Bussel J B, Saal S, Gordon B
Department of Pediatrics, Cornell Medical College/New York Hospital, New York.
Transfusion. 1988 Jan-Feb;28(1):38-41. doi: 10.1046/j.1537-2995.1988.28188127949.x.
Plasma exchange (PE) and intravenous gammaglobulin (IVGG) are potentially effective therapies in immune thrombocytopenic purpura (ITP). In this study, eight patients refractory to IVGG and to prednisone were treated with a protocol of combined PE and IVGG therapy using a single PE on each of 3 consecutive days, followed by 2 consecutive days of IVGG at 1 g per kg. Four of the eight patients responded to the combined therapy with a mean peak platelet count of 132,000 per microliter (range, 74,000 to 225,000/microliter). Responses lasted approximately 2 weeks. These four patients were among the five who had initially responded to IVGG before becoming refractory; none of the three patients without an initial response to IVGG responded to the combined therapy. Age, duration of disease, and splenectomy status did not appear to be related to response to the combined therapy. Two patients began maintenance treatment (1 PE followed by 1 g/kg IVGG on the same day), but both became unresponsive after three treatments. PE combined with IVGG may be a useful treatment for some patients with refractory ITP who have uncontrollable bleeding or require major surgery. The development of resistance to IVGG effect may be mediated by an increase in the level of antiplatelet antibodies. PE, by lowering antiplatelet antibody levels, may then allow IVGG infusion to be effective again in elevating the platelet count.
血浆置换(PE)和静脉注射丙种球蛋白(IVGG)是免疫性血小板减少性紫癜(ITP)潜在的有效治疗方法。在本研究中,8例对IVGG和泼尼松治疗无效的患者接受了联合PE和IVGG治疗方案,连续3天每天进行1次PE,随后连续2天静脉注射IVGG,剂量为每千克1克。8例患者中有4例对联合治疗有反应,平均血小板峰值计数为每微升132,000(范围为74,000至225,000/微升)。反应持续约2周。这4例患者是最初对IVGG有反应但后来变得难治的5例患者中的一部分;最初对IVGG无反应的3例患者中,没有1例对联合治疗有反应。年龄、病程和脾切除状态似乎与联合治疗的反应无关。2例患者开始维持治疗(1次PE,随后同一天静脉注射IVGG,剂量为每千克1克),但均在3次治疗后不再有反应。PE联合IVGG可能是一些难治性ITP患者有无法控制的出血或需要进行大手术时的一种有用治疗方法。对IVGG效应产生耐药性的发展可能是由抗血小板抗体水平的升高介导的。通过降低抗血小板抗体水平,PE可能会使IVGG输注再次有效地提高血小板计数。