Protein A Sepharose immunoadsorption can restore the efficacy of platelet concentrates in patients with Glanzmann's thrombasthenia and anti-glycoprotein IIb-IIIa antibodies.
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.
Martin
Isabelle
I
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.
Kriaa
Fayçal
F
Proulle
Valérie
V
Guillet
Benoît
B
Kaplan
Cécile
C
D'Oiron
Roseline
R
Debré
Marianne
M
Fressinaud
Edith
E
Laurian
Yyes
Y
Tchernia
Gil
G
Charpentier
Bernard
B
Lambert
Thierry
T
Dreyfus
Marie
M
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
D007136:Immunoglobulins; D007518:Isoantibodies; D019039:Platelet Glycoprotein GPIIb-IIIa Complex; D012685:Sepharose; C042030:Staphylococcal protein A-sepharose; D013205:Staphylococcal Protein A