Martin Isabelle, Kriaa Fayçal, Proulle Valérie, Guillet Benoît, Kaplan Cécile, D'Oiron Roseline, Debré Marianne, Fressinaud Edith, Laurian Yyes, Tchernia Gil, Charpentier Bernard, Lambert Thierry, Dreyfus Marie
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.
Br J Haematol. 2002 Dec;119(4):991-7. doi: 10.1046/j.1365-2141.2002.03936.x.
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.
I型Glanzmann血小板无力症是一种罕见的先天性血小板功能障碍，其特征是检测不到血小板膜糖蛋白IIb-IIIa（GPIIb-IIIa）。严重出血通过输注正常血小板来控制，在某些情况下会导致抗GPIIb-IIIa同种抗体的产生，从而导致输注血小板的疗效丧失。我们对三名患有Glanzmann血小板无力症和抗GPIIb-IIIa同种抗体的患者在五个不同时间点使用了蛋白A琼脂糖免疫吸附法（IA-PA），该方法已被证明在降低几种免疫疾病中的抗体滴度方面有效。IA-PA耐受性良好，未报告有害副作用。通过单克隆抗体特异性固定血小板抗原试验以及患者富血小板或贫血小板血浆对正常血小板聚集的体外抑制作用评估，它使总免疫球蛋白（Ig）G包括抗GPIIb-IIIa同种抗体水平显著降低。抗体的清除与血小板输注后出血时间的纠正相关。IA-PA与血小板输注相结合使得控制两次危及生命的出血成为可能，并允许安全地进行两次外科手术和一次骨髓移植。我们的经验表明，IA-PA恢复了血小板输注的止血效果，是治疗Glanzmann血小板无力症和抗GPIIb-IIIa同种抗体患者的一种有价值的治疗策略。
