Aronis S, Platokouki H, Mitsika A, Haidas S, Constantopoulos A
Hemostasis Unit, Aghia Sophia Children's Hospital, Athens, Greece.
Pediatr Hematol Oncol. 1994 Sep-Oct;11(5):487-98. doi: 10.3109/08880019409141687.
Between 1975 and 1992 450 children with idiopathic thrombocytopenic purpura (ITP) were diagnosed, and of those 100 (22%) developed the chronic form of the disease. Approximately half the patients with chronic ITP presented with mild to moderate hemorrhagic manifestations at the onset of purpura (30 cases) and/or later during the course of the disease (25 cases). The incidence of intracranial hemorrhage was 1%, and the mortality rate due to overwhelming septicemia after splenectomy was also 1%. Overall one-third of the patients received no therapy; two-thirds of them went into spontaneous remission within 8 months to 8 years from the onset of ITP. Steroids given in conventional or high doses (51 cases) achieved a transient (if any) rise in platelet count, but in no case were steroids curative. Remission related to intravenous immune globulin (IVIG) therapy was noticed in 38.5% of the children (10 of 26) after variable courses. The response rate to splenectomy was 95.0%. Ultimately the long-term outcome in children with chronic ITP was as follows: remission, 58 cases (spontaneous, 30; after IVIG therapy, 10; after splenectomy, 18); hemostatic platelet values, 22 cases (spontaneous, 16; after IVIG, 5; after splenectomy, 1). Thirteen children were lost in follow-up, and 7 remain thrombocytopenic but asymptomatic. These data indicate that chronic ITP in childhood runs a benign course in most cases and may remit with or without therapy even several years from onset. Therefore, therapeutic intervention has to be individualized, and splenectomy, which is not always safe, should be reserved for problematic cases that fail to respond to conventional therapeutic modalities.
1975年至1992年间，共诊断出450例特发性血小板减少性紫癜（ITP）患儿，其中100例（22%）发展为慢性疾病形式。大约一半的慢性ITP患者在紫癜发作时（30例）和/或疾病过程后期（25例）出现轻度至中度出血表现。颅内出血的发生率为1%，脾切除术后因暴发性败血症导致的死亡率也为1%。总体而言，三分之一的患者未接受治疗；其中三分之二的患者在ITP发病后8个月至8年内自发缓解。给予常规或高剂量类固醇治疗的患者（51例）血小板计数有短暂升高（若有升高的话），但类固醇治疗无一例能治愈。不同疗程后，38.5%的儿童（26例中的10例）接受静脉注射免疫球蛋白（IVIG）治疗后病情缓解。脾切除的有效率为95.0%。最终，慢性ITP患儿的长期预后如下：缓解，58例（自发缓解，30例；IVIG治疗后缓解，10例；脾切除术后缓解，18例）；血小板值达到止血水平，22例（自发缓解，16例；IVIG治疗后，5例；脾切除术后，1例）。13名儿童失访，7名仍有血小板减少但无症状。这些数据表明，儿童慢性ITP在大多数情况下病程呈良性，无论是否接受治疗，甚至在发病数年之后都可能缓解。因此，治疗干预必须个体化，而脾切除术并不总是安全的，应仅用于对传统治疗方式无反应的疑难病例。
