title: Protein A Sepharose immunoadsorption can restore the efficacy of platelet concentrates in patients with Glanzmann's thrombasthenia and anti-glycoprotein IIb-IIIa antibodies.
abstract: Type I Glanzmann's thrombasthenia is a rare congenital platelet function disorder, characterized by undetectable platelet membrane glycoprotein IIb-IIIa (GPIIb-IIIa). Severe bleeding is controlled by transfusion of normal platelets, leading in some cases to the occurrence of anti-GPIIb-IIIa isoantibodies, which induces a loss of transfused platelet efficacy. We used immunoadsorption on protein A Sepharose (IA-PA), which has been shown to be efficient in decreasing the titre of antibodies in several immune diseases, in three patients with Glanzmann's thrombasthenia and anti-GPIIb-IIIa isoantibodies on five different occasions. IA-PA was well tolerated with no deleterious side-effects reported. It induced a dramatic decrease of total immunoglobulin (Ig)G, including anti-GPIIb-IIIa isoantibody levels, as assessed by the monoclonal antibody-specific immobilization of platelet antigens test and the ex vivo inhibition of normal platelet aggregation induced by the patient's platelet-rich or platelet-poor plasma. Elimination of the antibody was associated with a correction of the bleeding time following platelet transfusion. IA-PA combined with platelet transfusion made it possible to control two life-threatening haemorrhages, and allowed two surgical procedures and one bone marrow transplantation to be performed safely. Our experience suggests that IA-PA, which restores the haemostatic efficacy of platelet transfusion, is a valuable therapeutic strategy in patients with Glanzmann's thrombasthenia and anti-GPIIb-IIIa isoantibodies.
authors.affiliation: Laboratoire d'Hématologie, Centre de Traitement de l'Hémophilie, Assistance Publique-Hôpitaux de Paris and Faculté de Médecine Paris XI, Le Kremlin-Bicêtre, Paris, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Martin
authors.forename: Fayçal
authors.initials: F
authors.lastname: Kriaa
authors.forename: Valérie
authors.initials: V
authors.lastname: Proulle
authors.forename: Benoît
authors.initials: B
authors.lastname: Guillet
authors.forename: Cécile
authors.initials: C
authors.lastname: Kaplan
authors.forename: Roseline
authors.initials: R
authors.lastname: D'Oiron
authors.forename: Marianne
authors.initials: M
authors.lastname: Debré
authors.forename: Edith
authors.initials: E
authors.lastname: Fressinaud
authors.forename: Yyes
authors.initials: Y
authors.lastname: Laurian
authors.forename: Gil
authors.initials: G
authors.lastname: Tchernia
authors.forename: Bernard
authors.initials: B
authors.lastname: Charpentier
authors.forename: Thierry
authors.initials: T
authors.lastname: Lambert
authors.forename: Marie
authors.initials: M
authors.lastname: Dreyfus
mesh_terms: D000293:Adolescent; D000328:Adult; D001760:Bleeding Time; D002648:Child; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D007163:Immunosorbent Techniques; D007518:Isoantibodies / Q000097:blood*; D008297:Male; D010974:Platelet Aggregation; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000276:immunology*; D017713:Platelet Transfusion; D012685:Sepharose / Q000031:analogs & derivatives*; D016060:Sorption Detoxification / Q000379:methods*; D013915:Thrombasthenia / Q000097:blood / Q000276:immunology / Q000628:therapy*; D013205:Staphylococcal Protein A
chemical_list: D007136:Immunoglobulins; D007518:Isoantibodies; D019039:Platelet Glycoprotein GPIIb-IIIa Complex; D012685:Sepharose; C042030:Staphylococcal protein A-sepharose; D013205:Staphylococcal Protein A