脾切除治疗儿童慢性原发性免疫性血小板减少症疗效分析
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摘要
目的研究儿童慢性原发性免疫性血小板减少症患者行脾切除术的疗效。方法选择2005年6月-2013年12月间在山西省儿童医院确诊的儿童慢性原发性免疫性血小板减少症患者6例,均经静脉注射用人免疫球蛋白、糖皮质激素、免疫抑制剂治疗无效,病程>1年,年龄>6岁,且有活动性出血需反复住院,严重影响患儿的生活质量,行脾切除治疗后分析其疗效。结果除1例术后随访8年,血小板持续低于20×10~9/L,但出血症状减轻外,远期总有效率达83.3%;另1例血小板恢复正常,但在术后半年发生化脓性脑膜炎,2年后因发热、白细胞高确诊结缔组织病(未分化型)外,余4例患者均治愈,且无严重感染等并发症。结论脾切除治疗儿童慢性原发性免疫性血小板减少症安全、有效,对经一线及二线药物治疗仍无明显效果的患者可以选择脾切除治疗。
Objective To investigate the clinical efficacy of splenectomy in children with chronic primary immune thrombocytopenia(CITP).Methods 6 children with CITP received splenectomy from June 2005 to December 2013,after getting no response to treatment with IVIG、corticosteroids,immunosuppressive agents,during the period over 1 year,all the patients were more than 6 years old.Results The long-term response rate was 83.3%,1 case was followed up for 8 years,with platelet counts less than 20 ×10~9 /L,although the bleeding symptom improved;another 1 case developed purulent meningitis 6 months later,even after platelet fully recovered.2 years later this patients was diagnosed as Connective Tissue Diseases(undifferentiated).The remaining 4 cases were cured,without any serious complications.Conclusions Splenectomy in children with CITP is safe and effective,for those who were failure to the first-line and second-line treatments.
Objective To investigate the clinical efficacy of splenectomy in children with chronic primary immune thrombocytopenia(CITP).Methods 6 children with CITP received splenectomy from June 2005 to December 2013,after getting no response to treatment with IVIG、corticosteroids,immunosuppressive agents,during the period over 1 year,all the patients were more than 6 years old.Results The long-term response rate was 83.3%,1 case was followed up for 8 years,with platelet counts less than 20 ×10~9 /L,although the bleeding symptom improved;another 1 case developed purulent meningitis 6 months later,even after platelet fully recovered.2 years later this patients was diagnosed as Connective Tissue Diseases(undifferentiated).The remaining 4 cases were cured,without any serious complications.Conclusions Splenectomy in children with CITP is safe and effective,for those who were failure to the first-line and second-line treatments.
引文
1.卢新天.儿童原发性免疫性血小板减少症诊断治疗的国际共识.中国小儿血液与肿瘤杂志,2011,16:283-286.
2.徐倩,刘文君.儿童原发性免疫性血小板减少症诊断与治疗.中国实用儿科杂志,2013,128:646-651.
3.张之南,沈悌.血液病诊断及疗效标准.第3版.北京:科学出版社.2007:172-174.
4.Zhang F,Chu X,Wang L,et al.Cell-mediated lysis of autologous platelets in chronic idiopathic thrombocytopenic purpura.Eur J Haemotol,2006,76:427431.
5.谷欣,朱燕昆.免疫性血小板减少性紫癜患者行腹腔镜脾切除术后免疫功能的研究.2012,33:121-124.
6.杨仁池,周泽平.脾切除治疗原发性免疫性血小板减少症.临床血液学杂志,201 1,24:380-383.
7.林晓骥,姚荣欣,朱宝玲,等.脾切除治疗特发性血小板减少性紫癜的疗效观察及预后因素分析.临床血液学杂志,2011,24:270-272.
8.Lyori H,Bessho F,Ookawa H,et al.Intracranial hemorrhage in children with immune thyombocytopenic purpura,Ann Hematol,2000,79:691-695.
9.林瑞祥,张洪红.部分脾脏柃塞治疗免疫性血小板减少性紫癜8例.福建医药杂志,201 1,33:32-34.
10.胡群.脾切除在治疗儿童慢性特发性血小板减少性紫癜中的意义.中国小儿血液与肿瘤杂志,201 1,16:55-56.
2.徐倩,刘文君.儿童原发性免疫性血小板减少症诊断与治疗.中国实用儿科杂志,2013,128:646-651.
3.张之南,沈悌.血液病诊断及疗效标准.第3版.北京:科学出版社.2007:172-174.
4.Zhang F,Chu X,Wang L,et al.Cell-mediated lysis of autologous platelets in chronic idiopathic thrombocytopenic purpura.Eur J Haemotol,2006,76:427431.
5.谷欣,朱燕昆.免疫性血小板减少性紫癜患者行腹腔镜脾切除术后免疫功能的研究.2012,33:121-124.
6.杨仁池,周泽平.脾切除治疗原发性免疫性血小板减少症.临床血液学杂志,201 1,24:380-383.
7.林晓骥,姚荣欣,朱宝玲,等.脾切除治疗特发性血小板减少性紫癜的疗效观察及预后因素分析.临床血液学杂志,2011,24:270-272.
8.Lyori H,Bessho F,Ookawa H,et al.Intracranial hemorrhage in children with immune thyombocytopenic purpura,Ann Hematol,2000,79:691-695.
9.林瑞祥,张洪红.部分脾脏柃塞治疗免疫性血小板减少性紫癜8例.福建医药杂志,201 1,33:32-34.
10.胡群.脾切除在治疗儿童慢性特发性血小板减少性紫癜中的意义.中国小儿血液与肿瘤杂志,201 1,16:55-56.