Bussel J B, Pham L C, Aledort L, Nachman R
Department of Pediatrics and Medicine, New York Hospital/Cornell Medical Center 10021.
Blood. 1988 Jul;72(1):121-7.
Intravenous infusion of gammaglobulin (IVGG) has been extensively used in the treatment of immune thrombocytopenic purpura (ITP) in adults to acutely raise the platelet count but not as a maintenance therapy. This report describes the maintenance treatment of adults with chronic ITP using repeated infusions of 800 to 1,000 mg/kg of IVGG. Sixteen of 40 patients were able to discontinue all therapy after receiving between one and 15 infusions. Five patients achieved remission and 11 other patients became stable without therapy (SWT) maintaining a platelet count greater than 20,000/microL without bleeding. The average quantity of gammaglobulin received for all patients was 606 g per patient. Of the 30 patients who underwent but did not respond to splenectomy, 11 (37%) were able to discontinue all therapy by either achieving remission (5) or becoming SWT (6). None of the five patients who achieved remission did so after only the initial therapy; all first received between one and 12 maintenance infusions. The ten splenectomized patients who were unresponsive to IVGG also failed to subsequently respond to conventional therapy including immunosuppressive agents and androgens. No toxicity of IVGG was seen except for postinfusion headaches. IVGG is an effective although expensive maintenance therapy for adults with ITP and is useful in patients who have not responded to splenectomy.
静脉输注丙种球蛋白(IVGG)已被广泛用于治疗成人免疫性血小板减少性紫癜(ITP),以急性提高血小板计数,但不作为维持治疗。本报告描述了使用800至1000mg/kg的IVGG重复输注对成人慢性ITP进行维持治疗的情况。40例患者中有16例在接受1至15次输注后能够停止所有治疗。5例患者实现缓解,另有11例患者在无治疗(SWT)情况下病情稳定,血小板计数维持在大于20,000/微升且无出血。所有患者接受的丙种球蛋白平均量为每人606g。在接受脾切除术但无反应的30例患者中,11例(37%)通过实现缓解(5例)或成为SWT(6例)能够停止所有治疗。实现缓解的5例患者中,无一例仅在初始治疗后就达到缓解;所有患者首先接受了1至12次维持输注。对IVGG无反应的10例脾切除患者随后对包括免疫抑制剂和雄激素在内的传统治疗也无反应。除输注后头痛外,未观察到IVGG的毒性。IVGG对成人ITP是一种有效的维持治疗方法,尽管费用昂贵,且对脾切除无反应的患者有用。