Wagner H P, Barandun S, Hirt A, Imbach P
Presse Med. 1983 Nov 17;12(41):2612-4.
In childhood approximately 90% of all idiopathic thrombocytopenic purpuras (ITP) are acute while in adults the majority is chronic (6 months duration). 0,5 - 1% of the children with ITP die. Conventional treatment comprises corticosteroids and, for chronic ITP, splenectomy and/or cytostatic immunosuppression. Inspired by the disappearance, during substitution, of concomitant ITP in 2 agammaglobulinemic boys, 13 children with ITP (3 chronic, 4 intermittent, 6 acute) were treated with high-dose intravenous immunoglobulin SRK. All responded favorably but one boy with chronic ITP became resistant after 11 months of infusions. F(ab')2 fragments are inactive. High doses of immunoglobulin could block Fc receptors of macrophages. They could also facilitate the formation and elimination of immune complexes and, indirectly, prevent the accumulation of platelet-associated immunoglobulin.
儿童期，所有特发性血小板减少性紫癜（ITP）中约90%为急性型，而在成人中多数为慢性型（病程6个月）。患ITP的儿童中有0.5% - 1%死亡。传统治疗方法包括使用皮质类固醇，对于慢性ITP，则采用脾切除术和/或细胞毒性免疫抑制治疗。受2名无丙种球蛋白血症男孩在替代治疗期间伴随的ITP消失的启发，对13例ITP患儿（3例慢性、4例间歇性、6例急性）采用大剂量静脉注射免疫球蛋白SRK进行治疗。所有患儿反应良好，但1例慢性ITP男孩在输注11个月后产生耐药性。F(ab')2片段无活性。高剂量免疫球蛋白可阻断巨噬细胞的Fc受体。它们还可促进免疫复合物的形成和清除，并间接防止血小板相关免疫球蛋白的蓄积。
