PMID- 23252513
OWN - NLM
STAT- MEDLINE
DCOM- 20131025
LR  - 20211021
IS  - 1537-2995 (Electronic)
IS  - 0041-1132 (Linking)
VI  - 53
IP  - 8
DP  - 2013 Aug
TI  - Maintaining hemostasis in acquired von Willebrand syndrome: a review of 
      intravenous immunoglobulin and the importance of rituximab dose scheduling.
PG  - 1730-5
LID - 10.1111/trf.12017 [doi]
AB  - BACKGROUND: The acute management of acquired von Willebrand syndrome (AVWS) is 
      aimed at achieving hemostasis with von Willebrand factor replacement, 
      counteracting the pathologic antibodies with intravenous immunoglobulin (IVIG), 
      and supportive care with blood transfusions. However, strategies for the 
      long-term management of AVWS are not described, resulting in persistent use of 
      these acute strategies to achieve hemostasis via high utilization of blood 
      products. Herein, we provide an updated review of the use of IVIG and rituximab 
      for AVWS and present rituximab maintenance as an effective and durable strategy 
      for the management of these patients. CASE REPORT: We report the successful 
      treatment of AVWS with anti-CD20 monoclonal antibody therapy (375 mg/m2 rituximab 
      as four weekly doses followed by 375 mg/m2 every 90 days) in a patient with 
      concurrent monoclonal B-cell lymphocytosis allowing for the early discontinuation 
      of blood product support after only 2 g/kg IVIG achieved acute hemostasis 
      control. RESULTS: This is the first documentation of the successful long-term 
      management of AVWS without prolonged blood product or IVIG support. This result 
      contrasts sharply to previously reported rituximab strategies that were deemed 
      ineffective in AVWS. CONCLUSION: A maintenance regimen of rituximab may be an 
      effective long-term management strategy for AVWS associated with 
      lymphoproliferative disorders, which may minimize the use of blood products and 
      IVIG.
CI  - (c) 2012 American Association of Blood Banks.
FAU - Kanakry, Jennifer A
AU  - Kanakry JA
AD  - Department of Hematology, The Johns Hopkins Hospital, Baltimore, Maryland 21287, 
      USA. jenkanakry@jhmi.edu
FAU - Gladstone, Douglas E
AU  - Gladstone DE
LA  - eng
GR  - P01 CA015396/CA/NCI NIH HHS/United States
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20121217
PL  - United States
TA  - Transfusion
JT  - Transfusion
JID - 0417360
RN  - 0 (Antibodies, Monoclonal, Murine-Derived)
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 0 (Immunologic Factors)
RN  - 4F4X42SYQ6 (Rituximab)
SB  - IM
MH  - Aged, 80 and over
MH  - Antibodies, Monoclonal, Murine-Derived/*therapeutic use
MH  - Blood Transfusion
MH  - Combined Modality Therapy
MH  - Drug Administration Schedule
MH  - Hemostasis
MH  - Humans
MH  - Immunoglobulins, Intravenous/*therapeutic use
MH  - Immunologic Factors/*therapeutic use
MH  - Induction Chemotherapy
MH  - Maintenance Chemotherapy
MH  - Male
MH  - Rituximab
MH  - von Willebrand Diseases/blood/*drug therapy/therapy
EDAT- 2012/12/21 06:00
MHDA- 2013/10/26 06:00
CRDT- 2012/12/21 06:00
PHST- 2012/05/30 00:00 [received]
PHST- 2012/09/18 00:00 [revised]
PHST- 2012/10/05 00:00 [accepted]
PHST- 2012/12/21 06:00 [entrez]
PHST- 2012/12/21 06:00 [pubmed]
PHST- 2013/10/26 06:00 [medline]
AID - 10.1111/trf.12017 [doi]
PST - ppublish
SO  - Transfusion. 2013 Aug;53(8):1730-5. doi: 10.1111/trf.12017. Epub 2012 Dec 17.