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Semin Thromb Hemost 2018; 44(06): 604-614
DOI: 10.1055/s-0038-1648233
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Alloimmunization in Congenital Deficiencies of Platelet Surface Glycoproteins: Focus on Glanzmann's Thrombasthenia and Bernard–Soulier's Syndrome

Man-Chiu Poon
1   Department of Medicine, Cumming School of Medicine, University of Calgary and Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program, Calgary, Alberta, Canada
,
Roseline d'Oiron
2   Centre de Traitement de l'Hémophilie et autres Maladies Hémorragiques Constitutionnelles Rares, Hôpitaux Universitaires Paris Sud APHP - Hôpital Bicêtre APHP, Le Kremlin-Bicêtre, France
› Author Affiliations Funding Dr. d'Oiron reports personal fees and nonfinancial support from Novo Nordisk during the conduct of the study; personal fees and nonfinancial support from Baxalta/Shire, personal fees and nonfinancial support from Bayer, personal fees and nonfinancial support from Octapharma, nonfinancial support from LFB, personal fees and nonfinancial support from CSL Behring, personal fees and nonfinancial support from Pfizer, personal fees and nonfinancial support from Sobi, and personal fees and nonfinancial support from Roche, outside the submitted work. Dr. Poon reports grants, personal fees, and nonfinancial support from Bayer, personal fees and nonfinancial support from Baxalta/Shire, personal fees from Biogen Idec, grants, personal fees, and nonfinancial support from CSL Behring, personal fees and nonfinancial support from Novo Nordisk, personal fees from Octapharma, personal fees and nonfinancial support from Pfizer, and personal fees from Roche, outside the submitted work.
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Publication History

Publication Date:
07 June 2018 (online)

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Abstract

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 αIIbβ3) 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 αIIbβ3 in GT or GPIb-IX in BSS), which may render future platelet transfusion ineffective. Anti-αIIbβ3 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.

Keywords

Glanzmann's thrombasthenia - Bernard–Soulier's syndrome - platelet transfusion - alloimmunization - platelet antibodies - HLA antibodies - αIIbβ3 (GPIIb/IIIa) antibodies - GPIb-IX antibodies - rFVIIa - pregnancy