Khalifa A S, Tolba K A, el-Alfy M S, Gadallah M, Ibrahim F H
Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Acta Haematol. 1993;90(3):125-9. doi: 10.1159/000204392.
350 patients with idiopathic thrombocytopenic purpura (ITP) aged 2/12-15 years (mean 6.3 +/- 2.7) were followed up during the period January 1st, 1975 to March 31, 1992. They constituted 40% of cases with hemorrhagic diathesis attending the Hematology/Oncology Clinic, Children's Hospital, Ain Shams University (relative frequency of 37.4/100.000 of the general Out-Patient Clinic in the same hospital). These patients presented with acute (71.4%), chronic (22.9%) and recurrent (5.7%) forms. The age of presentation was younger in acute ITP. In the recurrent form there was significant female predominance. Most cases of acute ITP (66%) presented in winter and spring, with a positive history of preceding viral illness in 50% in contrast to 10% in chronic form. Four chronic ITP cases developed lupus erythematosus; all were females > 9 years. As regards therapy, acute ITP cases with initial platelet count (PC) < 10 x 10(9)/l were randomized to receive either high-dose methyl prednisolone (HDMP) 10 mg/kg/day for 5 days i.v. (n = 10) or intravenous immunoglobulin (IVIG) 0.4 g/kg/day for 5 days (n = 10) or conventional-dose prednisone (CDP) 2 mg/kg/day 4 weeks p.o. (n = 10). A dramatic response was noticed in the first two groups. In chronic ITP, (n = 80) CDP induced complete response (CR) in 30% and partial response (PR) in 20%; 50% were nonresponders. Twenty-four refractory ITP with persistent PC < or = 20 x 10(9)/l received second-line therapy: vincristine 1.5 mg/m2/week i.v. 4 doses (n = 4) with no clinical or hematological improvement. IVIG 0.4 g/kg/day for 5 days (n = 8) with sustained CR only in 2 patients (25%) and PR in 2 patients (25%). Splenectomy was performed (n = 12) with CR in 50%; out of them, 2 patients had shown no improvement on prior IVIG therapy. In conclusion, ITP is a benign condition with no fatality reported, but it could run a chronic refractory course.
1975年1月1日至1992年3月31日期间，对350例年龄在2/12至15岁（平均6.3±2.7岁）的特发性血小板减少性紫癜（ITP）患者进行了随访。他们占艾因夏姆斯大学儿童医院血液科/肿瘤科出血性素质病例的40%（在同一家医院普通门诊中的相对发病率为37.4/100,000）。这些患者呈现急性（71.4%）、慢性（22.9%）和复发性（5.7%）形式。急性ITP患者的发病年龄较小。在复发性形式中，女性明显占优势。大多数急性ITP病例（66%）在冬季和春季发病，50%有前驱病毒感染史，而慢性形式中这一比例为10%。4例慢性ITP患者发展为红斑狼疮；均为年龄大于9岁的女性。关于治疗，初始血小板计数（PC）<10×10⁹/L的急性ITP病例被随机分为三组，分别接受静脉注射大剂量甲泼尼龙（HDMP）10mg/kg/天，共5天（n = 10）；或静脉注射免疫球蛋白（IVIG）0.4g/kg/天，共5天（n = 10）；或口服常规剂量泼尼松（CDP）2mg/kg/天，共4周（n = 10）。前两组出现显著反应。在慢性ITP患者（n = 80）中，CDP诱导30%完全缓解（CR），20%部分缓解（PR）；50%无反应。24例难治性ITP患者，血小板持续计数≤20×10⁹/L，接受二线治疗：静脉注射长春新碱1.5mg/m²/周，共4剂（n = 4），无临床或血液学改善。静脉注射IVIG 0.4g/kg/天，共5天（n = 8），仅2例（25%）持续CR，2例（25%）PR。12例行脾切除术，50% CR；其中2例先前接受IVIG治疗无改善。总之，ITP是一种良性疾病，尚无死亡报告，但可能呈慢性难治病程。
