Treatment outcome in children with central nervous system-positive Burkitt lymphoma using only intrathecal and systemic chemotherapy combined with rituximab / 中华医学杂志(英文版)

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% ± 4.7% and 71.4% ± 6.0%, 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.

Prognostic significance of the NPM-ALK fusion gene in bone marrow and peripheral blood for patients with anaplastic large cell lymphoma / 中华血液学杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>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.</p>

Molecular genetic abnormalities of N-myc and C-myc in pediatric neuroblastic tumors and clinical pathologic significance / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the molecular genetic abnormalities of N-myc and C-myc, and their clinical pathological implications in pediatric neuroblastic tumors (NTs).</p><p><b>METHODS</b>Abnormalities of N-myc were detected by interphase fluorescence in situ hybridization (FISH) technique in 246 cases of NTs, including neuroblastoma (NB,188 cases), ganglioneuroblastoma (GNB, 52 cases), ganglioneuroma (GN, 6 cases), and their association with the histological typing of the tumors and prognosis was analyzed. Abnormalities of C-myc were detected by FISH in 133 cases of NTs.</p><p><b>RESULTS</b>Of the 246 cases of NTs, N-myc amplification was only found in 27 cases (11.0%, 27/246) of NB, but not in any cases of GNB or GN (P < 0.05). 89.0% (219/246) N-myc non-amplification were found in NTs, and it included N-myc gain in 175 cases (71.1%, 175/246) and normal N-myc in 44 cases (17.9%, 44/246). Univariate analysis indicated significantly (P = 0.012) poorer outcome in patients with N-myc amplification than N-myc non-amplification. However no significant difference was observed between N-myc gain cases and normal N-myc cases (P = 0.057). C-myc gain was found in 74 of 133 cases (55.6%) of NTs; no C-myc amplification or translocation was detected. Forty percent (6/15) of cases with N-myc amplification and 57.6% (68/118) of cases with N-myc non-amplification were accompanied by C-myc gain. The difference between N-myc amplification and non-amplification with C-myc gain was not significant (P > 0.05). Univariate analysis indicated that the outcome difference was not statistically significant between C-myc gain cases and normal C-myc cases (P = 0.357).</p><p><b>CONCLUSIONS</b>The incidence of N-myc amplification only found in NB is low in pediatric NTs in China. Patients with N-myc amplification predict poorer outcome. No amplification or translocation of C-myc is detected in NTs, whereas C-myc gain is relatively common in NTs. There is no obvious association between N-myc amplification and C-myc gain.</p>

Primitive neuroectodermal tumor/Ewing's sarcoma of the penis in children: a case report and review of the literature / 中华男科学杂志

<p><b>OBJECTIVE</b>To investigate the clinical manifestations, pathological characteristics and treatment of primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) of the penis in children.</p><p><b>METHODS</b>We analyzed the clinical data of a case of PNET/EWS and reviewed relevant literature.</p><p><b>RESULTS</b>The patient was a 5-year-old boy, admitted for penis swelling with pain for 11 months. Biopsy showed a small round cell tumor, CD99 positive by immunohistochemical staining, with EWS translocation by fluorescence in situ hybridization on molecular biological examination. The tumor was confirmed to be PNET/EWS of the penis, and disappeared after 45 weeks of chemotherapy and local radiotherapy.</p><p><b>CONCLUSION</b>PNET/EWS of the penis is an extremely rare disease, with no specific clinical symptoms except penis enlargement with pain. Immunohistochemistry and molecular biological examination contribute to its diagnosis.</p>

N-myc gene copies in children with neuroblastoma and its clinical significance / 中国实验血液学杂志

This study was purposed to analyze the relation of N-myc gene copy number with clinical staging, pathological types and tumor biological factors in children with neuroblastoma (NB), and to investigate the influence of chemotherapy on N-myc gene expression and explore the relationship of N-myc gene copies with prognosis of NB children. The newly diagnosed children with NB from 1 March 2007 to 31 January 2011 were enrolled in this study. The treatment was carried out by BCH-NB-2007 based on Hongkong NB-07 protocol, and the patients were follow up to 31 January 2012. The N-myc gene in NB children was detected by FISH. According to number of N-myc gene copies, the NB children were divided into 3 groups. A group (N-myc gene negative) had less than 2 copies, B group (N-myc gene gains) had 3 to 9 copies, and C group (N-myc amplification) had more than 10 copies. The results showed that the N-myc gene expression in 58 cases of NB was observed. There were 36 males and 22 females. NB children aged from 6.5 to 138 months (median age 47.5 months), all patients were followed up for 11 - 57 months with an average of 31.5 months. INSS stages I-IV were 1, 5, 8 and 44 cases, respectively. Twenty-five cases had primary post mediastinal tumor, thirty-three cases had retroperitoneal and pelvic tumor, three of which also companied with post mediastinal tumor. Thirty-five cases had bone metastasis (60.3%), thirty-two cases had bone marrow metastasis (55%). Of the 54 patients with fully known biologic features, seventeen cases had ganglioneuroblastoma, thirty-seven cases had neuroblastoma (15 displayed differentiated, 7 poorly differentiated or undifferentiated, 15 with pathological changes after chemotherapy), four cases had bone marrow metastasis only detected by bone marrow biopsy. Eleven cases had N-myc gene negative, forty-three had N-myc gains, four had N-myc amplification. The average copy number of N-myc gene copies in 58 cases was 5.96 ± 7.81 in which 28 children were non chemotherapy cases, their average copy number was 4.00 ± 1.88, thirty cases out of 58 cases received preoperation chemotherapy (chemotherapy group), and their average copy number was 7.80 ± 10.46, the difference is significant (P = 0.064). The clinic stage, the location of primary tumor, pathological classification, urine VMA and serum neurogenic specific enolase had no effects on the N-myc gene expression, but the serum LDH level had influence (P < 0.01). Single factor Kaplan-Meier analysis showed that the number of N-myc gene copies in NB patients were closely related with the poor prognosis. The more copies of N-myc gene, the more poor prognosis, the difference is statistically significant (P < 0.05). It is concluded that the number of N-myc gene copies correlates with the rapid growth of NB and its poor prognosis, detecting the N-myc amplification can help to estimate the prognosis and decide the program of treatment. Serum LDH, which correlated with the rapid growth of NB, had effect on the N-myc gene expression and is closely related with the poor prognosis of NB.

Clinical features and therapeutic effect of 38 children with anaplastic large cell lymphoma / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze the clinical features and prognostic factors of children's anaplastic large cell lymphoma (ALCL), summarize the therapeutic effect and toxicities.</p><p><b>METHOD</b>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).</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>(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.</p>

Clinicopathological analysis of cutaneous natural killer/T cell lymphoma: 36 case report / 中华皮肤科杂志

ObjectiveTo investigate the clinicopathological features and prognosis of natural killer (NK)/T cell lymphoma and to analyze its relationship with Epstein-barr virus(EBV). MethodsTotally, 36 cases of cutaneous NK/T cell lymphoma were collected from 2000 to 2010 at the Department of Pathology, Peking University Health Science Center, and classified into primary and secondary groups according to whether there is evidence of extracutaneous involvement within 6 months after diagnosis. Clinicopathological features were analyzed and Epstein-barr virus (EBV) was detected. ResultsOf these 36 cases, 13 (36.1％) were classified as primary cutaneous NK/T cell lymphoma, 20 (55.6％) as secondary, and 3 (8.3％) remained unclassified because of the lack of clinical data. Males were more likely to develop both primary and secondary cutaneous NK/T cell lymphoma than females, but there was no striking difference in sex ratio between the patients with primary and secondary lymphoma (P ＞ 0.05 ). Compared with the patients with primary cutaneous NK/T cell lymphoma, those with secondary cutaneous NK/T cell lymphoma showed a younger median age at onset(43.5 vs. 54 years, P ＜ 0.05), higher prevalence of B symptoms(including fever, night sweat, body weight loss) and multiple skin lesions (P ＜ 0.05 and 0.01, respectively). EBV was positive in 92.3％ (12/13) of the primary lymphoma cases and 85％(17/20) of the secondary lymphoma cases. Moreover, the median survival was 8 months in all the cutaneous NK/T cell lymphoma cases, and was significantly shorter in secondary cases than in the primary cases(6 vs. 18 months, x2 = 6.074, P ＜ 0.05). ConclusionsCutaneous NK/T cell lymphoma is an EBV-associated, clinica]ly aggressive disease entity. Patients with primary cutaneous NI/T cell lymphoma seem to have an older age at onset and a better prognosis as compared with those with secondary cutaneous NK/T cell lymphoma.

Clinical features of 3 cases with acute interstitial pneumonia in children / 中华儿科杂志

<p><b>OBJECTIVE</b>Acute interstitial pneumonia (AIP) is a rare lung interstitial disease in children. This study was conducted to understand the clinical features of the AIP in children.</p><p><b>METHOD</b>The data of the three cases with AIP admitted to our hospital from March 2008 to November 2009 were reviewed. Of the 3 cases, 2 were male, one was female. Their age ranged from 1 year and 4 months to 10 years. The clinical manifestation, pulmonary function test and the high resolution computed tomography (HRCT) and pathology of the lung were studied retrospectively. Tissue specimens of the lung were obtained by video-assisted thoracoscopic biopsy. Viral etiologic examinations for the respiratory syncytial virus, adenovirus, influenza virus, parainfluenza viruses, EB virus, cytomegalovirus, enterovirus and herpes simplex virus were performed. The IgM antibody to Mycoplasma pneumoniae in the serum was also detected.</p><p><b>RESULT</b>All the 3 cases rapidly developed respiratory failure of unknown origin, none of these cases had failure of any other organs. All three cases had cough and dyspnea. No case had the rales and digital clubbing. The examinations for viruses, bacteria and Mycoplasma pneumoniae infection were all negative. No evidence for the diagnosis of connective tissue disease was obtained. The HRCT of the chest showed diffuse alveolar consolidation, air bronchogram and ground glass appearance in the bilateral lungs, and the traction-associated bronchiectasis in areas. All the three cases had the histological proof of diffuse alveolar damage by the biopsy. All the three cases were treated with CPAP and corticosteroid. Two cases were treated with corticosteroid in early stage of the disease, the condition of these cases were improved obviously. The third case was treated with high-dose steroid pulse therapy days, the condition of this case was improved slightly in a month. One year follow-up showed that case 1 and case 2 had no hypoxemia and the HRCT of the chest showed obvious improvement. The pulmonary function of case 2 had restrictive deficiency.</p><p><b>CONCLUSION</b>AIP has a rapidly progressive clinical course leading to respiratory failure. The HRCT of the chest showed alveolar consolidation and ground glass-like change. The pathology of the lung includes diffuse alveolar damage. The prognosis of the AIP in children may be improved by the treatment with respiratory assistance and corticosteroids.</p>

Clinical characteristics of 5 children with Castleman's disease and review of literature / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical characteristics of children with Castleman's disease and to improve doctors' awareness of this disease.</p><p><b>METHODS</b>Clinical characteristics of 5 cases with Castleman's disease were observed and analyzed and relevant reports in literature were reviewed.</p><p><b>RESULTS</b>(1) All the five patients' histories were long, and the first symptoms of them were painless lymphnode enlargement, and all of them were at school age; 3 patients' abdominal lymphnodes were enlarged, mediastinum lymphnodes enlarged in 3 cases, cervical lymphnodes were involved in 3 cases; (2) The clinical subtypes: the disease in 3 cases was localized Castleman's disease (LCD), all of their pathological subtype was hyaline vascular variant (HV). The rest of them were multicentric Castleman's disease (MCD), whose pathology was plasma cell variant (PC), and both of them had a febrile symptoms; (3) The white blood cells, C-reactive protein and ferritin levels were all elevated to different extents. Four of them had viral infections, and their cellular immune function was abnormal; (4) The LCD patients' prognosis was good after the complete resection. There is no standard therapy for MCD, the available therapies include antiviral, immune modulatory regimens, CD20 B cell monoclonal antibody and chemotherapy, but the prognosis was worse than that of LCD.</p><p><b>CONCLUSIONS</b>Castleman's disease is rare in children, which can be misdiagnosed because it has no specific manifestations. The prognosis depends on the subtype.</p>

Clinical characteristics of 34 children with Hodgkin lymphoma and efficacy of treatment with chemotherapy plus low dose radiotherapy on involved sites / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>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.</p>

Idiopathic interstitial pneumonias in 7 children / 中华儿科杂志

<p><b>OBJECTIVE</b>Idiopathic interstitial pneumonias (IIPs) have been increasing in children in recent years. The type and prognosis of IIPs in children in China has not been clear. The purpose of this study was to investigate the type and prognosis of IIPs in children.</p><p><b>METHOD</b>Seven children diagnosed with IIPs from December 2003 to March 2007 were retrospectively studied. The clinical, radiologic, and pathologic features, type and prognosis of IIPs, were reviewed.</p><p><b>RESULT</b>Of the seven patients with IIPs, three were diagnosed with cryptogenic organizing pneumonia (COP)/bronchiolitis obliterans organizing pneumonia (BOOP), one was diagnosed with usual interstitial pneumonia (UIP) and the remaining three were diagnosed with nonspecific interstitial pneumonia (NSIP). The chief complaints of these patients were chronic cough. Six patients had shortness of breath. Clubbed fingers were found in one patient with UIP and two patients with NSIP. The characteristic computed tomographic findings of COP/BOOP were multiple patchy peripheral consolidation, associated with centrilobular nodules and bronchiole wall thickening in 2 cases and bronchiolectasis in 1 case. In NSIP, diffuse or basal ground-glass opacities predominated over reticular opacities with traction bronchiectasis only in one case. UIP was manifested by patchy infiltrate with predominant honeycombing, traction bronchiectasis and interlobular septal thickening. Two patients with COP/BOOP had plugs of connective tissue within a bronchiole and the adjacent alveolar ducts and alveolar spaces. Lung biopsies showed predominantly interstitial chronic inflammation with infiltrate of lymphocytes and some plasma cells and alveolar septum thickening in three patients with NSIP. One case with NSIP showed a mixed fibrosing and prominent interstitial chronic inflammation. The key histological findings of the UIP was a heterogeneous appearance, with alternating areas of relatively normal lung, interstitial inflammation and fibrosis. All patients were treated with prednisone. The abnormal findings of chest radiography resolved completely after four to twelve months' therapy in three patients with COP/BOOP. The combination of prednisone and MTX or 6-MP was given to the two patients with NSIP. The symptoms and chest radiographic abnormality disappeared except the presence of a few cyst after treatment. One case with NSIP and the case with UIP had not been improved remarkably.</p><p><b>CONCLUSION</b>COP/BOOP, NSIP and UIP might occur in children in China. The typing of IIPs was based on clinical-radiologic-pathologic features. Steroid was the first choice for IIPs. The prognosis of IIPs varied among different types.</p>

Congenital intrapulmonary lymphangioma and pulmonary hemangioma in 5 infants and young children / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children.</p><p><b>METHOD</b>Data of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed.</p><p><b>RESULT</b>All the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath. Imaging study indicated cystic and space occupying changes of partial lung. In the two cases of hemangioma, the blood vessels passing through the hemangioma were observed on CT films. Histochemical studies showed that the cystic pockets from removed tissues were different in size and there was a line of flat endothelial tissue around these pockets. Immunochemical studies indicated D2-40 positive, factor VIII weak positive for lymphangioma cases; while in hemangioma cases, factor VIII was positive, D2-40 was negative or weakly positive, and both of cytokeratin was negative.</p><p><b>CONCLUSION</b>It is very important to consider and identify congenital intrapulmonary lymphangioma or hemangioma when a patient has cystic pockets and space occupying change in their lung by imaging and pathological studies.</p>

Detection of C-terminal 30 bp-deletion mutation of latent membrane protein 1 of EBV in childhood lymphoma / 中华实验和临床病毒学杂志

<p><b>OBJECTIVE</b>To study C-terminal 30 bp-deletion mutation of latent membrane protein 1 of the virus from childhood lymphoma.</p><p><b>METHODS</b>Nested-PCR was used to amplify C-terminal of EBV-LMP1 from childhood lymphoma and non-lymphoma associated to EBV, including Hodgkin lymphoma (HL), non-Hodgkin lymphoma (NHL)and reactive hyperplasia of lymph node (RL). Sequence analysis was performed on the positive PCR product.</p><p><b>RESULTS</b>LMP1 with 30 bp deletion in C-terminal was detected in 11/25 HL,3/8 NHL and 5/15 RL cases respectively. There were no significant differences among HL, NHL and RL (P = 0.793). Sequence analysis showed that LMP1 detected in this study belongs to the following three subgroups: B95.8, China1 and China2.</p><p><b>CONCLUSION</b>LMP1 with 30 bp deletion in C-terminal widely existed in childhood HL,NHL and RL. There was no correlation between special types of LMP1 and the diseases. There were 3 LMP1 subgroups of EBV in children's lymphoma in the cases studied, including B95.8, China 1 and China 2.</p>

Clinical study of 40 children with Burkitt's and Burkitt-like lymphoma / 中华儿科杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>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.</p>

Correlation of Epstein-Barr virus infection and childhood lymphoma / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To investigate the correlation of Epstein-Barr virus (EBV) infection and childhood lymphoma.</p><p><b>METHODS</b>Paraffin-embedded specimens of lymphoma collected between 1996 and 2005, including 36 Hodgkin lymphomas (HL) and 51 non-Hodgkin lymphomas (NHL), were included in this study. Paraffin-embedded specimens of reactive hyperplasia of lymph nodes (RL) collected during the same period were used as controls. Immunohistochemical (IHC) assay was used to detect EBV-LMP1 and in situ hybridization (ISH) to detect EBV-EBERs.</p><p><b>RESULTS</b>EBV was detected in 72.2% (26/36) of the Hodgkin lymphomas, 15.7% (8/51) of the non-Hodgkin lymphomas and 33.3% (15/45) of the reactive hyperplasia of lymph nodes. There was a significant difference among Hodgkin lymphoma, non-Hodgkin lymphoma and RL (P = 0. 000).</p><p><b>CONCLUSION</b>Childhood Hodgkin lymphoma is closely correlated with Epstein-Barr virus infection. However, the low rate of EBV infection detected in childhood non-Hodgkin lymphoma might be due to heterogeneous distribution of pathological types in this study.</p>

Diffuse panbronchiolitis in a child: case report and literature review / 中华儿科杂志

<p><b>OBJECTIVE</b>Diffuse panbronchiolitis (DPB) is a chronic progressive disease of the lower respiratory tract, which is prevalent in Asian population. So far, many DPB cases have been found in adults in China. To our knowledge, no pediatric DPB case has ever been reported in China. We describe the first pediatric DPB case in Chinese literature and the second case in the English-language literature.</p><p><b>METHOD</b>The clinical manifestations, characteristic imaging and histological features of this DPB case were summarized.</p><p><b>RESULTS</b>The patient was a 13-year old girl complained of chronic productive cough with wheezing. Chest auscultation revealed fine moist rales and wheezing over both lung fields. The chest X-ray showed small nodules and reticular opacities in left lower lobe. High-resolution thorax computerized tomography (HRCT) demonstrated bilateral diffuse small centrilobuler nodules and bronchial wall thickening or bronchiectasis in some parts of the lungs. Histopathological examination of transbronchial biopsy specimen revealed lymphocytes and foamy histocytes infiltrated in the walls of bronchi, respiratory bronchioles and adjacent alveoli. Lymphoid follicles were present around some bronchi. Sinus radiographs revealed sinusitis. Lung function studies showed obstruction and restriction. PaO2 was 65 mm Hg. The diagnosis of DPB was made according to the current diagnostic criteria. Low-dose erythromycin [5 - 10 mg/(kg.d)] was effective.</p><p><b>CONCLUSION</b>DPB could occur in children in China. The major diagnostic clues may include the following: (1) persistent cough, sputum, and dyspnea; (2) coexistent chronic sinusitis; (3) bilateral diffuse small nodular opacities on HRCT. Low-dose erythromycin was effective in treatment of the case with DPB.</p>

Relationship between hydroa vacciniforme-like cutaneous lymphoma and chronic active Epstein-Barr virus infection / 中华皮肤科杂志

Objective To report 6 cases of hydroa vacciniforme-like cutaneous lymphoma,and to inves- tigate the relationship between this disorder and Epstein-Barr virus(EBV)infection.Methods Pathological and immunohistochemical examinations were performed in the biopsy specimens obtained from all 6 patients. Skin lesions were subjected to EBV encoded RNA(EBER)detection by in situ hybridization.Serological assay and quantification of EBV DNA were performed.Results All the 6 patients had recurrent papules, papulovesicles,necrosis and variola-like scar with chronic intermittent fever;four of the patients also presented with edema of the face,hands and feet.Pathologically,there were multilocular vesicles in the epidermis,and large numbers of infiltrating lymphocytes through the dermis.The cells were atypical with mitotic figures. Immunohistochemical staining of the lesions of 4 patients showed large quantities of cells expressing CD56, scattered cells expressing CD3 and CD45RO,and cells expressing grazyme B and T cell intracellular antigen-1 (TIA-1);a diagnosis of hydroa vacciniforme-like cutaneous NK/T lymphoma was made in these 4 cases. In the lesions of another 2 patients,the cells expressing CD3 and CD45RO,but not CD56,were observed; the diagnosis of hydroa vacciniforme-like cutaneous T-cell lymphoma was made in them.EBER was detected in the tumor cells of all the 6 patients.The IgG titers of anti-Epstein-Barr viral capsid antigen increased in all patients(1:5120 in 2 cases,1:2560 in 2 cases,1:1280 in 2 cases).The copies of EBV DNA were increased in the peripheral blood of both the two detected cases.A chronic active EBV infection was confirmed in all patients.Conclusions Hydroa vacciniforme-like cutaneous lymphoma is clinically characterized by edema of face,hands and feet,vesicular eruptions and variola like scars;histologically,it is characterized by infiltrates of atypical cells consistent with lymphoma,and necrosis in the center of vessels.NK/T is the primary immunophenotype of this disease.There is a close association between chronic active EBV infection and hydroa vacciniforme-like cutaneous lymphoma.