1.Progress in the treatment of Langerhans cell histiocytosis
Journal of Clinical Pediatrics 2015;(3):291-294
Treatment of Langerhans cell histiocytosis (LCH) needs to be tailored for each individual patient according to LCH classiifcation currently. Single-system LCH (SS-LCH) has an excellent prognosis. However, there is a poor prognosis in multisystem LCH (MS-LCH) with risk organs (RO) involvement and refractory or recurrent LCH (Re-LCH). The prognosis of MS-LCH with RO involvement and Re-LCH has been improved markedly accompanying with progress of chemotherapy in recent years. The 5-year survival rate of MS-LCH reached above 80%, and the effective rate of Re-LCH reached above 60% after chemotherapy. Re-LCH can be cured by hematopoietic stem cell transplantation.
2.Comparison of anti-D immunoglobulin and high-dose intravenous immunoglobulin therapy in childhood acute idiopathic thrombocytopenic purpura:a systematic review and meta-analysis
Yongan NI ; Zhuang LIU ; Bicai LIAO
Journal of Clinical Pediatrics 2013;(8):768-773
Objectives To compare the effectiveness and safety of intravenous anti-D immunoglobulin (IV anti-D) with high-dose intravenous immunoglobulin (IVIG) as initial treatments of acute idiopathic thrombocytopenic purpura (ITP) in children. Methods Randomized controlled trials comparing anti-D with high-dose IVIG in the treatment of childhood acute ITP were systematically reviewed from PubMed, Embase and Cochrane Central Register of Controlled Trials and hand-searched reference lists. The number of patients with a platelet count>20 × 109/L at 72 hours after treatment initia-tion, and the decrease in hemoglobin were the primary outcomes. The meta-analysis was performed by RevMan 5.1. Results A total of 771 relevant articles were retrieved, and ifve studies were included. The RR (anti-D versus high dose IVIG) of achieving a platelet count>20×109/L at 72 hours was 0.90 (95%CI:0.82~0.98). However, subgroup analysis suggested no signiifcant difference between anti-D at a dose of 50μg/kg and high-dose IVIG (RR 0.98, 95% CI: 0.84~1.13), as well as between anti-D at a dose of 75μg/kg and high-dose IVIG (RR 0.88, 95%CI:0.75~1.03). Hemoglobin drop was greater in the anti-D group. No patients, however, required transfusions of erythrocyte suspensions. Conclusions IV anti-D may be as ef-fective as high-dose IVIG in the treatment of childhood acute ITP at 72 hours after therapy. The side effects of anti-D were tolerated and acceptable.
3.Clinical features and outcomes of 78 children with non-Hodgkin lymphoma
Jian JIANG ; Xuewen SONG ; Huijuan XU ; Ren ZHONG ; Yongan NI ; Lirong SUN
Journal of Clinical Pediatrics 2015;(8):715-719
ObjectiveTo explore the clinical features and factors inlfuencing the prognosis of childhood non-Hodgkin's lymphoma (NHL).MethodsPathologically diagnosed 78 pediatric patients with NHL and treated in the Afifliated Hospital of Qingdao University from January 2004 to August 2013 were collected and analyzed. Patients were grouped according to age, sex, tumor size, immunologic classiifcation, B-symptoms, LDH, hemoglobin and clinical staging. The 5-years event-free survival rate (EFS) were calculated and analyzed by Kaplan-Meier method, and the difference of the survival rate between groups were com-pared. Using Cox proportional hazards model, we analyzed the possible factors that might inlfuence 5-years event-free survival rate EFS , such as age and clinical staging. TheOR value and the 95%CI were calculated.ResultsAmong the 78 cases, median age of onset is 7 years old, male to female ratio is 2.90:1, there are 25 cases of T-cell type and 53 cases of B-cell type. According to pathological types,Burkitt lymphoma is the most common (34.6%), followed by T-lymphoblastic lymphoma (20.5%), diffuse large B-cell lymphoma (11.5%). According to the St. Jude malignant lymphoma staging system, there are 2 cases in stage I, 9 in stageⅡ, 35 in stageⅢ and 32 in stageⅣ. Swelling of periphery lymph node (80.7%) was observed as initial symptom in 26 cases of lymphoblastic lymphoma. Among 45 cases of mature B-cell tumor, the main clinical feature including abdominal cavity and gingival were observed in 27 cases of Burkitt lymphoma. Among the 73 cases received treatments, 66 cases (90.5%) attained CR (complete remission) and 4 cases (5.5%) attained PR (partial remission) by cytology and radiographic assessment after two course of combined chemotherapy, 2 cases (2.7%) rapidly relapsed after the remisson of one course treatment, 1 case (1.3%) appeared the central nervous system inifltration in the chemotherapy. With median follow-up time of 42 months, the 5-year EFS of the 73 cases was (67.0+5.5)%. Single factor analysis showed that B-symptom, LDH, and clinical staging were signiifcantly correlated with prognosis (P<0.05), while age, sex, tumor size, hemoglobin and immune classiifcation was independent of prog-nosis (P>0.05). Multiple factor analysis showed that LDH and clinical staging inlfuenced the prognosis (OR=3.34,95%CI 2.275?10.683,P<0.01;OR=4.354,95%CI 1.519?12.475,P<0.01) .Conclusionclinical features of childhood NHL are variable. LDH and clinical staging at primary diagnosis are important factors affecting the prognosis.