Inborn errors of immunity and its clinical significance in children with lymphoma in China: a single-center study.

OBJECTIVE: To investigate the incidence, clinical and genetic characteristics of pediatric lymphoma patients of China with inborn errors of immunity (IEI)-related gene mutations, which have not been fully studied. METHOD: From Jan. 2020 to Mar. 2023, IEI-related genetic mutations were retrospectively explored in 108 children with lymphomas admitted to Beijing Children's Hospital by NGS. Genetic rule and clinical characteristics as well as treatment outcomes were compared between patients with or without IEI-related gene mutations. RESULTS: A total of 17 patients (15.7 %) harbored IEI-associated mutations, including 4 cases with X-linked lymphoproliferative syndrome (XLP), 3 cases had mutations in tumor necrosis factor receptor superfamily 13B (TNFRSF13B), 2 cases with Activated p110 syndrome (APDS). Patients with IEI all had alteration of immunocompetence with decreased levels of immunoglobulin and lymphocyte subsets. Recurrent infection existed in 41.2 % of patients. The 18-month event-free survival (EFS) and the overall response rate (ORR) of patients with IEI are significantly lower than those without IEI (33.86% vs. 73.26 %, p = 0.011; 52.94% vs. 87.91 %, p = 0.002, respectively). In addition, patients with IEI had a higher progression disease (PD) rate of 23.5 % than those without IEI of 4.4 % (p = 0.006). CONCLUSION: The present study demonstrated that IEI-associated lymphomas were much more common than originally appreciated in pediatric lymphomas, and those were insensitive to treatment and more likely to progress or relapse. The genomic analysis and a thorough review of the medical history of IEI can be used to distinguish them from pediatric lymphomas without IEI, which are beneficial for the early diagnosis and direct intervention.

Efficacy of ruxolitinib for HAVCR2 mutation-associated hemophagocytic lymphohistiocytosis and panniculitis manifestations in children.

Frequent germline mutations of HAVCR2, recently identified in subcutaneous panniculitis-like T-cell lymphoma (SPTCL), are associated with an increased risk of hemophagocytic lymphohistiocytosis (HLH). However, SPTCL-HLH represents a challenge because of the difficulties in treatment with poor survival. Its malignant nature, specifically harbouring HAVCR2 mutations, has also been questioned. To better understand its pathology and treatment, we analysed the clinical data of six patients diagnosed at our centre. The median age at onset was 10.5 years (range, 0.8-12.4). Five patients presented with skin lesions of subcutaneous nodules/plaques and/or ulceration. All patients developed HLH; notably, one infant only had HLH without skin involvement. Histopathologically, only two patients were diagnosed with SPTCL and three were reported as panniculitis with no sufficient evidence of lymphoma. Genetically, germline homozygous mutation of HAVCR2 (p.Y82C) was identified in all patients, with a median diagnosis time of 4.6 months. All patients initially received corticosteroids, immunosuppressants or chemotherapy, achieving unfavourable responses. Strikingly, they responded well to ruxolitinib targeting inflammatory cytokines, allowing rapid disease resolution and/or long-term maintenance of remission. The excellent efficacy of ruxolitinib highlights this disease as an inflammatory condition instead of neoplastic nature and indicates novel agents targeting key inflammatory pathways as an encouraging approach for this disease entity.

Diagnosis and treatment of pediatric anaplastic lymphoma kinase-positive large B-cell lymphoma: A case report.

BACKGROUND: Anaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare type of lymphoma with high invasiveness and rapid progression. It occurs in all age groups, but is extremely rare in children. The lesions mainly involve the lymph nodes and may present with extra-nodal involvement. Response to conventional chemotherapies and local radiotherapy is poor, with a 5-year overall survival of less than 40%. Recently, the use of ALK inhibitors for the treatment of this disease has been reported. CASE SUMMARY: We present a case of a 12-year-old boy diagnosed with ALK+LBCL. The patient had a 2-mo medical history of a calvarial mass, extensive systemic involvement, and positive bone marrow clathrin heavy chain (CLTC)-ALK fusion gene. Complete remission 1 (CR1) was achieved using the modified LMB89 Group C regimen followed by autologous stem cell transplantation. The patient relapsed 3 mo later. He then achieved CR2 with three short courses of chemotherapy (COP, reduced-dose ICE, low-dose Ara-c+VP16) and continuous alectinib targeted therapy. Afterward, allogeneic hematopoietic stem cell transplantation (allo-HSCT) was performed. At 16 mo after the allo-HSCT, the patient was still in CR2. CONCLUSION: The modified LMB89 Group C regimen and ALK inhibitors are effective. Allo-HSCT should be performed after remission.

Treatment outcome in children with central nervous system-positive Burkitt lymphoma using only intrathecal and systemic chemotherapy combined with rituximab.

BACKGROUND: With current chemotherapy treatment, >90% of survival has been obtained for Burkitt lymphoma (BL). In this study, the demographic characteristics and treatment outcomes are presented for 78 children in China with central nervous system-positive (CNS+) BL. METHODS: This retrospective study consecutively enrolled 78 CNS+ BL patients in Beijing Children's Hospital (BCH) from 2007 to 2019 who received the BCH B-cell non-Hodgkin's lymphoma regimen (modified by French-American-British mature lymphoma B-cell 96 [FAB/LMB96] C1 arm ± rituximab). Clinical characteristics, methods of disease detection in the CNS, and outcomes were evaluated. Univariate and multivariate analyses were used to assess prognostic factors. RESULTS: The median age of 65 boys and 13 girls at the time of diagnosis was 5.7 years (ranging from 1 to 14 years). Patients were followed up for a median time of 34 months (ranging from 1 to 72 months). Bone marrow invasion was found in 38 (48.7%) patients. There were 48 (61.5%), 44 (56.4%), and 25 (32%) patients with cranial nerve palsy, intracerebral mass (ICM), and para-meningeal extension, respectively. Abnormal cerebrospinal fluid (CSF) morphology and CSF immunophenotype appeared in 15 (19.2%) and 15 (19.2%) patients, respectively. There were 69 (88.5%) patients treated with chemotherapy combined with rituximab, and nine patients were treated solely with chemotherapy. Finally, five patients died of treatment-related infection, recurrence occurred for 13, and one developed a second tumor. The 3-year overall survival and event-free survival rates were 78.9% ±â€Š4.7% and 71.4% ±â€Š6.0%, respectively. Treatment with chemotherapy only, ICM positivity, and >4 organs involved at diagnosis were independent risk factors. CONCLUSIONS: Rituximab combined with a modified LMB96 regimen has greatly increased the efficacy of treatment for Chinese children with CNS+ BL, and with the continuous collection of outcome data, treatment-related complications are decreasing. For further verification, a large sample multicentre randomized controlled study should be performed to explore a treatment scheme for Chinese children with even greater efficacy.

Central nervous system relapse in a pediatric anaplastic large cell lymphoma patient with CLTC/ALK translocation treated with alectinib: A case report.

BACKGROUND: The aberrant expression of the anaplastic lymphoma kinase (ALK) gene in ALK-positive (ALK+) anaplastic large cell lymphoma (ALCL) is usually due to t(2;5)/NPM-ALK. However, rarely, aberrant ALK expression can also result from a rearrangement of the ALK gene with various partner genes. Central nervous system (CNS) metastasis is very rare in ALK+ALCL. Patients with CNS involvement show an inferior prognosis. CASE SUMMARY: Here, we present the case of an 8-year-old girl diagnosed with ALK+ALCL. She presented with fever, skin nodules, leg swelling, and abdominal pain over the preceding 6 mo. She had extensive involvement and showed an extraordinary rare translocation, t(2;17)/CLTC-ALK, as demonstrated by RNA-seq. She underwent chemotherapy as per ALCL99, followed by vinblastine (VBL) maintenance treatment, and achieved complete remission. However, she developed CNS relapse during VBL monotherapy. The patient achieved a durable second remission with high-dose chemotherapy (including methotrexate 8 g/m2) and continuous treatment with alectinib and VBL. CONCLUSION: Alectinib showed significant and durable CNS effects in this patient. However, more cases are needed to prove the efficacy and safety of alectinib for pediatric ALK+ALCL patients.

[Prognostic significance of the NPM-ALK fusion gene in bone marrow and peripheral blood for patients with anaplastic large cell lymphoma].

OBJECTIVE: To investigate the expression of NPM- ALK fusion gene in bone marrow (BM) and peripheral blood (PB) in anaplastic large cell lymphoma (ALCL) patients and its prognostic significance. METHODS: NPM- ALK fusion gene of 21 BM and 15 PB samples from patients with NPM-ALK positive ALCL was detected by RT- PCR, and the relationship between NPM- ALK expression and prognosis and clinical characters was evaluated. RESULTS: Of the 21 patients, 12 cases were male and 9 case were female with a median age of 9 (range, 2-14) years old. The median follow- up was 31 months. Patients with a positive NPM-ALK expression in BM had a 3-years EFS of (35.6±18.6)%, compared with (91.7±8.0)% for patients with negative NPM-ALK (P=0.038). The incidence of positive expression in BM was significantly higher in patients who had more than 3 organs involved by tumor (P=0.032). 86.7% patients had a concordant results of NPM-ALK expression in PB and BM. CONCLUSION: We could evaluate the minimal disseminated disease of NPM-ALK positive ALCL patients by screening the NPM-ALK fusion gene in BM and PB by RT-PCR. The positive expression is associated with a poor prognosis and could be used for stratification of ALCL.

[Clinical features and prognosis of children with lymphoblastic lymphoma].

OBJECTIVE: To evaluate the clinical characteristics of childhood lymphoblastic lymphoma (LBL) and therapeutic efficacy of BCH-LBL-2003 regimen modified from BFM-90 protocol. The drug-related toxicities and prognostic factors were explored at the same time. METHODS: From Janurary 2003 to December 2009, 112 newly diagnosed LBL patients at the Hematology Center of Beijing Children's Hospital were enrolled in this study. The patients were treated with modified BFM-90-LBL protocol. RESULTS: At a median follow-up of 29 months (1 to 90 months), the patients were evaluated on day 33 and at the end of induction therapy. The bone marrow complete remission (CR) rates were 96.4% and 100%, respectively. Meanwhile, the complete remission (CR) rates of tumor were 77.7% and 94.5%, and the partial remission (PR) rates were 22.3% and 5.5%, respectively. The overall response rate was 100%. The 3-year overall survival (OS) rate was 89.1% and 5-year OS was 87.0%. The 3-year event-free survival (EFS) was 85.4% and 5-year EFS was 83.3%. Eleven cases relapsed during the treatment (4 BM relapses, 3 CNS recurrences, 3 primary site and 1 lymph node of neck and BM). Eleven patients died (3 died of infection and 8 died of progressive disease after relapse). All patients experienced grade 3-4 hematological toxicity. Univariate analysis indicated that lack of CR at the end of induction therapy, immunophenotype, bulky tumor and course of disease had prognostic significance. CONCLUSIONS: Lymphoblastic lymphoma in childhood and adolescence is highly aggressive. BCH-LBL-2003 protocol is very effective. The treatment-associated side effects were tolerable. Patients who didn't get CR at the end of induction therapy, with T-cell immunophenotype, with bulky disease and the course of disease less than 30 days may have a poor prognosis.

[Clinical features and therapeutic effect of 38 children with anaplastic large cell lymphoma].

OBJECTIVE: To analyze the clinical features and prognostic factors of children's anaplastic large cell lymphoma (ALCL), summarize the therapeutic effect and toxicities. METHOD: A total of 38 ALCL patients admitted to Beijing Children's Hospital from Jan. 2003 to Apr. 2010 were treated with BCH-ALCL-2003 regimen (modified from HK-ALCL-2000). RESULT: Thirty-four cases were ALK(+), male:female ratio = 2.16:1. The median age was 9 years; 86.8% had B symptoms. 94.7% evolved to Stage III and IV on admission. The median follow-up duration was 48 months (12 to 99 months). Median event-free survival (EFS) time was 43 months. Thirty-four patients (89.5%) achieved a remission. The disease relapsed in 3 patients within 20 months after diagnosis. Estimated 4-year EFS was (81.2 ± 6.4)%, estimated 4-year overall survival (OS) rate was (86.4 ± 5.7)%. Univariate analysis indicated that the unfavorable prognostic factors included: more than 3 extra nodal involvement, hepatosplenomegaly (> 3 cm), elevated lactate dehydrogenase (LDH), stage IV, hemophagocytosis in bone marrow, and age < 3 years. The major toxicity was myelosuppression and mucositis. no chemotherapy related death occurred. CONCLUSION: (1) Childhood ALCL patients often have B symptoms and extranodal involvement. (2) In the study, therapeutic effects was good. The disease relapsed mostly within the first 2 years, maintenance therapy with vinblastine is necessary. (3) The regimen is safe to patients.

[Clinical analysis of 18 cases with acute tumor lysis syndrome in children with B-cell lymphoma].

OBJECTIVE: To investigate risk factors associated with acute tumor lysis syndrome (ATLS) in children with B-cell lymphoma and to explore feasible means for the prophylaxis and treatment. METHOD: Data from 18 children with ATLS in B-cell lymphoma were collected to assess their tumor burden at diagnosis and before chemotherapy. Evaluation was performed at the 8th day, 3 month, and the end of chemotherapy and follow up. The incidence of ATLS in B-cell lymphoma, and the relationship between the incidence of ATLS and whether the kidney was involved and large tumor burden were analyzed respectively. All patients received hydration, alkalinization and received allopurinol routinely. Urate oxidase and hemodialysis treatment were administered in some cases. RESULT: Of the 103 children with B-cell lymphoma, 18 were diagnosed as having ATLS (17.5%). All the 18 cases with ATLS were histopathologically confirmed as having Burkitt's lymphoma. All the patients were at stage III or IV and all had large tumor sizes, and 7 were found to have blasts in the bone marrow>25% (38.9%). Lactate dehydrogenase (LDH) levels≥1000 U/L were found in 11 (61.1%) cases. All patients had developed metabolic abnormalities, including hyperuricemia, hyperphosphatemia, hypocalcemia, and uremia. In terms of clinical features and prognosis, all cases had nausea, vomiting, anorexia, oliguria, and anuria at different levels. One had gastrointestinal bleeding, 7 patients experienced seizures. The etiology in five was hypocalcemia and two had reversible posterior encephalopathy syndrome and all responded well to treatment. Nine cases of ATLS responded to supportive care, 4 required hemodialysis, and the other 4 responded to urate oxidase. Ten cases survived and 8 died. The major cause of death was severe complications and treatment was given up in 5 cases and recurrence occurred in 3 cases. CONCLUSION: ATLS was commonly seen in Burkitt's subtype of B-cell lymphoma. Higher LDH and large tumor sizes and kidney involvement were important risk factors for the development of ATLS in children with B-cell lymphoma. Treatments with hydration, alkalinization and allopurinol were safe and effective. Urate oxidase and hemodialytic treatments should be given timely.

[Clinical analysis of children with lymphoma complicated with severe pneumonia due to novel influenza A (H1N1) virus infection].

OBJECTIVE: To study the clinical features and treatment of severe pneumonia due to novel influenza A (H1N1) virus in children with lymphoma during chemotherapy. METHOD: The clinical manifestations, radiologic features, reasons of misdiagnosis, experiences in treatment and prognosis of 4 children with lymphoma complicated with pneumonia due to novel influenza A (H1N1) virus during chemotherapy were analyzed retrospectively. RESULT: Four children out of the 54 patients with hematologic disorders who were receiving chemotherapy suffered from H1N1 influenza. Neutrophil counts were less than 0.5 × 10(9)/L in all 4 patients. The body temperature was higher than 39°C accompanied by chill and low blood pressure at the onset of the illness. Dyspnea and hypoxemia occurred quickly. Two of them developed acute respiratory distress syndrome (ARDS). C-reactive protein (CRP) was higher than 50 mg/L in all these cases, and was higher than 200 mg/L in 2 cases. Chest X-ray showed that there were extensive infiltrations in several lung lobes in all the 4 patients. The first patient was misdiagnosed as sepsis at the beginning. The results of 17 blood cultures for the 4 patients were all negative. Fungi were found in 2 of 20 sputum cultures in 2 patients and these 2 patients had been considered as having fungal pneumonias. All the 4 patients were treated with oseltamivir phosphate. The oseltamivir treatment started on the 5(th) day in patient number 1, whereas on the 1(st) day in the other 3 patients. Intravenous immunoglobulin (IVIG) was used in all 4 patients. Methylprednisolone was used in 3 patients. After treatment, 2 died and 2 were improved. CONCLUSION: The children with lymphoma who undergo chemotherapy are prone to develop severe pneumonia during epidemics of influenza A H1N1. The pneumonia may be aggravated very quickly and have a higher mortality. The patients might be easily misdiagnosed as sepsis at early stage. The pneumonia may be misdiagnosed as fungal infection. During H1N1 prevalent season when high fever occurred, H1N1 infection should be considered. Early detection of the virus and use of oseltamivir phosphate and high-dose IVIG and methylprednisolone might reduce the mortality.

[Clinical characteristics of 34 children with Hodgkin lymphoma and efficacy of treatment with chemotherapy plus low dose radiotherapy on involved sites].

OBJECTIVE: To summarize the clinical features and to evaluate outcomes and to assess therapeutic effects in 34 children and adolescents with Hodgkin lymphoma treated with risk-adapted combination chemotherapy and low-dose, involved-field radiation therapy (IFRT) in China. METHOD: From January 2003 to April 2009, 34 hospitalized children with Hodgkin lymphoma were enrolled into the BCH-HL 2003 protocol (revised CCG 5942) in our hospital. Pathological samples were reviewed centrally and classified based on the World Health Organization guidelines. Staging was based on clinical evaluation and was defined by the Ann Arbor staging system. The 34 patients were treated according to the different risk factors in three treatment groups (standard, intermediate, and high risk), and received risk-adapted combination chemotherapy and IFRT. All analyses were calculated by the statistical program SPSS. RESULT: Of the 34 Hodgkin lymphoma patients, 28 were male and 6 were female. The median age was 8.7 years (range from 4 years to 15 years) at the time of diagnosis. In terms of clinical presentation, 53% had bulky lymph nodes, 47.1% had more than 4 node regions involved and 44% had "B" symptoms at presentation. The distribution for stage of disease was 0% for Stage I, 21% for Stage II, 35% for Stage III and 44% for Stage IV disease. All patients had classical histology consisting of three different sub-discipline: 22 cases of mixed cellularity (64.7%). In pathological samples of 25 cases there was EBV encoded RNA (EBER) or latent membrane protein (LMP) staining. The overall survival (OS) was 100% and the 5-year event-free survival was 94.1% with a median follow-up of (26.1 ± 16.3) months. Two patients had early relapse after treatment was finished. Organ toxicity was limited to hematological grades III and IV at rates of 40% and 71% respectively. CONCLUSION: Childhood Hodgkin lymphoma in our study was more frequently seen in male school aged children. Combined-modality therapy using risk-adapted chemotherapy with radiation is effective and well tolerated. The overall prognosis was good.

[Clinical features of hepatitis-associated aplastic anemia in children].

OBJECTIVE: To study the clinical features of hepatitis-associated aplastic anemia (HAAA) in children. METHODS: The clinical data of the children with newly diagnosed HAAA from January 2007 to December 2008 were respectively studied, including clinical manifestations, and blood routine, bone marrow examination, viral serology and immune function results as well as treatment and prognosis. RESULTS: A total of 8 children were confirmed as HAAA, accounting for 4.9% in children with aplastic anemia. There were 7 males and 1 female. The median age was 7.5 years (range 4.4 to 10.3 years) at diagnosis. They had negative serologic results and the causes of hepatitis could not be identified. The median interval from hepatitis occurrence to blood cell reduction was 6 weeks. Three cases were diagnosed as severe aplastic anemia and 5 cases as very severe aplastic anemia. Severe T cell immune disorders were found in all 8 cases. The percentage of Ts cells increased and the percentage of Th cells decreased significantly in the 8 children with HAAA. Four children survived after immune suppress treatment, three children died within one month after diagnosis and one child required own discharge without treatment. CONCLUSIONS: HAAA is more frequent in male school children. The children with HAAA have severe T cell immune disorders, with a higher early death rate. Immune suppress treatment is effective.

[Tolerability of 6-mercaptopurine in children with acute lymphoblastic leukemia].

OBJECTIVE: 6-Mercaptopurine (6-MP) has been the backbone of maintenance chemotherapy for acute lymphoblastic leukemia (ALL), the response to 6-MP is highly variable, adverse events leading to discontinuation or dose-reduction (children intolerant) of 6-MP occur in many children with ALL. The aim of this study was to investigate the tolerability of 6-MP and to optimize thiopurine use. METHODS: The authors evaluated in a prospective manner the tolerance of 6-MP in ALL children from Oct. 1, 2004 to Sept. 30, 2007 who were newly diagnosed in Beijing Children's Hospital, using BCH-ALL-2003 protocols, during the maintenance therapy and followed up to Sept. 30, 2008. All children had a treatment period of at least 3 months for maintenance therapy. RESULTS: Totally 133 children including 81 boys and 52 girls at median age of 67 months (18 - 188 months), 100% of the patients went into complete remission (CR) on day 33 of induction chemotherapy, and the median time to CR was 26 months (6 - 47 months). All the children had maintenance therapy from 3 to 25 months (mean 13.5 +/- 7.4) and 72(54%) received 6-MP standard doses continuously for total courses, the median daily dose of 6-MP was 46 mg/(m(2).d) 6-MP, their WBC was (3 - 4) x 10(9)/L, ANC (1.5 - 2) x 10(9)/L, they had no severe liver toxicity. In 4 children the dose of 6-MP was increased to 125% because WBC was higher than 6 x 10(9)/L, ANC higher than 3 x 10(9)/L. Sixty one children (46%) had poor tolerability to 6-MP, they experienced adverse events that led to discontinuation (n = 19) or dose reduction (n = 42) of 6-MP, the actual mean dose for the 42 cases was 25 - 30 mg/(m(2).d) and the time to occurrence of toxic effects was 2.5 weeks. Reasons for discontinuation or dose reduction were severe myelotoxicity occurred in 48 children, hepatotoxicity in 12, and skin rash in one. CONCLUSIONS: In this cohort of ALL children, the difference of tolerance to oral 6-MP was obvious, 54% of the children well tolerated 6-MP during the whole course at oral standard dose, and severe granulocytopenia did not occur. However, 46% developed severe granulopenia or hepatotoxicity, the dosage had to be reduced in order to decrease the probability of severe toxicity. It is suggested that standard dose of 6-MP is not always the maximum tolerant dose in some children and inadequate dose may be the cause of therapy failure.

[An analysis of mutations causing Gaucher disease in Chinese population].

OBJECTIVE: To review and investigate the relationship of genotype and phenotype in Chinese patients with Gaucher disease (GD). METHODS: The samples were first screened for known mutations as reported previously in Chinese population. Long chain PCR and nested PCR were employed to amplify the segments of glucocerebrosidase functional gene in patients with unknown mutant alleles. The products of nested-PCR were subjected to DNA sequencing to detect the new mutations. RESULTS: Forty kinds of mutations were detected in this panel of patients. The L444P mutation was the most common one accounting for 33.0% of mutant alleles. It was followed by F213I, N188S, V375L and M416V. CONCLUSION: There are at least 40 mutations in Chinese GD patients. The spectrum of mutation is significantly different from that in Caucasians. 70% of mutant alleles have been characterized. It becomes feasible to make clinical and prenatal diagnoses through gene analysis.

[Clinical study of 40 children with Burkitt's and Burkitt-like lymphoma].

OBJECTIVE: To summarize the histological and clinical characteristics of 40 cases with Burkitt's and Burkitt-like lymphoma in children, to evaluate the effects of treatment with international regimen, and to explore the treatment-related complications and prognostic factors. METHODS: Forty patients with Burkitt's and Burkitt-like lymphoma were registered in Beijing Children Hospital from Feb 2003 to Apr 2006. The diagnosis was confirmed by histology and immunohistochemistry of biopsy, and clinical staging by the examination of imaging, cerebrospinal fluid and bone marrow based on St. Jude system. Intensive, short-term chemotherapy witch was modified from LMB89 protocol was given to the patients. RESULTS: Of the 40 patients, 30 were diagnosed as Burkitt's lymphoma (BL) and 10 as Burkitt-like lymphoma (BLL). Antibody against Epstein-Barr virus (EBV-Ab) was positive in 19 cases at diagnosis, only 7 of the patients were positive for EBER. Thirty-three of the cases were male and 7 female (M:F = 4.7:1); the median age was 6 years 9 months. The most frequently seen clinical characteristics were abdominal masses and surgical abdomen. Nine cases were at stage I - II and 31 cases at stage III - IV at diagnosis; CNS was involved in 4 cases and bone marrow in 2 cases. The courses of treatment were approximately 2 - 8 months. All the patients were followed up, the median follow-up period was 22.6 months. After chemotherapy, 35 patients (88.7%) were still alive during the one-year follow-up. The 3-year event-free survival (EFS) rate was 81.8%. Major toxicity was myelosuppression and mucositis. Stage III to IV of myelosuppression occurred in the most patients with unresected tumor and CNS-involvement. Of 5 patients who died, 2 died of infection, 2 died of lymphoma progression during chemotherapy, and 1 died of relapse. CONCLUSION: Burkitt's and Burkitt-like lymphoma are the most common NHL in children with rapid clinical process. Outcome was greatly improved by current intensive, short-term chemotherapy regimen, the 3-year EFS was 81.8% including the patients who were in advanced stage. Childhood lymphoma with short clinical history, stage IV and residual disease after 3 months of therapy are associated with poor prognosis.