PMID- 8172182
OWN - NLM
STAT- MEDLINE
DCOM- 19940602
LR  - 20190821
IS  - 0361-8609 (Print)
IS  - 0361-8609 (Linking)
VI  - 46
IP  - 2
DP  - 1994 Jun
TI  - Acquired von Willebrand's syndrome in association with a lupus-like anticoagulant 
      corrected by intravenous immunoglobulin.
PG  - 141-6
AB  - We are reporting on a 47-year-old man who presented with a prolongation of the 
      activated partial thromboplastin time (APTT) prior to orthopedic surgery. An 
      evaluation suggested an inhibitor when his plasma prolonged a normal control APTT 
      upon 50:50 solution of patients with normal plasma. The platelet-neutralizing 
      procedure (PNP), anticardiolipin antibody, and antinuclear antibody (ANA) were 
      positive. Further studies revealed decreased von Willebrand factor ristocetin 
      cofactor (vWF:RCoF), von Willebrand factor antigen (vWF:Ag), an inhibitor to vWF, 
      and absent high-molecular-weight vWF multimeters. Assays of FVIII:C, FIX, and FXI 
      were nonparallel to the standard curve. Intravenous immunoglobulin (IVIG) 
      corrected the APTT, multimeric pattern, and FVIII:C by the 7th day postinfusion. 
      This case demonstrates the efficacy of IVIG for acquired von Willebrand's 
      syndrome (vWS) and also represents a unique combination of a lupus-like 
      anticoagulant and acquired vWS in a patient without the full serological 
      requirement for systemic lupus erythematosus (SLE). Whether patients with 
      acquired vWS and lupus inhibitors are more or less susceptible to either a 
      thrombotic complication or hemorrhage is not established. Prospective studies for 
      the incidence of lupus inhibitor/antiphospholipid syndromes and vWF deficiencies 
      are needed to assess this question.
FAU - Hanley, D
AU  - Hanley D
AD  - Seton Hall University School of Graduate Medical Education, South Orange, New 
      Jersey.
FAU - Arkel, Y S
AU  - Arkel YS
FAU - Lynch, J
AU  - Lynch J
FAU - Kamiyama, M
AU  - Kamiyama M
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Hematol
JT  - American journal of hematology
JID - 7610369
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 0 (Lupus Coagulation Inhibitor)
RN  - 0 (von Willebrand Factor)
SB  - IM
MH  - Blood Coagulation
MH  - Humans
MH  - Immunoglobulins, Intravenous/therapeutic use
MH  - *Lupus Coagulation Inhibitor
MH  - Male
MH  - Middle Aged
MH  - Thrombosis/etiology
MH  - von Willebrand Diseases/complications/*etiology/therapy
MH  - von Willebrand Factor/chemistry
EDAT- 1994/06/01 00:00
MHDA- 1994/06/01 00:01
CRDT- 1994/06/01 00:00
PHST- 1994/06/01 00:00 [pubmed]
PHST- 1994/06/01 00:01 [medline]
PHST- 1994/06/01 00:00 [entrez]
AID - 10.1002/ajh.2830460216 [doi]
PST - ppublish
SO  - Am J Hematol. 1994 Jun;46(2):141-6. doi: 10.1002/ajh.2830460216.