非肿瘤性血液病住院患儿血小板无效输注——单中心资料分析

目的通过对我院非肿瘤儿童病房血小板无效输注病例临床资料分析,了解儿童血小板无效输注的原因,为临床诊治提供帮助。方法顺序收集2015年1月到2015年12月北京儿童医院血液科非肿瘤病房住院的发生血小板无效输注的40例患儿病例,对相关数据进行回顾性分析。结果 40例患儿输注前中位血小板数目为10.2×10~9/L(0.0~20×10~9/L);出血情况:0级12例(30%)、Ⅰ级10例(25%)、Ⅱ级8例(20%)。Ⅲ级6例(15%),IV级4例(10%)。原发疾病:噬血细胞综合症10例(25%)、骨髓增生异常综合症6例(15%)再生障碍性贫血8例(20%)、免疫性血小板减少症7例(17.5%)、系统性红斑狼疮2例(5%)、慢性活动性EB病毒感染1例(2.5%),弥散性血管内凝血(DIC)3例(7.5%)、其他3例(7.5%)。发生无效输注原因:免疫性因素21例(占52.5%):自身抗体5例、同族免疫性抗体16例(HLA特异性抗体10例、血小板特异性抗原(HPA)抗体6例),非免疫因素19例(47.5%):包括分布异常8例、非分布异常11例。治疗及预后:发生血小板输注无效后,在积极治疗原发病同时,针对21例免疫给用糖皮质激素和(或)免疫球蛋白,18例有效(有效率85.7%);32例应用促血小板生成素(rh TPO),25例有效(有效率78.1%)。结论非肿瘤性血液病患儿可发生血小板无效输注,引起严重血小板减少以及出血症状,免疫因素和非免疫因素均为常见发病机制,积极治疗原发病针对性应用免疫治疗可以起到有效控制作用。

Non Neoplastic Hematologic Diseases in Hospitalized Children with a Single Center data Analysis

Objective To analyze the reason for Platelet transfusion refractoriness (PTR)according to the clinical data of Kofi children's ward,which can provide help for clinical diagnosis and treatment.Methods Collecting in sequence in Beijing Children's hospital blood Kofi oncology ward hospitalized of ineffective platelet infusion of 40 cases of patients from January 2015 to December 2015,data were analyzed retrospectively.Results General situation:40 cases,transmission of platelet number fluctuations in the 10.2 × 1010/L lose before injection in a number of platelet.Bleeding:0 grade 12 cases(30％),Ⅰ grade 10 cases (25％),Ⅱ grade 8 cases (20％),Ⅲ grade 6 cases (15％),Ⅳ grade 4 cases (10％).Primary disease:hemophagocytic syndrome in 10 cases (25％),bone marrow hyperplasia myelodysplastic syndrome in 6 cases (15％) regeneration barrier anemia in 8 cases (20％),primary immune thrombocytopenic disease in 7 cases (17.5 ％),systematic lupus erythematosus in 2 cases (5 ％),slow activity of Epstein Barr virus infection in 1 case(2.5％).disseminated intravascular coagulation (DIC) 3 cases (7.5％),other 3 patients (7.5％).Ineffective transfusion reason:immune factors in 21 cases (52.5％):autoantibodies in 4 cases,isoimmune antibody in 16 cases,drug 1 case:HLA specific antibodies in 10 cases,platelet specific antigen (HPA) antibody in 6 cases;15 cases of non immune factors(37.5％),including splenomegaly in 6 cases,fever in 3 cases,infection in 4 cases,DIC 1 cases.3,treatment and prognosis:after the occurrence of platelet transfusion refractoriness,aiming at the application of immunity of,treatment with hormone and (or) immune globulin as well as in the active treatment of the original diseases at the same time,efficiency of 60％;for non immune applications,giving special with platelet,anti infection,(20％);all cases with TPO,efficiency of 60％.In 10 cases(25％)of the control of the Central Plains in the effective cases,2 cases (5％)were controlled by the original disease.Conclusion The patients with non neoplastic hematologic diseases can suffer from PTR which can lead to severe thrombocytopenia and bleeding symptoms and needs to be identified correctly and treated actively.Immune and non immune factors are common pathogenesis.Active treatment of the original disease targeted immunotherapy can play an effective contro.