PMID- 35086107
OWN - NLM
STAT- MEDLINE
DCOM- 20220721
LR  - 20221205
IS  - 1421-9662 (Electronic)
IS  - 0001-5792 (Print)
IS  - 0001-5792 (Linking)
VI  - 145
IP  - 4
DP  - 2022
TI  - Distinct Mechanisms of IgM Antibody-Mediated Acquired von Willebrand Syndrome and 
      Successful Treatment with Recombinant von Willebrand Factor in One Patient.
PG  - 454-457
LID - 10.1159/000522236 [doi]
AB  - Acquired von Willebrand Syndrome (AVWS) is a rare coagulation disorder which can 
      be associated with IgM paraproteinaemia. Recently, recombinant von Willebrand 
      factor (rVWF) has become available for the treatment of bleedings in patients 
      with inherited von Willebrand disease, but experience in patients with AVWS is 
      limited. We report on 2 patients with AVWS with underlying IgM paraproteinaemia 
      with distinct underlying pathomechanisms. In 1 patient, the paraprotein built 
      unspecific complexes with von Willebrand factor (VWF). In the other patient, we 
      were able to detect an IgM antibody against VWF. Bleeding in this patient was 
      successfully treated with rVWF. To our knowledge, this is the first report about 
      the successful use of rVWF in a patient with AVWS with the detection of a 
      VWF-specific antibody.
CI  - (c) 2022 The Author(s). Published by S. Karger AG, Basel.
FAU - Hopting, Matthias
AU  - Hopting M
AD  - Department of Hematology and Oncology, Internal Medicine III, University Hospital 
      Regensburg, Regensburg, Germany.
FAU - Budde, Ulrich
AU  - Budde U
AD  - Medilys Laborgesellschaft mbH, Asklepios Klinik Hamburg-Altona, Hamburg, Germany.
FAU - Tiede, Andreas
AU  - Tiede A
AD  - Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, 
      Hannover Medical School, Hannover, Germany.
FAU - Grube, Matthias
AU  - Grube M
AD  - Department of Hematology and Oncology, Internal Medicine III, University Hospital 
      Regensburg, Regensburg, Germany.
FAU - Hahn, Joachim
AU  - Hahn J
AD  - Department of Hematology and Oncology, Internal Medicine III, University Hospital 
      Regensburg, Regensburg, Germany.
FAU - Herr, Wolfgang
AU  - Herr W
AD  - Department of Hematology and Oncology, Internal Medicine III, University Hospital 
      Regensburg, Regensburg, Germany.
FAU - Heimerl, Susanne
AU  - Heimerl S
AD  - Institute of Clinical Chemistry and Laboratory Medicine, University Hospital 
      Regensburg, Regensburg, Germany.
FAU - Hart, Christina
AU  - Hart C
AD  - Department of Hematology and Oncology, Internal Medicine III, University Hospital 
      Regensburg, Regensburg, Germany.
LA  - eng
PT  - Case Reports
DEP - 20220127
PL  - Switzerland
TA  - Acta Haematol
JT  - Acta haematologica
JID - 0141053
RN  - 0 (Immunoglobulin M)
RN  - 0 (von Willebrand Factor)
SB  - IM
MH  - Hemorrhage/drug therapy/etiology
MH  - Humans
MH  - Immunoglobulin M/therapeutic use
MH  - *Paraproteinemias/diagnosis
MH  - *von Willebrand Diseases/diagnosis/drug therapy
MH  - von Willebrand Factor/therapeutic use
PMC - PMC9501758
OTO - NOTNLM
OT  - Acquired von Willebrand syndrome
OT  - Bleeding disorders
OT  - Monoclonal gammopathy of undetermined significance
OT  - Paraproteinaemia
OT  - von Willebrand factor
COIS- A.T. received grants or honoraria for lectures or consultancy from Biotest, CSL 
      Behring, Octapharma, and Takeda. All other authors declare no conflicts of 
      interest.
EDAT- 2022/01/28 06:00
MHDA- 2022/07/22 06:00
PMCR- 2022/01/27
CRDT- 2022/01/27 20:18
PHST- 2021/10/03 00:00 [received]
PHST- 2022/01/17 00:00 [accepted]
PHST- 2022/01/28 06:00 [pubmed]
PHST- 2022/07/22 06:00 [medline]
PHST- 2022/01/27 20:18 [entrez]
PHST- 2022/01/27 00:00 [pmc-release]
AID - 000522236 [pii]
AID - aha-0145-0454 [pii]
AID - 10.1159/000522236 [doi]
PST - ppublish
SO  - Acta Haematol. 2022;145(4):454-457. doi: 10.1159/000522236. Epub 2022 Jan 27.