PMID- 29651351
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20240313
IS  - 2090-6560 (Print)
IS  - 2090-6579 (Electronic)
IS  - 2090-6579 (Linking)
VI  - 2018
DP  - 2018
TI  - Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma.
PG  - 7973297
LID - 10.1155/2018/7973297 [doi]
LID - 7973297
AB  - We present a rare case of acquired von Willebrand syndrome (AVWS) caused by a 
      mantle cell lymphoma. A 61-year-old male suffered from recurrent bleeding 
      symptoms since a few months. Initially, physical examination was normal. von 
      Willebrand factor antigen (VWF:Ag) level and factor VIII activity (FVIII:C) were 
      low (0.31 IU/ml and 0.43 IU/ml, resp.). Ristocetin cofactor activity (VWF:RCo) 
      was 0.09 IU/ml, and collagen binding activity (VWF:CB) was 0.10 IU/ml. 
      VWF:RCo/VWF:Ag ratio was 0.29, and RIPA value was normal. Highest molecular 
      weight VWF multimers were absent. A diagnosis of von Willebrand Disease (VWD) 
      type 2A was made. However, no genetic mutation was found. No inhibitory 
      antibodies against VWF or factor VIII were detected. A few months later, 
      cervical, axillary, and inguinal lymphadenopathy was found on physical 
      examination. A CT scan confirmed multiple enlarged lymph nodes, and a clonal 
      B-cell population matching a mantle cell lymphoma was detected in the bone 
      marrow. Chemoimmunotherapy resulted in a very good partial remission and 
      concomitantly in a rapid decrease of bleeding problems and complete normalization 
      of FVIII:C and VWF:Ag. The diagnosis of AVWS cannot be rejected by negative 
      mixing studies due to difficulties in the detection of autoantibodies and because 
      of a highly heterogeneous pathogenesis of AVWS. When the suspicion of AVWS is 
      high, an extensive investigation should be performed to find the underlying 
      cause.
FAU - Maas, Dominique
AU  - Maas D
AUID- ORCID: 0000-0003-1169-4354
AD  - Department of Hematology, Radboud University Medical Center, Nijmegen, 
      Netherlands.
AD  - Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, 
      Netherlands.
FAU - Laros-van Gorkom, Britta
AU  - Laros-van Gorkom B
AD  - Department of Hematology, Radboud University Medical Center, Nijmegen, 
      Netherlands.
AD  - Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, 
      Netherlands.
FAU - Gianotten, Sanne
AU  - Gianotten S
AD  - Department of Hematology, Radboud University Medical Center, Nijmegen, 
      Netherlands.
FAU - Cruijsen, Marjan
AU  - Cruijsen M
AD  - Department of Hematology, Radboud University Medical Center, Nijmegen, 
      Netherlands.
FAU - van Heerde, Waander
AU  - van Heerde W
AD  - Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, 
      Netherlands.
AD  - Department of Laboratory Medicine, Laboratory for Hematology, Radboud University 
      Medical Center, Nijmegen, Netherlands.
FAU - Nijziel, Marten
AU  - Nijziel M
AD  - Department of Hematology, Radboud University Medical Center, Nijmegen, 
      Netherlands.
AD  - Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, 
      Netherlands.
LA  - eng
PT  - Case Reports
DEP - 20180130
PL  - United States
TA  - Case Rep Hematol
JT  - Case reports in hematology
JID - 101576456
PMC - PMC5830951
EDAT- 2018/04/14 06:00
MHDA- 2018/04/14 06:01
PMCR- 2018/01/30
CRDT- 2018/04/14 06:00
PHST- 2017/08/27 00:00 [received]
PHST- 2017/12/26 00:00 [revised]
PHST- 2017/12/28 00:00 [accepted]
PHST- 2018/04/14 06:00 [entrez]
PHST- 2018/04/14 06:00 [pubmed]
PHST- 2018/04/14 06:01 [medline]
PHST- 2018/01/30 00:00 [pmc-release]
AID - 10.1155/2018/7973297 [doi]
PST - epublish
SO  - Case Rep Hematol. 2018 Jan 30;2018:7973297. doi: 10.1155/2018/7973297. 
      eCollection 2018.