired von Willebrand disease associated with multiple myeloma; 
      characterization of an inhibitor to von Willebrand factor.
PG  - 561-6
AB  - Acquired von Willebrand disease (vWD) has been described in a few patients with 
      multiple myeloma. The present study characterizes an inhibitor of von Willebrand 
      factor (vWF) isolated from a patient with multiple myeloma (IgG-kappa). 
      Multimeric analysis of vWF from this patient's plasma showed a reduction in 
      multimers of all sizes. The inhibitor (IgG) detected only the vWF subunit from 
      plasma of normal individuals. It reacted with intact vWF subunit and a 39/34kDa 
      dispase-digested fragment of vWF (residues; Leu480/Val481-Gly718), but did not 
      react with platelet membrane glycoproteins (GPs) or adhesive proteins. The 
      binding of vWF to GPIb mediated by ristocetin and by botrocetin was inhibited by 
      the patient's IgG with an IC50s of 0.3 mg/ml and 0.48 mg/ml, respectively. The 
      platelet aggregation induced by ristocetin or botrocetin was also inhibited by 
      the IgG. These results indicate that this inhibitor may recognize the binding 
      region of vWF to GPIb. Therefore, the antibody to vWF appears to represent the 
      likely pathophysiological mechanism responsible for the acquired vWD in this 
      patient.
FAU - Mohri, H
AU  - Mohri H
AD  - First Department of Internal Medicine, Yokohama City University School of 
      Medicine, Japan.
FAU - Tanabe, J
AU  - Tanabe J
FAU - Ohtsuka, M
AU  - Ohtsuka M
FAU - Yoshida, M
AU  - Yoshida M
FAU - Motomura, S
AU  - Motomura S
FAU - Nishida, S
AU  - Nishida S
FAU - Fujimura, Y
AU  - Fujimura Y
FAU - Okubo, T
AU  - Okubo T
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - England
TA  - Blood Coagul Fibrinolysis
JT  - Blood coagulation & fibrinolysis : an international journal in haemostasis and 
      thrombosis
JID - 9102551
RN  - 0 (Autoantibodies)
RN  - 0 (Crotalid Venoms)
RN  - 0 (Immunoglobulin G)
RN  - 0 (Macromolecular Substances)
RN  - 0 (Platelet Membrane Glycoproteins)
RN  - 0 (von Willebrand Factor)
RN  - 1404-55-3 (Ristocetin)
RN  - 85537-36-6 (botrocetin)
SB  - IM
MH  - Autoantibodies/*blood
MH  - Binding Sites
MH  - Blotting, Western
MH  - Crotalid Venoms/pharmacology
MH  - Humans
MH  - Immunoglobulin G/blood/pharmacology
MH  - Macromolecular Substances
MH  - Male
MH  - Middle Aged
MH  - Multiple Myeloma/*complications
MH  - Platelet Aggregation/drug effects
MH  - Platelet Membrane Glycoproteins/metabolism
MH  - Ristocetin/pharmacology
MH  - von Willebrand Diseases/blood/*etiology/*immunology
MH  - von Willebrand Factor/chemistry/immunology/metabolism
EDAT- 1995/09/01 00:00
MHDA- 1995/09/01 00:01
CRDT- 1995/09/01 00:00
PHST- 1995/09/01 00:00 [pubmed]
PHST- 1995/09/01 00:01 [medline]
PHST- 1995/09/01 00:00 [entrez]
AID - 10.1097/00001721-199509000-00010 [doi]
PST - ppublish
SO  - Blood Coagul Fibrinolysis. 1995 Sep;6(6):561-6. doi: 
      10.1097/00001721-199509000-00010.