Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.

OBJECTIVE: To establish HPLC method for the determination of thioguanosine in human red blood cells. METHODS: The determination was performed on Hypersil ODS colum. The mobile phase consisted of methanol-water (10∶90) with detection wavelength at 342nm. 6-Thioguanine(6-TG)was generated from thioguanosine through heating and hydrolyzing. 6-TG was extracted into 0.1mol/L hydrochloric acid for sample injection assay with external reference method for the quantification. RESULTS: The linear range of 6-TG was 30～1 200pmol/8?108RBCs. Its optimum hydrolyzing time was 1 hour and optimal extraction pH value ranged from 11 to 12. The content of phenyl mercury acetate in the extraction solution was 1.3mmol/L. CONCLUSION: Under optimized conditions, HPLC method for the determination of thioguanosine is fast, accurate and sensitive.

Objective To report the clinical features,management and prognosis of local recurrence with inferior vena cava tumor thrombus for renal cell carcinoma after laparoscopic partial nephrectomy.Methods A case of 37-year-old man was admitted to hospital for recurrent renal tumor in May 2013.The patient undervent laparoscopic partial nephrectomy for right kidney mass in June 2011,and renal cell carcinoma was diagnosed by pathological examination.The patient complained about pain of right flank in May 2013.Imaging examination (including CT,MRI and PET-CT) showed a 5 cm tumor on the upper-middle right kidney associated with inferior vena cava tumor thrombus,bulky retroperitoneal lymph nodes and extensive local invasion including psoas,ureter,perirenal fascia.Surgical excision of local recurrence,nephrectomy plus inferior vena eava thrombectomy was performed.Results Right nephrectomy and local recurrence incision plus inferior vena eava thrombectomy was performed successfully.The operation time was 360 min,blood loss was 4 000 ml.The patient was discharged on the 14th day after surgery.The pathological characteristics were as follows:the tumor was limited to the upper-middle right kidney,psoas,ureter,and inferior vena cava tumor thrombus,but carcinoma was not found in the lymph nodes,pelvis and others.The final pathological diagnosis was papillary renal cell carcinoma,type Ⅱ.Local recurrence and other distant metastasis were not demonstrated with CT in 12 months' follow-up.Conclusion Excision of locally recurrent renal cell carcinoma is possible,and complete surgical resection could lead to prolonged disease-free survival.

Objective To evaluate the outcomes of mature B-cell acute lymphoblastic leukemia(mature B-ALL) and to assess the safety and efficacy of the treatment protocol.Methods From February of 2003 to December of 2012,15 children were diagnosed as mature B-cell acute lymphoblastic leukemia/lymphoma possible (mature B-ALL/NHLp) in Shanghai Children's Medical Center(SCMC) were enrolled,and they were treated with SCMC-mature B-ALL/NHLp-2003 protocol.All of the clinical characteristics,therapeutic effects and long-term outcomes were analyzed.The statistical data were processed by SPSS 21.0.Results The median age on diagnosis was 8.7 years (1 year and 5 months to 14 years and 4 months).Among them,4 cases presented with local mass including maxillofacial tumors,neck and abdominal mass.The others had systemic manifestations such as fever and pale face.These neoplastic cells retained the expressions of surface membrane immunoglobulin M,terminal deoxynucleotidyl transferase,Cμ,CD10,CD19,cCD79 a differently.Follow-up was updated to November 30,2013.The median follow-up period was 80 months (39-128 months).Theestimated 5-year event free survival rate was (80.0 ± 10.3) ％.According to univariate analysis,increased lactate dehydrogenase level (＞ 4-times the normal value),increased serum ferritin level (＞ 2-times the normal value),no small residual disease markers were indepen-dent poor prognostic factors(x2 =5.49,4.89,5.49,all P ＜ 0.05).Conclusions SCMC-mature B-NHL/ALLp-2003 protocol is feasible and safe for children with mature B-ALL/NHLp,but more sample cases need to be investigated.

Objective To evaluate the outcomes of children with stage Ⅳ malignant extracranial germ cell tumors. Methods Twenty-five patients were enrolled in the retrospective analysis. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier method with SPSS 13.0. Results Of the 25 children, there were 13 males and 12 females. The mean age at diagnosis was 2 years old (ranged 1 to 11). Five patients receiving chemotherapy in another hospital before (n=1), or giving up treatment after confirmed diagnosis (n=1), or giving up effective treatment after received less than 2 cycles (n=3) were excluded from this analysis. Of the 20 patients, 90.0% (18/20) achieved complete remission and 5.0% (1/20) achieved partial remission after treatment. The 5-year EFS rate and 5-year OS rate were 70.0%±10.2% and 82.4%±9.2% respectively. There was no death occurred due to complications. Conclusions The effect of this treatment program is positive. The cumulative dose of the drugs is not high, compared with other schemes such as PEB, but there are more drugs involved. Whether these drugs may cause long-term adverse reactions needs further research.

Objectives To evaluate the long-term outcomes of childhood low-or intermediate-risk neuroblastoma (NB) and their relevant prognostic factors. Methods A total of 70 new cases of low-or intermediate-risk NB diagnosed and treated by NB-99 protocol between 1999 and 2008 were analyzed retrospectively. Results Of these 70 NB patients, fourteen patients were in low-risk group and 56 were in intermediate-risk group. Sixty-seven patients reached complete remission (CR) or very good partial remission and 3 (5%) achieved partial remission. Ten patients relapsed. One patient occured second malignant neo-plasm. No patients died of chemotherapy-related adverse events or infections. The 5 year overall survival rate was 85.9%, event-free survival rate was 81.0%. Bone marrow infiltration, age at diagnosis, stage, lactate dehydrogenase level had a significant effect on prognosis. Conclusion Develop cytogenetic and molecular biology tests and pretreatment risk stratification are im-portant for further improvement of treatment protocol.

Objectives To evaluate the clinical features, treatment scheme and long-term outcomes of stage 1、2 childhood neuroblastoma (NB). Methods The retrospective study included 49 newly diagnosed NB stage 1、2 patients from June 1998 to December 2010. Clinical data and long-term outcomes were analyzed. Results Twenty-four patients with stage 1 NB and twenty patients with stage 2 NB were found among all 237 patients with NB enrolled in this study. The median age at diagnosis was 25 months( 2 week to 9 year old),29 males and 20 females. Thirty-one patients (63.6%) without symptoms were discovered with tumor by physical or imaging examination. Thorax and abdomen were the most common sites of primary tumor (21 and 22 cases, accounting for 42.9% and 44.9% of all patients, respectively). Forty (81.6%) NB patients had favorable pathology classification. One patient was of MYCN amplification status. Urine vanilla mandelic acid was normal in 32 (91.4%) patients, and serum lactate dehydrogenase was less than five times of the normal value in all patients. Ten NB patients were treated ac-cording to the low-risk protocol who received surgery alone.Thirty-nine patients were treated according to intermediate-risk protocol who received both surgery and chemotherapy. All the patients achieved very good partial remission (100%).The medi-an follow-up period was 60 months(22 months to148months). Nine patients were lost after a follow up of 3 months in medi-an. The 2-、3-、5-year event free survival and overall survial of all 49 patients was 100%. Conclusions The prognosis for neu-roblastoma of stage 1、2 in this study was with 100%survival, which provides opportunity for further reduction of dosage and/or duration of episodes in chemotherapy.

Objective: To investigate the efficacy and the prognostic factors in pediatric hepatoblastoma according to the standard diagnostic and therapeutic regimen. Method: Eighty-four consecutive patients were enrolled in this study between June 2000 and June 2015. Diagnosis and staging was decided by the multi-disciplinary team including oncologists, surgeons, pathologists and sub-specialized radiologists refering to protocol of Children′s Oncology Group(COG) and International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL) in a case observational study. Univariate analysis was tested by the log-rank and multivariate analysis by COX regression. All consecutive cases were divided into low risk group and high risk group according to grouping criteria. Complete remission was defined as both imaging negative and α fetoprotein (AFP) normalization. Retrospective analysis was performed in clinical features, long-term outcomes and prognostic factors. Result: Ten patients were excluded because of giving up after less than or equal to three cycles of treatment. A total of 74 cases were included in this study; 45 males and 29 females. The median age at diagnosis was 1.7 years(range 0.2-14.8 years). Untill August 30, 2016, the median follow-up time was 24.2 months (range 4.1-135.3 months); 59 cases achieved complete remission.The estimated five years overall survival (OS) and event free survival(EFS) were 90%(68/74)and 72%(58/74). AFP could be normalized after 5 circles of treatment or 2 circles of postoperation.In univariate analysis , the five years OS and EFS in low risk group were both 100%(18/18), and those in high risk group were 88%(50/56)and 68%(40/56), respectively. The five years OS rates were 75%(15/19) and 95%(53/55) in patients with or without distant metastasis (P=0.016). After 3 cycles of chemotherapy post tumor resection, we divided these patients into 2 groups according to AFP recover or not, the five years OS were 100%(43/43)and 81%(22/26), respectively (P=0.011). Conclusion The result of this protocol is reasonable when comparing with other worldwide research. Except for staging, metastasis, pathological subtypes, postoperative AFP recover or not is a prognostic factor after 3 cycles of chemotherapy.

Objective: To investigate the long-term efficacy and prognostic factors of pediatric relapsed Wilms tumor (WT) after retreatment. Method: Sixteen children in Shanghai Children′s Medical Center with relapsed Wilms tumor were enrolled consecutively in this study between April 2006 and June 2016. All patients were diagnosed according to pathology, imaging and medical and surgical oncologist′s assistance. Relapse treatment included surgical excision, chemotherapy and selective radiation therapy. The clinical features, long-term outcomes and prognostic factors of patients were analyzed retrospectively.Survival data were analyzed by Kaplan-Meier.Log-Rank analysis was used for univariate analysis. Result: One case was excluded because of giving up the therapy even though no disease progress was identified. A total of 15 cases (5 males and 10 females) were included in this study. The median age at diagnosis was 3.8 years (range 0.5-9.1 years). The tumor staging at diagnosis included one case of stageⅠ, 7 cases of stageⅡand 7 cases of stage Ⅲ. Among cases of stage Ⅲ, 6 cases had radiation therapy history. The pathology of all patients′ recurrent tumor was favorable histology (FH). The median follow-up time was 34.6 months (range 12.5-132.7 months) until March 21, 2017. The time from initial diagnosis to relapse was 7.9 months (range 3.1-17.9 months). Four cases experienced local recurrence, 9 cases relapsed with metastases (6 cases in lungs, 2 in livers, 1 in mediastinum) and 2 cases relapsed in both local site and with metastases. Except to 2 cases received irregular retreatment, 13 cases received regimen I (doxorubicin, vincristine, epoposide and cyclophosphamide for 25 weeks) as relapsed chemotherapy. Five cases received autologous bone marrow transplantation (ABMT). Until the last follow-up, 8 cases achieved continuous complete remission (range 6.7-104.3 months), 3 cases had relapse again or progressing and 4 cases died. The estimated 5-year overall survival (OS) rate and event free survival (EFS) rate were (70±15)% and (52±15)%. According to whether received ABMT or not, the 5-year EFS rate were 51% and 53%. According to whether relapsed within 6 months after diagnosis or not, the 5-year EFS rate were 38% and 56% respectively. Conclusion The 5-year EFS rate of pediatric relapsed FH WT have reached above 50% by multi-disciplinary treatment in our experience and we encourage patients and doctors to receive retreatment.

OBJECTIVETo explore the efficiency and safety of immune thrombocytopenia（ITP）in children through classification treatment.

METHODS405 newly diagnosed ITP patients were enrolled in this study from January 1st 2013 to August 31st 2014. The cases were divided into observation group and therapy group according to the initial platelet count of less than 20×10⁹/L or the cases of active bleeding. There were 104 male cases and 76 female cases in observation group with the media platelet count of 46 （20-89）×10⁹/L. They were followed up with a median of 20 months. The therapy group，including 131 males and 94 females with a median platelet count of 11（1-19）×10⁹/L, were followed up by 22 months.

RESULTSThe total curative rate at acute period was 80.44%（181/225）in therapy group with the first line treatment. In observation group, 148 cases（82.22%）reached complete response（CR）or response（R） criteria. 44 patients came into persistent period with an effective rate of 34.09%（15/44）in therapy group. The overall effectiveness over one year was 87.11%（196/255）. In observation group, 32 cases came into persistent period and 13 cases（40.63%）got the CR or R line. After one year of observation, 161 cases （89.44%）reached the CR or R standard. In therapy group, 5 out of 29 patients（17.24%）in chronic period got CR or R. While in observation group, 6 out of 19 cases（31.58%）reached the CR or R standard. The elder children over 10 years had risk factors in response in two groups. There was no severe bleeding or adverse effect or dead cases in this study.

CONCLUSIONIt is reasonable to take platelet count <20×10⁹/L and（or）active bleeding as the dividing line for classification therapy indications. Nearly half of the cases could avoid over therapy and decreased the risk of drugs side effect to improve life quality.

Child ; Female ; Hemorrhage ; Humans ; Male ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; classification ; therapy ; Quality of Life ; Remission Induction ; Risk Factors