Cheung Eric, Liebman Howard A
Jane Anne Nohl Division of Hematology and Center for the Study of Blood Diseases, University of Southern California-Keck School of Medicine, Los Angeles, CA, USA.
Biologics. 2009;3:57-62. Epub 2009 Jul 13.
Immune thrombocytopenia (ITP) is an acquired bleeding autoimmune disorder characterized by a markedly decreased blood platelet count. The disorder is variable, frequently having an acute onset of limited duration in children and a more chronic course in adults. A number of therapeutic agents have demonstrated efficacy in increasing the platelet counts in both children and adults. Anti-RhD immunoglobulin (anti-D) is one such agent, and has been successfully used in the setting of both acute and chronic immune thrombocytopenia. In this report we review the use of anti-D in the management of ITP. While the FDA-approved dose of 50 mg/kg has documented efficacy in increasing platelet counts in approximately 80% of children and 70% of adults, a higher dose of 75 microg/kg has been shown to result in a more rapid increase in platelet count without a greater reduction in hemoglobin. Anti-D is generally ineffective in patients who have failed splenectomy. Anti-RhD therapy has been shown capable of delaying splenectomy in adult patients, but does not significantly increase the total number of patients in whom the procedure can be avoided. Anti-D therapy appears to inhibit macrophage phagocytosis by a combination of both FcR blockade and inflammatory cytokine inhibition of platelet phagocytosis within the spleen. Anti-RhD treatment is associated with mild to moderate infusion toxicities. Rare life-threatening toxicities such as hemoglobinuria, acute renal failure and disseminated intravascular coagulation have been reported. Recommendations have been proposed to reduce the risk of these complications. Anti-D immunoglobulin can be an effective option for rapidly increasing platelet counts in patients with symptomatic ITP.
免疫性血小板减少症（ITP）是一种获得性出血性自身免疫性疾病，其特征是血小板计数显著降低。该疾病具有变异性，在儿童中通常急性起病，病程有限，而在成人中病程更为慢性。多种治疗药物已证明对增加儿童和成人的血小板计数有效。抗RhD免疫球蛋白（抗-D）就是这样一种药物，已成功用于急性和慢性免疫性血小板减少症的治疗。在本报告中，我们回顾了抗-D在ITP治疗中的应用。虽然美国食品药品监督管理局（FDA）批准的50mg/kg剂量已证明对约80%的儿童和70%的成人增加血小板计数有效，但75μg/kg的更高剂量已显示可使血小板计数更快增加，且血红蛋白降低幅度更小。抗-D对脾切除失败的患者通常无效。抗RhD治疗已显示能够延迟成年患者的脾切除，但并不能显著增加可避免该手术的患者总数。抗-D治疗似乎通过FcR阻断和抑制脾脏内血小板吞噬的炎性细胞因子的联合作用来抑制巨噬细胞吞噬作用。抗RhD治疗与轻度至中度输注毒性相关。已报告了罕见的危及生命的毒性，如血红蛋白尿、急性肾衰竭和弥散性血管内凝血。已提出降低这些并发症风险的建议。抗-D免疫球蛋白对于快速增加有症状ITP患者的血小板计数可能是一种有效的选择。
