Kelton J G, Sheridan D, Santos A, Smith J, Steeves K, Smith C, Brown C, Murphy W G
Department of Medicine, McMaster University Medical Centre, Hamilton, Ontario, Canada.
Blood. 1988 Sep;72(3):925-30.
This report describes studies into the pathophysiology of heparin-induced thrombocytopenia. The IgG fraction from each of nine patients with heparin-induced thrombocytopenia caused heparin-dependent platelet release of radiolabeled serotonin. Both the Fc and the Fab portions of the IgG molecule were required for the platelet reactivity. The platelet release reaction could be inhibited by the Fc portion of normal human or goat IgG, and patient F(ab')2, but not F(ab')2 from healthy controls. These results suggested that the Fab portion of IgG binds to heparin forming an immune complex and the immune complexes initiate the platelet release reaction by binding to the platelet Fc receptors. To directly challenge this hypothesis, we preincubated the serotonin-labeled platelets with the monoclonal antibody against the platelet Fc receptor (IV.3). This monoclonal antibody completely inhibited the release reaction caused by heparin and patient sera, as well as heat aggregated IgG, but did not block collagen or thrombin-induced platelet release. Heparin-dependent platelet release also could be inhibited in vitro by the addition of monocytes and neutrophils, but not by red cells, presumably because the Fc receptors on the phagocytic cells have a higher binding affinity for IgG complexes than do platelets. Platelets from patients with congenital deficiencies of specific glycoproteins Ib and IX (Bernard-Soulier syndrome) and IIb and IIIa (Glanzmann's thrombasthenia) displayed normal heparin-dependent release indicating that the release reaction did not require the participation of these glycoproteins. These studies indicate that heparin-induced thrombocytopenia is an IgG-heparin immune complex disorder involving both the Fab and Fc portion of the IgG molecule.
本报告描述了对肝素诱导的血小板减少症病理生理学的研究。来自9例肝素诱导的血小板减少症患者的IgG组分导致放射性标记的血清素的肝素依赖性血小板释放。IgG分子的Fc和Fab部分对于血小板反应性都是必需的。血小板释放反应可被正常人或山羊IgG的Fc部分以及患者的F(ab')2抑制,但不能被健康对照者的F(ab')2抑制。这些结果表明,IgG的Fab部分与肝素结合形成免疫复合物,并且免疫复合物通过与血小板Fc受体结合引发血小板释放反应。为了直接验证这一假设,我们用抗血小板Fc受体的单克隆抗体(IV.3)对血清素标记的血小板进行预孵育。该单克隆抗体完全抑制了由肝素和患者血清以及热聚集IgG引起的释放反应,但不阻断胶原或凝血酶诱导的血小板释放。在体外,添加单核细胞和中性粒细胞也可抑制肝素依赖性血小板释放,但红细胞则不能,推测是因为吞噬细胞上的Fc受体对IgG复合物的结合亲和力高于血小板。患有特定糖蛋白Ib和IX先天性缺陷(Bernard-Soulier综合征)以及IIb和IIIa先天性缺陷(Glanzmann血小板无力症)患者的血小板表现出正常的肝素依赖性释放,表明释放反应不需要这些糖蛋白的参与。这些研究表明,肝素诱导的血小板减少症是一种IgG-肝素免疫复合物疾病,涉及IgG分子的Fab和Fc部分。