ABSTRACT
Objective:To investigate the clinical characteristics and gene mutations of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) secondary to familial hemophagocytic syndrome (FHL).Methods:The clinical features, disease evolution, gene mutation and genetic characteristics of 1 SPTCL patient secondary to FHL in Henan Children's Hospital in June 2012 were analyzed retrospectively, and the related literatures were reviewed.Results:The UNC13D of FHL patient was homozygous mutation accompanied by STXBP2 heterozygous mutation, while that of his parents and elder brother was heterozygous mutation. After regular chemotherapy with HLH-2004 regimen, the disease relapsed 4 years later, and secondary SPTCL developed after 1 year of remission with the second chemotherapy. After giving SMILE regimen chemotherapy, allogeneic hematopoietic stem cell transplantation was performed, and now the patient had disease-free survival.Conclusions:The detection of related genes in children with hemophagocytic syndrome should be improved in time to confirm the diagnosis of primary disease. FHL can follow SPTCL, and chemotherapy combined with allogeneic hematopoietic stem cell transplantation can be the only method to cure this disease.
ABSTRACT
Objective To explore the clinical efficacy and adverse reactions of children with acute promyelocytic leukemia (APL) by adopting Chinese children APL-2010-protocol in single center.Methods Forty-seven children with newly diagnosed APL in Soochow University Affiliated Children's Hospital from October 2010 to September 2015 were selected,including 30 boys and 17 girls with the median age of 7 years (1.2-14.0 years).The patients were divided into 3 groups,including 11 cases in the low-risk group,20 cases in the intermediate-risk group and 16 cases in the high-risk group.Their clinical features and therapeutic prognosis were analyzed retrospectively and the clinical efficacies were compared in low-risk group[white blood cell(WBC) ≤ 10 × 109/L,platelets (PLT) ＜ 40 × 109/L],intermediate-risk group (WBC ≤ 10 × 109/L,PLT ≤40 × 109/L) and high-risk group (WBC ＞ 10 × 109/L).Five-year event-free survival (EFS) rate and overall survival (OS) rate of each group were analyzed by using Kaplan-Meier curve method.Results The peak comparative difference of peripheral blood WBC among 3 groups was statisticallysignificant(x2 =7.618,P =0.002).The occurrence rate of disseminated intravascular coagulation (DIC) was 68.8％ and the occurrence rate in the high-risk group was higher compared with low-risk group and intermediate-risk group (x2 =8.217,P =0.016).Arsenic trioxide related side effects including abnormal liver tests,electrocardiogram and anaphylactic reaction were invertible after supportive therapy.Hematologic complete response (HCR) rate in the high-risk group was 93.7％,HCR time was (39.3 ± 2.7) days and molecular complete remission (MCR) time was(71.0 ± 9.7) days.HCR rate in the intermediate-risk group was 95.0％,HCR time was (44.6 ± 3.1) days and MCR time was (80.0 ± 8.2) days.HCR rate in the low-risk group was 100.0％,HCR time was (32.4 ±2.3) days and MCR's time was (71.5 ± 12.0) days.HCR time among 3 groups had statistical significance (F =3.652,P =0.034),but HCR rate and MCR time among 3 groups showed no statistical significance (F =0.318,P =0.729).In the high-risk group,EFS rate was (93.3 ± 6.4) ％,5-year OS rate was (86.7-± 8.8) ％.Meanwhile,5-year OS rate and EFS rate were 100.0％ in other 2 groups.Conclusions Treating patients with APL by using 2010 protocol according to the risk stratification in our center has achieved high long-time survival.The higher the risk,the higher the incidence of DIC in induction period,but eventually achieve a very high rate of 5-year EFS was achieved.No chronic arsenic toxicity or second malignancies were found during the follow-up time.