PMID- 1415174
OWN - NLM
STAT- MEDLINE
DCOM- 19921026
LR  - 20190819
IS  - 0361-8609 (Print)
IS  - 0361-8609 (Linking)
VI  - 41
IP  - 2
DP  - 1992 Oct
TI  - Effectiveness of high-dose intravenous immunoglobulin in a case of acquired von 
      Willebrand syndrome with chronic melena not responsive to desmopressin and factor 
      VIII concentrate.
PG  - 132-6
AB  - A patient with benign monoclonal IgG lambda paraproteinemia, acquired von 
      Willebrand syndrome (AVWS), and chronic melena successfully responding to 
      high-dose intravenous immunoglobulin (lvlg) is reported. Coagulation parameters 
      at admission were APTT (ratio) 1.68; VIII:C 11 IU/dL; vWF:Ag 7 IU/dL:Ricof less 
      than 3 IU/dl. RIPA was greater than 1.8 mg/ml, and bleeding time (BT) was 
      prolonged (18 min). No evidence for an in vitro inhibitor against the VIII/vWF 
      complex was observed. VIII/vWF measurements showed a short-lived increase after 
      both DDAVP and Hemate P, and BT was transiently normalized. After intravenous Ig 
      (1 g/kg for 2 days), VIII/vWF measurements, hemostatic parameters and multimeric 
      pattern were completely corrected (VIII/C 106 IU/dl, vWF:Ag 168 IU/dl, RiCof 147 
      IU/dl, APTT ratio 0.89, BT 5'), with a return to pre-infusion values after 15 
      days. Hemoccult test became negative and packed red cell transfusions, of which 
      130 units were administered during the last year, were no longer required. After 
      18 months the patient is on maintenance treatment with repeated courses of Ig, at 
      3 to 4-week intervals based on VIII/vWF and BT monitoring.
FAU - Castaman, G
AU  - Castaman G
AD  - Department of Hematology, San Bortolo Hospital, Vicenza, Italy.
FAU - Tosetto, A
AU  - Tosetto A
FAU - Rodeghiero, F
AU  - Rodeghiero F
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Am J Hematol
JT  - American journal of hematology
JID - 7610369
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 0 (von Willebrand Factor)
RN  - 9001-27-8 (Factor VIII)
RN  - ENR1LLB0FP (Deamino Arginine Vasopressin)
SB  - IM
MH  - Bleeding Time
MH  - Chronic Disease
MH  - Deamino Arginine Vasopressin/*therapeutic use
MH  - Dose-Response Relationship, Drug
MH  - Factor VIII/analysis/*therapeutic use
MH  - Humans
MH  - Immunoelectrophoresis
MH  - Immunoglobulins, Intravenous/*therapeutic use
MH  - Male
MH  - Melena/complications/*drug therapy
MH  - Middle Aged
MH  - Paraproteinemias/complications/drug therapy
MH  - von Willebrand Diseases/complications/*drug therapy
MH  - von Willebrand Factor/analysis
EDAT- 1992/10/01 00:00
MHDA- 1992/10/01 00:01
CRDT- 1992/10/01 00:00
PHST- 1992/10/01 00:00 [pubmed]
PHST- 1992/10/01 00:01 [medline]
PHST- 1992/10/01 00:00 [entrez]
AID - 10.1002/ajh.2830410212 [doi]
PST - ppublish
SO  - Am J Hematol. 1992 Oct;41(2):132-6. doi: 10.1002/ajh.2830410212.