PMID- 30534006
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220408
IS  - 1477-9560 (Print)
IS  - 1477-9560 (Electronic)
IS  - 1477-9560 (Linking)
VI  - 16
DP  - 2018
TI  - Combination of acquired von Willebrand syndrome (AVWS) and Glanzmann 
      thrombasthenia in monoclonal gammopathy of uncertain significance (MGUS), a case 
      report.
PG  - 30
LID - 10.1186/s12959-018-0184-2 [doi]
LID - 30
AB  - BACKGROUND: Autoimmune paraphenomena, are associated with B-cell 
      lymphoproliferative disorders, including monoclonal gammopathy of uncertain 
      significance. These paraphenomena can rarely include acquired bleeding disorders. 
      CASE PRESENTATION: This case study reports an unusual clinical presentation of 2 
      acquired bleeding disorders, Acquired von Willebrand syndrome (disease) and 
      Acquired Glanzmann's thrombasthenia, in an elderly patient with monoclonal 
      gammopathy of uncertain significance. CONCLUSIONS: Acquired bleeding disorders 
      are often underdiagnosed and a high degree of clinical suspicion is required. The 
      patient in this study demonstrated platelet aggregometry which was atypical for 
      isolated Glanzmann's thrombosthenia because of the severe concomitant endogenous 
      decrease in von Willebrand factor. There was an absence of platelet aggregation 
      to all tested agonists including ristocetin. Once the diagnosis was made, 
      however, the patient showed a partial response to intravenous immunoglobulin 
      confirming the immunological pathogenesis in this case. This case highlights the 
      need to consider acquired bleeding disorders in patients with a possible 
      predisposing factor.
FAU - Mayne, Elizabeth Sarah
AU  - Mayne ES
AUID- ORCID: 0000-0002-6360-3488
AD  - Department of Molecular Medicine and Haematology, Faculty of Health Sciences, 
      University of the Witwatersr and National Health Laboratory Services, 7 York 
      Road, Parktown, Johannesburg, 2196 South Africa. ISNI: 0000 0004 0630 4574. GRID: 
      grid.416657.7
FAU - Tait, Malcolm
AU  - Tait M
AD  - Department of Molecular Medicine and Haematology, Faculty of Health Sciences, 
      University of the Witwatersr and National Health Laboratory Services, 7 York 
      Road, Parktown, Johannesburg, 2196 South Africa. ISNI: 0000 0004 0630 4574. GRID: 
      grid.416657.7
FAU - Jacobson, Barry Frank
AU  - Jacobson BF
AD  - Department of Molecular Medicine and Haematology, Faculty of Health Sciences, 
      University of the Witwatersr and National Health Laboratory Services, 7 York 
      Road, Parktown, Johannesburg, 2196 South Africa. ISNI: 0000 0004 0630 4574. GRID: 
      grid.416657.7
FAU - Pillay, Evashin
AU  - Pillay E
AD  - Department of Molecular Medicine and Haematology, Faculty of Health Sciences, 
      University of the Witwatersr and National Health Laboratory Services, 7 York 
      Road, Parktown, Johannesburg, 2196 South Africa. ISNI: 0000 0004 0630 4574. GRID: 
      grid.416657.7
FAU - Louw, Susan J
AU  - Louw SJ
AD  - Department of Molecular Medicine and Haematology, Faculty of Health Sciences, 
      University of the Witwatersr and National Health Laboratory Services, 7 York 
      Road, Parktown, Johannesburg, 2196 South Africa. ISNI: 0000 0004 0630 4574. GRID: 
      grid.416657.7
LA  - eng
PT  - Case Reports
DEP - 20181126
PL  - England
TA  - Thromb J
JT  - Thrombosis journal
JID - 101170542
PMC - PMC6260759
OTO - NOTNLM
OT  - Acquired Glanzmann's Thrombasthenia
OT  - Acquired von Willebrand syndrome
OT  - Monoclonal Gammopathy
COIS- This case study was approved by the Human Research Ethics Committee of the 
      University of the Witwatersrand. The participant provided consent to participate 
      and for publication. The ethics approval number is M1706103.The participant 
      provided consent to publish.The authors declare that they have no competing 
      interests.Springer Nature remains neutral with regard to jurisdictional claims in 
      published maps and institutional affiliations.
EDAT- 2018/12/12 06:00
MHDA- 2018/12/12 06:01
PMCR- 2018/11/26
CRDT- 2018/12/12 06:00
PHST- 2018/06/23 00:00 [received]
PHST- 2018/09/12 00:00 [accepted]
PHST- 2018/12/12 06:00 [entrez]
PHST- 2018/12/12 06:00 [pubmed]
PHST- 2018/12/12 06:01 [medline]
PHST- 2018/11/26 00:00 [pmc-release]
AID - 184 [pii]
AID - 10.1186/s12959-018-0184-2 [doi]
PST - epublish
SO  - Thromb J. 2018 Nov 26;16:30. doi: 10.1186/s12959-018-0184-2. eCollection 2018.