PMID- 29879742
OWN - NLM
STAT- MEDLINE
DCOM- 20181211
LR  - 20181211
IS  - 1098-9064 (Electronic)
IS  - 0094-6176 (Linking)
VI  - 44
IP  - 6
DP  - 2018 Sep
TI  - Alloimmunization in Congenital Deficiencies of Platelet Surface Glycoproteins: 
      Focus on Glanzmann's Thrombasthenia and Bernard-Soulier's Syndrome.
PG  - 604-614
LID - 10.1055/s-0038-1648233 [doi]
AB  - Glanzmann's thrombasthenia (GT) and Bernard-Soulier's syndrome (BSS) are 
      well-understood congenital bleeding disorders, showing defect/deficiency of 
      platelet glycoprotein (GP) IIb/IIIa (integrin alphaIIbbeta3) and GPIb-IX-V complexes 
      respectively, with relevant clinical, laboratory, biochemical, and genetic 
      features. Following platelet transfusion, affected patients may develop 
      antiplatelet antibodies (to human leukocyte antigen [HLA], and/or alphaIIbbeta3 in GT or 
      GPIb-IX in BSS), which may render future platelet transfusion ineffective. 
      Anti-alphaIIbbeta3 and anti-GPIb-IX may also cross the placenta during pregnancy to 
      cause thrombocytopenia and bleeding in the fetus/neonate. This review will focus 
      particularly on the better studied GT to illustrate the natural history and 
      complications of platelet alloimmunization. BSS will be more briefly discussed. 
      Platelet transfusion, if unavoidable, should be given judiciously with good 
      indications. Patients following platelet transfusion, and women during and after 
      pregnancy, should be monitored for the development of platelet antibodies. There 
      is now a collection of data suggesting the safety and effectiveness of 
      recombinant activated factor VII in the management of affected patients with 
      platelet antibodies.
CI  - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
FAU - Poon, Man-Chiu
AU  - Poon MC
AD  - Department of Medicine, Cumming School of Medicine, University of Calgary and 
      Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program, 
      Calgary, Alberta, Canada.
FAU - d'Oiron, Roseline
AU  - d'Oiron R
AD  - Centre de Traitement de l'Hemophilie et autres Maladies Hemorragiques 
      Constitutionnelles Rares, Hopitaux Universitaires Paris Sud APHP - Hopital 
      Bicetre APHP, Le Kremlin-Bicetre, France.
LA  - eng
PT  - Journal Article
DEP - 20180607
PL  - United States
TA  - Semin Thromb Hemost
JT  - Seminars in thrombosis and hemostasis
JID - 0431155
RN  - 0 (Platelet Membrane Glycoproteins)
SB  - IM
MH  - Bernard-Soulier Syndrome/*genetics
MH  - Female
MH  - Hemorrhage/etiology
MH  - Humans
MH  - Platelet Membrane Glycoproteins
MH  - Pregnancy
MH  - Thrombasthenia/*genetics
COIS- M. C. P. was chair of Novo Nordisk's expert panel on the GTR (2007-2011), has 
      been an ad hoc speaker for Bayer, Novo Nordisk, and Pfizer, has attended advisory 
      board meetings of Biogen Idec, Baxalta/Shire, CSL Behring, Novo Nordisk, 
      Octapharma, Pfizer, and Roche, and received grant funding from CSL Behring and 
      Bayer. R. d'O. was a member of Novo Nordisk's expert panel on the GTR (2007-2011) 
      and has received fees or honoraria for attending advisory boards or speaking at 
      symposia from Novo Nordisk, Octapharma, Baxalta/Shire, Bayer, LFB, Pfizer, Sobi, 
      Roche, and CSL Behring.
EDAT- 2018/06/08 06:00
MHDA- 2018/12/12 06:00
CRDT- 2018/06/08 06:00
PHST- 2018/06/08 06:00 [pubmed]
PHST- 2018/12/12 06:00 [medline]
PHST- 2018/06/08 06:00 [entrez]
AID - 10.1055/s-0038-1648233 [doi]
PST - ppublish
SO  - Semin Thromb Hemost. 2018 Sep;44(6):604-614. doi: 10.1055/s-0038-1648233. Epub 
      2018 Jun 7.