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儿童慢性特发性血小板减少性紫癜的转归。

Outcome of chronic idiopathic thrombocytopenic purpura in children.

机构信息

Hematology/Oncology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Chandigarh, India.

出版信息

Pediatr Blood Cancer. 2010 Mar;54(3):403-7. doi: 10.1002/pbc.22346.

Abstract

BACKGROUND

There is paucity of data on long-term probability of remission in chronic idiopathic thrombocytopenic purpura (ITP). Aim was to study the course and factors influencing remission of chronic ITP. Chronic ITP was defined as thrombocytopenia persisting >6 months following initial diagnosis.

PROCEDURES

Case-records of children with chronic ITP, aged <14 years, were reviewed in this retrospective study (1987-2006).

RESULTS

Two hundred seventy children were followed. Median age at diagnosis was 6 years. Median duration of follow up was 30 months (range 6-166). Isolated thrombocytopenia (even if <10 x 10(9)/L) in the absence of "significant" bleeds, by itself was not considered an indication for drug therapy. Sixty-seven (24.8%) children attained complete remission (CR) over a median period of 18 months (range 7-120). The probabilities of remission at 5 years for males and females were 24% and 39.6%, respectively (P = 0.01). The probability of achieving remission at 10 years in children <8 and > or =8 years was 51.2% and 34%, respectively (P = 0.02). The probability of remission at 5 years for children who received some treatment, versus no treatment was 31.4% and 27%, respectively (P = 0.8). Nine of 18 children, who underwent splenectomy, achieved CR. Intracranial hemorrhage (ICH) occurred in 11 (4%) cases. The time of occurrence of ICH from onset of symptoms varied from 6 to 55 months.

CONCLUSIONS

The predicted spontaneous remission rate with chronic ITP was 30% and 44% at 5 and 10 years, respectively. Platelet count at diagnosis and the treatment administered did not influence remission outcomes. Age <8 years and female gender were predictors of a favorable outcome.

摘要

背景

慢性特发性血小板减少性紫癜(ITP)患者长期缓解的概率数据有限。本研究旨在探讨慢性 ITP 的病程和影响缓解的因素。慢性 ITP 定义为初始诊断后持续 >6 个月的血小板减少症。

方法

本回顾性研究分析了 1987 年至 2006 年期间年龄<14 岁的慢性 ITP 患儿的病历资料。

结果

共 270 例患儿纳入研究,中位诊断年龄为 6 岁,中位随访时间为 30 个月(6-166 个月)。孤立性血小板减少症(即使血小板计数<10×109/L),且无“显著”出血,本身不考虑药物治疗。67 例(24.8%)患儿中位缓解时间为 18 个月(7-120 个月),获得完全缓解(CR)。男女患儿 5 年缓解率分别为 24%和 39.6%(P=0.01)。<8 岁和≥8 岁患儿 10 年缓解率分别为 51.2%和 34%(P=0.02)。接受治疗和未接受治疗的患儿 5 年缓解率分别为 31.4%和 27%(P=0.8)。18 例行脾切除术的患儿中,9 例获得 CR。11 例(4%)患儿发生颅内出血(ICH)。ICH 从发病到发生的时间为 6-55 个月。

结论

慢性 ITP 的自发缓解率预测值分别为 5 年时 30%和 10 年时 44%。诊断时的血小板计数和治疗方式对缓解结果无影响。<8 岁和女性是预后良好的预测因素。

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