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: Isabelle Martin, Fayçal Kriaa, Valérie Proulle, Benoît Guillet, Cécile Kaplan, Roseline D'Oiron, Marianne Debré, Edith Fressinaud, Yyes Laurian, Gil Tchernia, Bernard Charpentier, Thierry Lambert, Marie Dreyfus
• Journal: British journal of haematology (Br J Haematol) Vol. 119 Issue 4 Pg. 991-7 (Dec 2002) ISSN: 0007-1048 [Print] England
• PMID: 12472579 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Immunoglobulins
• Isoantibodies
• Platelet Glycoprotein GPIIb-IIIa Complex
• Staphylococcal protein A-sepharose
• Sepharose

Topics

• Adolescent
• Adult
• Bleeding Time
• Child
• Female
• Humans
• Immunoglobulins (blood)
• Immunosorbent Techniques
• Isoantibodies (blood)
• Male
• Platelet Aggregation
• Platelet Glycoprotein GPIIb-IIIa Complex (immunology)
• Platelet Transfusion
• Sepharose (analogs & derivatives)
• Sorption Detoxification (methods)
• Thrombasthenia (blood, immunology, therapy)