Wordell C J, Stubits E A, Tietze K J, Gaska J A, Cosgrove E M
Clin Pharm. 1985 Mar-Apr;4(2):206-13.
Two patients with autoimmune thrombocytopenic purpura (ATP) who received high-dose intravenous immune globulin before undergoing splenectomy are described, and the pathogenesis, clinical presentation, diagnosis, and treatment of chronic ATP are reviewed. One patient was a 62-year-old white man who was admitted to the hospital with a history of thrombocytopenia and probable steroid-induced diabetes mellitus. The second patient was a 24-year-old black woman who was admitted for recurrent bleeding episodes and splenectomy. In both patients, immune globulin 1.5 g/kg administered over four to six days resulted in marked elevations of platelet counts. ATP is a platelet disorder of unknown etiology. Platelets with surface-bound antiplatelet antibody are destroyed by the reticuloendothelial system. As the platelet count falls below 30,000-50,000/cu mm, the patient may manifest signs of bleeding such as petechiae, purpura, ecchymosis, menorrhagia, epistaxis, and bleeding from other mucosal surfaces. Corticosteroids are the initial treatment of choice. Splenectomy is considered for children with the life-threatening hemorrhage and for patients who do not respond to corticosteroids. Patients who are refractory to steroid therapy and splenectomy may respond to immunosuppressant agents. Approximately, 80% of patients treated with immune globulin have responded with an increase in platelet count, although this increase is sometimes transient. Immune globulin therapy is recommended for emergency treatment of ATP, as preoperative medication before splenectomy, and for young children in order to postpone splenectomy. Despite a good response to treatment, immune globulin therapy should not be considered a cure for ATP.
本文描述了两名自身免疫性血小板减少性紫癜(ATP)患者,他们在接受脾切除术前接受了大剂量静脉注射免疫球蛋白,并对慢性ATP的发病机制、临床表现、诊断和治疗进行了综述。一名患者是一名62岁的白人男性,因血小板减少病史和可能的类固醇诱导的糖尿病入院。第二名患者是一名24岁的黑人女性,因反复出血发作和脾切除术入院。在这两名患者中,在四到六天内给予1.5 g/kg的免疫球蛋白导致血小板计数显著升高。ATP是一种病因不明的血小板疾病。表面结合抗血小板抗体的血小板被网状内皮系统破坏。当血小板计数降至30,000-50,000/立方毫米以下时,患者可能会出现出血迹象,如瘀点、紫癜、瘀斑、月经过多、鼻出血和其他粘膜表面出血。皮质类固醇是初始治疗的首选。对于有危及生命的出血的儿童和对皮质类固醇无反应的患者,考虑进行脾切除术。对类固醇治疗和脾切除术难治的患者可能对免疫抑制剂有反应。大约80%接受免疫球蛋白治疗的患者血小板计数增加,尽管这种增加有时是短暂的。免疫球蛋白疗法推荐用于ATP的紧急治疗、作为脾切除术前的术前用药以及用于幼儿以推迟脾切除术。尽管对治疗反应良好,但免疫球蛋白疗法不应被视为ATP的治愈方法。