Burkitt lymphoma is a highly aggressive B-cell neoplasm. New therapeutic methods are needed to overcome the adverse effect of intensive chemotherapy regimens. Valproic acid and (-)-gossypol are two kinds of chemical...Burkitt lymphoma is a highly aggressive B-cell neoplasm. New therapeutic methods are needed to overcome the adverse effect of intensive chemotherapy regimens. Valproic acid and (-)-gossypol are two kinds of chemical compounds used as new anti-tumor drugs in recent years.展开更多
OBJECTIVE To summarize the clinical characteristics and treatment effects of Burkitt lymphoma (BL) and Burkitt-like lymphoma (BLL), and to explore the best possible optimal regimens and the treatment-related compl...OBJECTIVE To summarize the clinical characteristics and treatment effects of Burkitt lymphoma (BL) and Burkitt-like lymphoma (BLL), and to explore the best possible optimal regimens and the treatment-related complications.METHODS Clinical data of 13 BL and BLL patients, who were pathologically diagnosed and treated in the Beijing Cancer Hospital from August 1996 to October 2008, were retrospectively analyzed, All patients received the therapeutic regimen with chemotherapy as the first-line treatment. The treatment effect and adverse reactions were evaluated.RESULTS Of the 13 patients, 12 were men and 1 was woman, with a median age of 15 years (range, 11-62). Three of the patients were in stage Ⅰ, 2 in stage Ⅱ, 2 in stage Ⅲ, and 6 in stage Ⅳ. Advanced cases were 8 (stage Ⅲ and Ⅳ), accounting for 61.5% of the patients. Bone marrow involvement was found in 2 cases (15.4%) and central nervous system invasion in 4 (30.8%) at the beginning of the treatment. The common involved sites included the superficial lymph nodes (61.5%), abdominal organs (53.8%), and abdominal and retro-peritoneal lymph nodes (38.5%). B symptoms were observed in 7 patients (53.8%). Serum lactate dehydrogenase (LDH) level was increased in 8 of the 10 patients who underwent the serum LDH determination, while serum uric acid level was increased in 1 of the 10. Pathological diagnosis showed that 11 of the cases were BL and 2 BLL. Of the 13 patients, 11 (84.6%) achieved a complete remission (CR) or CR/unconfirmed (CRu), and 1 (7.7%) a partial remission (PR), with a total responsive rate of 92.3%. At a median follow-up of 8 months (range 5-35), 6 patients were found dead at the end of the follow-up. One of the 13 patients was lost to the follow-up. The 1-year overall survival, progression-free survival and disease-free survival rates were 56.98%, 32.31% and 39.77%, respectively. The grade-Ⅲ or Ⅳ myelosuppression was found in 9 patients during the chemotherapy (69.2%), and tumor lysis syndrome and grade- Ⅳ total gastrointestinal mucositis were seen in 1.CONCLUSION Intensive short-course chemotherapy is recommended as the optimal first-line treatment for BL and BLL, and actively preventive treatments for chemotherapy-related adverse reactions are essential in the treatment course.展开更多
Objective:To characterize modified R-CODOX-M/IVAC-based chemotherapy to lower the severe adverse events in Chinese adult patients with sporadic Burkitt lymphoma.Methods:We enrolled a retrospective cohort including 123...Objective:To characterize modified R-CODOX-M/IVAC-based chemotherapy to lower the severe adverse events in Chinese adult patients with sporadic Burkitt lymphoma.Methods:We enrolled a retrospective cohort including 123 adult patients with untreated sporadic Burkitt lymphoma from August 2008 to September 2019 at Sun Yat-sen University Cancer Center.We studied a dose-modified and long-course R-CODOX-M/IVAC regimen utilizing a low dose of 1.0 g/m2/cycle cyclophosphamide,2 g/m2/cycle methotrexate,4,500 mg/m2/cycle ifosfamide,and 4.0 g/m2/cycle cytarabine.Forty-nine patients with low risk disease underwent 4–6 cycles of dose-modified R-CODOX-M-based chemotherapy.Seventy-four patients with high risk disease underwent 6–8 cycles of dose-modified alternating R-CODOX-M/IVAC regimens.Results:The objective remission was 87.0%.The event-free survival rate and overall survival at 3 years were 81.2%and 92.1%,respectively.Major grade 3–4 adverse events included leukopenia(91.9%),anemia(58.5%),thrombocytopenia(73.2%),and febrile neutropenia(48.8%).A total of 26.0%and 37.4%of patients received red blood cell and platelet transfusions,respectively.We observed 4 cases(3.3%)of septic shock after chemotherapy.Two treatment-related deaths occurred from severe infection.Conclusions:The modified R-CODOX-M/IVAC chemotherapy regimen was effective for sporadic Burkitt lymphoma in the Chinese population,with a lower toxicity than standard regimens.展开更多
BACKGROUND The incidence of gastric Burkitt lymphoma(BL),presenting as paraplegia and acute pancreatitis,is extremely low.BL is a great masquerader that presents in varied forms and in atypical locations,and it is pro...BACKGROUND The incidence of gastric Burkitt lymphoma(BL),presenting as paraplegia and acute pancreatitis,is extremely low.BL is a great masquerader that presents in varied forms and in atypical locations,and it is prone to misdiagnosis and missed diagnosis.The prognosis of BL remains poor because of the difficulty in early diagnosis and the limited advances in chemotherapy.CASE SUMMARY A 53-year-old man was referred to our hospital from the local county hospital due to abdominal pain for two weeks and weakness in the lower extremities for one day.Magnetic resonance imaging of the abdomen and lumbar spine showed a swollen pancreas and gallbladder,with peripancreatic exudation and liquid collection,indicating acute pancreatitis and acute cholecystitis.Additionally,we observed abnormally thickened lesions of the gastric wall,multiple enlarged retroperitoneal lymph nodes and a well-demarcated,posterolateral extradural mass lesion between T9 and T12,with extension through the spinal foramen and definite bony destruction,suggesting metastasis in gastric malignancy.Subsequent whole-body positron emission tomography/computed tomography examination showed multifocal malignant lesions in the stomach,pancreas,gallbladder,bone,bilateral supraclavicular fossa,anterior mediastinum,bilateral axillary and retroperitoneal lymph nodes.Gastroduodenal endoscopy revealed primary BL with massive involvement of the gastric body and duodenum.The patient refused chemotherapeutic treatment and died one week later due to upper gastrointestinal hemorrhage.Afterward,we reviewed the characteristics of 11 patients with BL involving the stomach,pancreas or spinal cord.CONCLUSION Clinicians should be aware that BL can be the potential cause of acute pancreatitis or a rapidly progressive spinal tumor with accompanying paraplegia.For gastric BL,gastroscopy biopsies and pathology are necessary for a definite diagnosis.展开更多
BACKGROUND Multiple primary cancer refers to more than one synchronous or sequential cancer in the same individual.Multiple primary cancer always presents as solid cancer or acute myeloid leukemia(AML)secondary to lym...BACKGROUND Multiple primary cancer refers to more than one synchronous or sequential cancer in the same individual.Multiple primary cancer always presents as solid cancer or acute myeloid leukemia(AML)secondary to lymphoma.Here,we report a rare case of secondary peripheral T-cell lymphoma and AML after Burkitt lymphoma treatment.CASE SUMMARY A 54-year-old female patient was admitted to our hospital complaining of edema on her left lower limb.Physical examination revealed multiple superficial lymphadenectasis on her neck and pelvis.Color ultrasonography examination showed multiple uterine fibroids and a solid mass at the lower left side of the abdomen.Pathological biopsy revealed Burkitt lymphoma.After three hyper-CVAD(A+B)regimens,she achieved complete remission.Two years later,lymphadenectasis reoccurred.A relevant biopsy confirmed the diagnosis of peripheral T-cell lymphoma,which was accompanied by gastrointestinal invasion and hemocytopenia.Meanwhile,bone marrow examination revealed AML.On the second day of scheduled treatment,she developed gastrointestinal bleeding,peptic ulcers,and hemorrhagic shock and was critically ill.She was then discharged from the hospital due to financial concerns.CONCLUSION This is the first report of secondary peripheral T-cell lymphoma and AML after Burkitt lymphoma treatment with heterochronous and synchronal multiple primary cancers.展开更多
<strong>Introduction:</strong> Non-Hodgkin’s malignant lymphoma is found primarily in African children. Prostate localization is a rare entity. We report the case of a secondary prostatic localization of ...<strong>Introduction:</strong> Non-Hodgkin’s malignant lymphoma is found primarily in African children. Prostate localization is a rare entity. We report the case of a secondary prostatic localization of Burkitt lymphoma in a 14-year-old child. <strong>Observation:</strong> the 14-year-old child NNJ was followed at the Libreville Cancer Institute (ICL) for Burkitt maxillo-facial stage II bilateral Murphy lymphoma. The clinical examination noted an alteration of the general state, total hematuria, a voluminous prostatic mass filling the rectal ampoule. The paraclinical assessment noted renal insufficiency, a prostatic mass on ultrasound. Anatomo-pathological examination of the prostatic biopsy diagnosed Burkitt type non-Hodgkin’s malignant lymphoma. He died a week later. <strong>Conclusion:</strong> Primary or secondary prostatic lymphoma is rare. Obstructive renal insufficiency is an additional complication that darkens its prognosis by delaying etiological treatment based on polychemotherapy.展开更多
BACKGROUND Burkitt lymphoma(BL)is an exceptionally aggressive malignant neoplasm that arises from either the germinal center or post-germinal center B cells.Patients with BL often present with rapid tumor growth and r...BACKGROUND Burkitt lymphoma(BL)is an exceptionally aggressive malignant neoplasm that arises from either the germinal center or post-germinal center B cells.Patients with BL often present with rapid tumor growth and require high-intensity multidrug therapy combined with adequate intrathecal chemotherapy prophylaxis,however,a standard treatment program for BL has not yet been established.It is important to identify biomarkers for predicting the prognosis of BLs and discriminating patients who might benefit from the therapy.Microarray data and sequencing information from public databases could offer opportunities for the discovery of new diagnostic or therapeutic targets.AIM To identify hub genes and perform gene ontology(GO)and survival analysis in BL.METHODS Gene expression profiles and clinical traits of BL patients were collected from the Gene Expression Omnibus database.Weighted gene co-expression network analysis(WGCNA)was applied to construct gene co-expression modules,and the cytoHubba tool was used to find the hub genes.Then,the hub genes were analyzed using GO and Kyoto Encyclopedia of Genes and Genomes analysis.Additionally,a Protein-Protein Interaction network and a Genetic Interaction network were constructed.Prognostic candidate genes were identified through overall survival analysis.Finally,a nomogram was established to assess the predictive value of hub genes,and drug-gene interactions were also constructed.RESULTS In this study,we obtained 8 modules through WGCNA analysis,and there was a significant correlation between the yellow module and age.Then we identified 10 hub genes(SRC,TLR4,CD40,STAT3,SELL,CXCL10,IL2RA,IL10RA,CCR7 and FCGR2B)by cytoHubba tool.Within these hubs,two genes were found to be associated with OS(CXCL10,P=0.029 and IL2RA,P=0.0066)by survival analysis.Additionally,we combined these two hub genes and age to build a nomogram.Moreover,the drugs related to IL2RA and CXCL10 might have a potential therapeutic role in relapsed and refractory BL.CONCLUSION From WGCNA and survival analysis,we identified CXCL10 and IL2RA that might be prognostic markers for BL.展开更多
Adult intussusception is rare, highly associated with a malignant lead point, and often requires emergent surgical management. We report the case of a 44-year-old male who presented with generalized abdominal pain and...Adult intussusception is rare, highly associated with a malignant lead point, and often requires emergent surgical management. We report the case of a 44-year-old male who presented with generalized abdominal pain and was found to have early ileocolic intussusception secondary to a large ileocecal mass. Biopsies of the mass and an enlarged cardiophrenic lymph node, as well as pleural fluid cytology were all consistent with Burkitt lymphoma (BL). Curiously, the patient’s abdominal exam was reassuring, and the intussusception and malignant bowel obstruction resolved over 36 hours with conservative management alone. With a Burkitt lymphoma international prognostic index (BL-IPI) score of 2, the patient proceeded to treatment with combination chemoimmunotherapy and attained a complete response after four cycles. There was no bowel perforation or recurrent intussusception throughout treatment. Thus, this report marks the first reported case of adult BL-associated intussusception to resolve with non-invasive management and establishes a precedent for conservative management in select patients.展开更多
In 1964,a new herpesvirus,Epstein-Barr virus(EBV),was discovered in cultured tumor cells derived from a Burkitt lymphoma(BL)biopsy taken from an African patient.This was a momentous event that reinvigorated research i...In 1964,a new herpesvirus,Epstein-Barr virus(EBV),was discovered in cultured tumor cells derived from a Burkitt lymphoma(BL)biopsy taken from an African patient.This was a momentous event that reinvigorated research into viruses as a possible cause of human cancers.Subsequent studies demonstrated that EBV was a potent growth-transforming agent for primary B cells,and that all cases of BL carried characteristic chromosomal translocations resulting in constitutive activation of the c-MYC oncogene.These results hinted at simple oncogenic mechanisms that would make Burkitt lymphoma paradigmatic for cancers with viral etiology.In reality,the pathogenesis of this tumor is rather complicated with regard to both the contribution of the virus and the involvement of cellular oncogenes.Here,we review the current understanding of the roles of EBV and c-MYC in the pathogenesis of BL and the implications for new therapeutic strategies to treat this lymphoma.展开更多
Background: Tumor lysis syndrome is unknown to many neurosurgical teams. A dreaded complication, especially in the course of hematological malignancies, like it was the case in two of our patients operated for spinal ...Background: Tumor lysis syndrome is unknown to many neurosurgical teams. A dreaded complication, especially in the course of hematological malignancies, like it was the case in two of our patients operated for spinal cord compression by Burkitt’s lymphoma. Objectives: To describe the clinical and biological presentation of this potentially fatal complication, which can be improved by early preventive or curative treatments. Case Reports: Patient 1: A 49-year-old woman was admitted for vertebral and intercostal pain and paraparesis. Myelography revealed epidural medullary compression at the 7th thoracic vertebrae (Th7) level. Patient 2: A 67-year-old woman was admitted for cervico-thoracic pain and paraparesis. MRI showed posterior epidural medullary compression between the 1st thoracic vertebrae (Th1) and 5th thoracic vertebrae (Th5). In both cases, an urgent decompressive laminectomy was performed. They had also received postoperative corticosteroid therapy. The multi-visceral failure occurred on the 1st day (D1) and 3rd day (D3) postoperatively, respectively. In the absence of adequate management, the outcome was fatal for the two patients respectively at D3 and D5 postoperatively. This syndrome was retained in the presence of glucocorticoid (triggering factor) and the histological result in favor of Burkitt lymphoma (target disease). Conclusion: Malignant hemopathies is a common cause of spinal cord compressions. From these two cases, the importance of biological and clinical surveillance emerges, with patients requiring urgent management for radiculo-medullar tumor compression.展开更多
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 ...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 parameningeal 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.展开更多
Background:Non-Hodgkin lymphoma is the fourth most common malignant tumors in children,Burkitt lymphoma (BL) accounts for 30-50% of all pediatric lymphomas.The aim of this study was to investigate the clinicopatho...Background:Non-Hodgkin lymphoma is the fourth most common malignant tumors in children,Burkitt lymphoma (BL) accounts for 30-50% of all pediatric lymphomas.The aim of this study was to investigate the clinicopathologic features,immunophenotype,Epstein-Barr virus (EBV) infection and c-myc gene rearrangement of sporadic BL in children.Methods:Ninety-two cases of pediatric BL were retrospectively analyzed for clinical features,immunohistochemistry,EBV-encoded RNA (EBER) status by in situ hybridization and c-myc gene rearrangement by fluorescence in situ hybridization.Results:In the 92 cases,male is predominant in sex distribution (M:F =3.38:1).The average age at diagnosis was 4.97 years.Polypoid BL showed a lower clinical stage (P =0.002),and advanced clinical stage and low serum albumin level at diagnosis were associated with poor outcome (P =0.024 and 0.053,respectively).The positive expression of CD10,B-cell lymphoma-6,MUM1 and EBER were 95.7% (88 cases),92.4% (85 cases),22.8% (21 cases),41.3% (38 cases),respectively.The expression of MUM1 were not associated with EBV infection status (P =1.000).c-myc gene rearrangement was detected in 94.6% (87/92).Clinical treatment information for 54 cases was collected,21 patients died of tumor after surgery alone,33 patients received surgery and chemotherapy,and of which six patients died shortly afterwords (MUM 1 positive expression in 3 cases,P =0.076).Conclusions:The anatomical location,growth pattern and serum albumin level of BL were associated with biological behavior.MUM1 may be a potential adverse prognostic marker,and not associated with EBV infection status.展开更多
Importance Burkitt lymphoma with bone marrow involvement and Burkitt leukemia behave aggressively.Thus far,there are limited data concerning survival and toxicity in Chinese children with Burkitt lymphoma or Burkitt l...Importance Burkitt lymphoma with bone marrow involvement and Burkitt leukemia behave aggressively.Thus far,there are limited data concerning survival and toxicity in Chinese children with Burkitt lymphoma or Burkitt leukemia who have undergone treatment with the non-Hodgkin’s lymphoma Berlin-Frankfurt-Münster-90/95(NHL-BFM-90/95)protocol.Objective To analyze outcomes and toxicity in pediatric patients who exhibit Burkitt lymphoma with bone marrow involvement or Burkitt leukemia following treatment with the NHL-BFM-90/95 protocol.Methods Patients aged<18 years with bone marrow involvement/leukemia who were treated with the NHL-BFM-90/95 protocol,with or without rituximab,in Sun Yat-Sen University Cancer Center from April 2004 to December 2018 were included in this retrospective analysis.Results Twenty-five patients were eligible.Burkitt lymphoma with bone marrow involvement and Burkitt leukemia were present in 10 and 15 patients,respectively.Central nervous system infiltration was not observed in any patients.All patients underwent chemotherapy involving NHL-BFM-90/95 protocol.Six courses of treatment were administered to each patient(v-AA-BB-CC-AA-BB-CC).The BFM-90/95 plus rituximab protocol was administered to 13 patients.The median follow-up interval was 31.9 months(range,2.5–158 months).Of the 25 patients,four died:three died of tumor progression and one died of therapy abandonment after relief of tumor lysis syndrome.The estimated 5-year event-free survival and overall survival rates were both 85.8%±5.0%.Interpretation Chinese pediatric patients who exhibit Burkitt lymphoma with bone marrow involvement or Burkitt leukemia can achieve optimal treatment outcomes and exhibit good tolerance when using the NHL-BFM-90/95 protocol.展开更多
Background:The prognostic values of interim and post-therapy fluorine-18-fluorodeoxyglucose(^(18)F-FDG) positron emission tomography(PET) and PET/computed tomography(CT) scanning have been confirmed in several subtype...Background:The prognostic values of interim and post-therapy fluorine-18-fluorodeoxyglucose(^(18)F-FDG) positron emission tomography(PET) and PET/computed tomography(CT) scanning have been confirmed in several subtypes of lymphoma.However,its prognostic value in Burkitt's lymphoma has not been clearly defined.The aim of the present study was to assess the prognostic value of PET/CT scanning during different treatment processes of Burkitt's lymphoma.Methods:A total of 29 adult patients with newly diagnosed Burkitt's lymphoma were retrospectively involved in this study;of them,23 patients underwent baseline PET/CT,15 patients underwent mid-therapy PET/CT after 1-4 cycles of chemotherapy,and 17 patients underwent post-therapy PET/CT after all planned first-line chemotherapy cycles.Mid-therapy and post-therapy PET/CT results(positive vs.negative) were visually interpreted according to the criteria of the International Harmonization Project.The reduction in the maximum standardizes uptake values(ASUVmax)of 25%,50%,and 75%were regarded as cutoff points.Overall survival(OS) and progression-free survival(PFS) were regarded as the major endpoints.Results:The median OS and PFS were 27.6 months(range 6.5-78.3 months) and 27.2 months(range 3.0-78.3 months),respectively.The median SUVmax of the baseline PET/CT was 18.3(range 1.6-35.9),whereas the median SUVmax of the mid-therapy and post-therapy PET/CT decreased to 4.0(range 0-17.6) and 3.0(range 0-14.5),respectively.The patients' Eastern Cooperative Oncology Group(ECOG) scores(<2 vs.≥2) were significantly associated with the baseline PET/CT SUVmax.The mid-therapy and post-therapy PET/CT results(positive vs.negative) showed no significant association with OS or PFS.The optimal cutoff ASUVmax from the baseline to the post-therapy PET/CT that could predict a change in OS in patients with Burkitt's lymphoma was 50%(P = 0.019).Conclusions:^(18)F-FDG uptake was intense in Burkitt's lymphoma,and there was a significant reduction in SUVmax during the interim and post-therapy PET/CT procedures.A ASUVmax of greater than 50%was a favorable cutoff point to predict the OS of Burkitt's lymphoma patients.展开更多
Burkitt's lymphoma is a highly aggressive non-Hodgkin lymphoma, often presenting in extra-nodal sites. It generally has a poor spontaneous outcome and needs aggressive treatment with systemic and intrathecal chemothe...Burkitt's lymphoma is a highly aggressive non-Hodgkin lymphoma, often presenting in extra-nodal sites. It generally has a poor spontaneous outcome and needs aggressive treatment with systemic and intrathecal chemotherapy. Occurrence at the gastric site is rare. We report the case of a 39-year old woman who presented with a prominent ulcerated lesion of the antrum corresponding histologically to a Burkitt's lymphoma associated with He/icobacter py/ori (H pylori) infection. Interphase fluorescence in situ hybridization (FISH) demonstrated c-MYC gene rearrangement in tumour cells without BCL2 or BCL6 gene translocations. Ulcer healing and tumour regression with a complete histological response were obtained 8 wk after Hpylori eradication. In spite of this complete remission, taking into account the high risk of recurrence, the patient received systemic and intrathecal chemotherapy. Two years later, the patient remained in complete remission. This is the first report of a gastric Burkitt's lymphoma responding to Hpylori eradication. These findings raise the question of the potential role of H pylori in the pathogenesis of some gastric Burkitt's lymphomas, and show the importance of searching for and eradicating the bacteria in combination with conventional chemotherapy regimens.展开更多
This article reported the distribution of DNase-1 sensitive regions in genomes of three Burkitt's lymphoma cell lines, P3HR-1, Raji and Ramos cell lines using a new method of in situ nick translation of chromosome...This article reported the distribution of DNase-1 sensitive regions in genomes of three Burkitt's lymphoma cell lines, P3HR-1, Raji and Ramos cell lines using a new method of in situ nick translation of chromosomes substituted completely by BrdU. The results showed that the Blym locus on chromosomes In three cell lines and the c-myc locus on chromosomes in P3HR-1 were the DNase-1 sensitive regions and found that the rearrangemental sites of chromosomes present in three Burkltt' s lymphoma cell lines were sensitive to DNase-1 digestion, Indicating that c-myc, bcl-1 genes located at the rearrangemental sites and the Blym gene in Burkltt' s lymphoma are the active genes having the capability of expression.展开更多
基金supported by the National Natural Science Foundation of China(81170467 and 81270569)Major Project of PLA Medical S&T foundation(AWS11C004)Medical Science Research Foundation of Chongqing Health and Family Planning Committee(2015MSXM224)
文摘Burkitt lymphoma is a highly aggressive B-cell neoplasm. New therapeutic methods are needed to overcome the adverse effect of intensive chemotherapy regimens. Valproic acid and (-)-gossypol are two kinds of chemical compounds used as new anti-tumor drugs in recent years.
文摘OBJECTIVE To summarize the clinical characteristics and treatment effects of Burkitt lymphoma (BL) and Burkitt-like lymphoma (BLL), and to explore the best possible optimal regimens and the treatment-related complications.METHODS Clinical data of 13 BL and BLL patients, who were pathologically diagnosed and treated in the Beijing Cancer Hospital from August 1996 to October 2008, were retrospectively analyzed, All patients received the therapeutic regimen with chemotherapy as the first-line treatment. The treatment effect and adverse reactions were evaluated.RESULTS Of the 13 patients, 12 were men and 1 was woman, with a median age of 15 years (range, 11-62). Three of the patients were in stage Ⅰ, 2 in stage Ⅱ, 2 in stage Ⅲ, and 6 in stage Ⅳ. Advanced cases were 8 (stage Ⅲ and Ⅳ), accounting for 61.5% of the patients. Bone marrow involvement was found in 2 cases (15.4%) and central nervous system invasion in 4 (30.8%) at the beginning of the treatment. The common involved sites included the superficial lymph nodes (61.5%), abdominal organs (53.8%), and abdominal and retro-peritoneal lymph nodes (38.5%). B symptoms were observed in 7 patients (53.8%). Serum lactate dehydrogenase (LDH) level was increased in 8 of the 10 patients who underwent the serum LDH determination, while serum uric acid level was increased in 1 of the 10. Pathological diagnosis showed that 11 of the cases were BL and 2 BLL. Of the 13 patients, 11 (84.6%) achieved a complete remission (CR) or CR/unconfirmed (CRu), and 1 (7.7%) a partial remission (PR), with a total responsive rate of 92.3%. At a median follow-up of 8 months (range 5-35), 6 patients were found dead at the end of the follow-up. One of the 13 patients was lost to the follow-up. The 1-year overall survival, progression-free survival and disease-free survival rates were 56.98%, 32.31% and 39.77%, respectively. The grade-Ⅲ or Ⅳ myelosuppression was found in 9 patients during the chemotherapy (69.2%), and tumor lysis syndrome and grade- Ⅳ total gastrointestinal mucositis were seen in 1.CONCLUSION Intensive short-course chemotherapy is recommended as the optimal first-line treatment for BL and BLL, and actively preventive treatments for chemotherapy-related adverse reactions are essential in the treatment course.
基金supported by grants from the Natural Science Foundation of Guangdong Province,China(Grant No.2019A1515010742)。
文摘Objective:To characterize modified R-CODOX-M/IVAC-based chemotherapy to lower the severe adverse events in Chinese adult patients with sporadic Burkitt lymphoma.Methods:We enrolled a retrospective cohort including 123 adult patients with untreated sporadic Burkitt lymphoma from August 2008 to September 2019 at Sun Yat-sen University Cancer Center.We studied a dose-modified and long-course R-CODOX-M/IVAC regimen utilizing a low dose of 1.0 g/m2/cycle cyclophosphamide,2 g/m2/cycle methotrexate,4,500 mg/m2/cycle ifosfamide,and 4.0 g/m2/cycle cytarabine.Forty-nine patients with low risk disease underwent 4–6 cycles of dose-modified R-CODOX-M-based chemotherapy.Seventy-four patients with high risk disease underwent 6–8 cycles of dose-modified alternating R-CODOX-M/IVAC regimens.Results:The objective remission was 87.0%.The event-free survival rate and overall survival at 3 years were 81.2%and 92.1%,respectively.Major grade 3–4 adverse events included leukopenia(91.9%),anemia(58.5%),thrombocytopenia(73.2%),and febrile neutropenia(48.8%).A total of 26.0%and 37.4%of patients received red blood cell and platelet transfusions,respectively.We observed 4 cases(3.3%)of septic shock after chemotherapy.Two treatment-related deaths occurred from severe infection.Conclusions:The modified R-CODOX-M/IVAC chemotherapy regimen was effective for sporadic Burkitt lymphoma in the Chinese population,with a lower toxicity than standard regimens.
基金The Science and Technology Planning Project of Guangzhou City,No.201803010018.
文摘BACKGROUND The incidence of gastric Burkitt lymphoma(BL),presenting as paraplegia and acute pancreatitis,is extremely low.BL is a great masquerader that presents in varied forms and in atypical locations,and it is prone to misdiagnosis and missed diagnosis.The prognosis of BL remains poor because of the difficulty in early diagnosis and the limited advances in chemotherapy.CASE SUMMARY A 53-year-old man was referred to our hospital from the local county hospital due to abdominal pain for two weeks and weakness in the lower extremities for one day.Magnetic resonance imaging of the abdomen and lumbar spine showed a swollen pancreas and gallbladder,with peripancreatic exudation and liquid collection,indicating acute pancreatitis and acute cholecystitis.Additionally,we observed abnormally thickened lesions of the gastric wall,multiple enlarged retroperitoneal lymph nodes and a well-demarcated,posterolateral extradural mass lesion between T9 and T12,with extension through the spinal foramen and definite bony destruction,suggesting metastasis in gastric malignancy.Subsequent whole-body positron emission tomography/computed tomography examination showed multifocal malignant lesions in the stomach,pancreas,gallbladder,bone,bilateral supraclavicular fossa,anterior mediastinum,bilateral axillary and retroperitoneal lymph nodes.Gastroduodenal endoscopy revealed primary BL with massive involvement of the gastric body and duodenum.The patient refused chemotherapeutic treatment and died one week later due to upper gastrointestinal hemorrhage.Afterward,we reviewed the characteristics of 11 patients with BL involving the stomach,pancreas or spinal cord.CONCLUSION Clinicians should be aware that BL can be the potential cause of acute pancreatitis or a rapidly progressive spinal tumor with accompanying paraplegia.For gastric BL,gastroscopy biopsies and pathology are necessary for a definite diagnosis.
基金Medical and Health Research Project of Hainan Province,No.20A200013.
文摘BACKGROUND Multiple primary cancer refers to more than one synchronous or sequential cancer in the same individual.Multiple primary cancer always presents as solid cancer or acute myeloid leukemia(AML)secondary to lymphoma.Here,we report a rare case of secondary peripheral T-cell lymphoma and AML after Burkitt lymphoma treatment.CASE SUMMARY A 54-year-old female patient was admitted to our hospital complaining of edema on her left lower limb.Physical examination revealed multiple superficial lymphadenectasis on her neck and pelvis.Color ultrasonography examination showed multiple uterine fibroids and a solid mass at the lower left side of the abdomen.Pathological biopsy revealed Burkitt lymphoma.After three hyper-CVAD(A+B)regimens,she achieved complete remission.Two years later,lymphadenectasis reoccurred.A relevant biopsy confirmed the diagnosis of peripheral T-cell lymphoma,which was accompanied by gastrointestinal invasion and hemocytopenia.Meanwhile,bone marrow examination revealed AML.On the second day of scheduled treatment,she developed gastrointestinal bleeding,peptic ulcers,and hemorrhagic shock and was critically ill.She was then discharged from the hospital due to financial concerns.CONCLUSION This is the first report of secondary peripheral T-cell lymphoma and AML after Burkitt lymphoma treatment with heterochronous and synchronal multiple primary cancers.
文摘<strong>Introduction:</strong> Non-Hodgkin’s malignant lymphoma is found primarily in African children. Prostate localization is a rare entity. We report the case of a secondary prostatic localization of Burkitt lymphoma in a 14-year-old child. <strong>Observation:</strong> the 14-year-old child NNJ was followed at the Libreville Cancer Institute (ICL) for Burkitt maxillo-facial stage II bilateral Murphy lymphoma. The clinical examination noted an alteration of the general state, total hematuria, a voluminous prostatic mass filling the rectal ampoule. The paraclinical assessment noted renal insufficiency, a prostatic mass on ultrasound. Anatomo-pathological examination of the prostatic biopsy diagnosed Burkitt type non-Hodgkin’s malignant lymphoma. He died a week later. <strong>Conclusion:</strong> Primary or secondary prostatic lymphoma is rare. Obstructive renal insufficiency is an additional complication that darkens its prognosis by delaying etiological treatment based on polychemotherapy.
文摘BACKGROUND Burkitt lymphoma(BL)is an exceptionally aggressive malignant neoplasm that arises from either the germinal center or post-germinal center B cells.Patients with BL often present with rapid tumor growth and require high-intensity multidrug therapy combined with adequate intrathecal chemotherapy prophylaxis,however,a standard treatment program for BL has not yet been established.It is important to identify biomarkers for predicting the prognosis of BLs and discriminating patients who might benefit from the therapy.Microarray data and sequencing information from public databases could offer opportunities for the discovery of new diagnostic or therapeutic targets.AIM To identify hub genes and perform gene ontology(GO)and survival analysis in BL.METHODS Gene expression profiles and clinical traits of BL patients were collected from the Gene Expression Omnibus database.Weighted gene co-expression network analysis(WGCNA)was applied to construct gene co-expression modules,and the cytoHubba tool was used to find the hub genes.Then,the hub genes were analyzed using GO and Kyoto Encyclopedia of Genes and Genomes analysis.Additionally,a Protein-Protein Interaction network and a Genetic Interaction network were constructed.Prognostic candidate genes were identified through overall survival analysis.Finally,a nomogram was established to assess the predictive value of hub genes,and drug-gene interactions were also constructed.RESULTS In this study,we obtained 8 modules through WGCNA analysis,and there was a significant correlation between the yellow module and age.Then we identified 10 hub genes(SRC,TLR4,CD40,STAT3,SELL,CXCL10,IL2RA,IL10RA,CCR7 and FCGR2B)by cytoHubba tool.Within these hubs,two genes were found to be associated with OS(CXCL10,P=0.029 and IL2RA,P=0.0066)by survival analysis.Additionally,we combined these two hub genes and age to build a nomogram.Moreover,the drugs related to IL2RA and CXCL10 might have a potential therapeutic role in relapsed and refractory BL.CONCLUSION From WGCNA and survival analysis,we identified CXCL10 and IL2RA that might be prognostic markers for BL.
文摘Adult intussusception is rare, highly associated with a malignant lead point, and often requires emergent surgical management. We report the case of a 44-year-old male who presented with generalized abdominal pain and was found to have early ileocolic intussusception secondary to a large ileocecal mass. Biopsies of the mass and an enlarged cardiophrenic lymph node, as well as pleural fluid cytology were all consistent with Burkitt lymphoma (BL). Curiously, the patient’s abdominal exam was reassuring, and the intussusception and malignant bowel obstruction resolved over 36 hours with conservative management alone. With a Burkitt lymphoma international prognostic index (BL-IPI) score of 2, the patient proceeded to treatment with combination chemoimmunotherapy and attained a complete response after four cycles. There was no bowel perforation or recurrent intussusception throughout treatment. Thus, this report marks the first reported case of adult BL-associated intussusception to resolve with non-invasive management and establishes a precedent for conservative management in select patients.
基金supported by a grant from the Cancer Research UK,London(Programme Award C5575/A15032)
文摘In 1964,a new herpesvirus,Epstein-Barr virus(EBV),was discovered in cultured tumor cells derived from a Burkitt lymphoma(BL)biopsy taken from an African patient.This was a momentous event that reinvigorated research into viruses as a possible cause of human cancers.Subsequent studies demonstrated that EBV was a potent growth-transforming agent for primary B cells,and that all cases of BL carried characteristic chromosomal translocations resulting in constitutive activation of the c-MYC oncogene.These results hinted at simple oncogenic mechanisms that would make Burkitt lymphoma paradigmatic for cancers with viral etiology.In reality,the pathogenesis of this tumor is rather complicated with regard to both the contribution of the virus and the involvement of cellular oncogenes.Here,we review the current understanding of the roles of EBV and c-MYC in the pathogenesis of BL and the implications for new therapeutic strategies to treat this lymphoma.
文摘Background: Tumor lysis syndrome is unknown to many neurosurgical teams. A dreaded complication, especially in the course of hematological malignancies, like it was the case in two of our patients operated for spinal cord compression by Burkitt’s lymphoma. Objectives: To describe the clinical and biological presentation of this potentially fatal complication, which can be improved by early preventive or curative treatments. Case Reports: Patient 1: A 49-year-old woman was admitted for vertebral and intercostal pain and paraparesis. Myelography revealed epidural medullary compression at the 7th thoracic vertebrae (Th7) level. Patient 2: A 67-year-old woman was admitted for cervico-thoracic pain and paraparesis. MRI showed posterior epidural medullary compression between the 1st thoracic vertebrae (Th1) and 5th thoracic vertebrae (Th5). In both cases, an urgent decompressive laminectomy was performed. They had also received postoperative corticosteroid therapy. The multi-visceral failure occurred on the 1st day (D1) and 3rd day (D3) postoperatively, respectively. In the absence of adequate management, the outcome was fatal for the two patients respectively at D3 and D5 postoperatively. This syndrome was retained in the presence of glucocorticoid (triggering factor) and the histological result in favor of Burkitt lymphoma (target disease). Conclusion: Malignant hemopathies is a common cause of spinal cord compressions. From these two cases, the importance of biological and clinical surveillance emerges, with patients requiring urgent management for radiculo-medullar tumor compression.
基金by a grant from the special fund of the pediatric medical coordinated development center of the Beijing Children’s Hospital(No.XTZD20180204).
文摘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 parameningeal 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.
文摘Background:Non-Hodgkin lymphoma is the fourth most common malignant tumors in children,Burkitt lymphoma (BL) accounts for 30-50% of all pediatric lymphomas.The aim of this study was to investigate the clinicopathologic features,immunophenotype,Epstein-Barr virus (EBV) infection and c-myc gene rearrangement of sporadic BL in children.Methods:Ninety-two cases of pediatric BL were retrospectively analyzed for clinical features,immunohistochemistry,EBV-encoded RNA (EBER) status by in situ hybridization and c-myc gene rearrangement by fluorescence in situ hybridization.Results:In the 92 cases,male is predominant in sex distribution (M:F =3.38:1).The average age at diagnosis was 4.97 years.Polypoid BL showed a lower clinical stage (P =0.002),and advanced clinical stage and low serum albumin level at diagnosis were associated with poor outcome (P =0.024 and 0.053,respectively).The positive expression of CD10,B-cell lymphoma-6,MUM1 and EBER were 95.7% (88 cases),92.4% (85 cases),22.8% (21 cases),41.3% (38 cases),respectively.The expression of MUM1 were not associated with EBV infection status (P =1.000).c-myc gene rearrangement was detected in 94.6% (87/92).Clinical treatment information for 54 cases was collected,21 patients died of tumor after surgery alone,33 patients received surgery and chemotherapy,and of which six patients died shortly afterwords (MUM 1 positive expression in 3 cases,P =0.076).Conclusions:The anatomical location,growth pattern and serum albumin level of BL were associated with biological behavior.MUM1 may be a potential adverse prognostic marker,and not associated with EBV infection status.
基金The authors would like to thank the participants and study staff of Sun Yat-sen Univesity Cancer Center for their contributions to this article.
文摘Importance Burkitt lymphoma with bone marrow involvement and Burkitt leukemia behave aggressively.Thus far,there are limited data concerning survival and toxicity in Chinese children with Burkitt lymphoma or Burkitt leukemia who have undergone treatment with the non-Hodgkin’s lymphoma Berlin-Frankfurt-Münster-90/95(NHL-BFM-90/95)protocol.Objective To analyze outcomes and toxicity in pediatric patients who exhibit Burkitt lymphoma with bone marrow involvement or Burkitt leukemia following treatment with the NHL-BFM-90/95 protocol.Methods Patients aged<18 years with bone marrow involvement/leukemia who were treated with the NHL-BFM-90/95 protocol,with or without rituximab,in Sun Yat-Sen University Cancer Center from April 2004 to December 2018 were included in this retrospective analysis.Results Twenty-five patients were eligible.Burkitt lymphoma with bone marrow involvement and Burkitt leukemia were present in 10 and 15 patients,respectively.Central nervous system infiltration was not observed in any patients.All patients underwent chemotherapy involving NHL-BFM-90/95 protocol.Six courses of treatment were administered to each patient(v-AA-BB-CC-AA-BB-CC).The BFM-90/95 plus rituximab protocol was administered to 13 patients.The median follow-up interval was 31.9 months(range,2.5–158 months).Of the 25 patients,four died:three died of tumor progression and one died of therapy abandonment after relief of tumor lysis syndrome.The estimated 5-year event-free survival and overall survival rates were both 85.8%±5.0%.Interpretation Chinese pediatric patients who exhibit Burkitt lymphoma with bone marrow involvement or Burkitt leukemia can achieve optimal treatment outcomes and exhibit good tolerance when using the NHL-BFM-90/95 protocol.
基金supported by grants from the National Natural Science Foundation of China(No.81472759 and 81301903)Guangdong Natural Science Foundation(No.S2013010016331)+3 种基金Science and Technology Planning Project of Guangdong Province,China(No. 2014J4100163)Science and Technology Planning Project of Guangzhou, China(No.2013B021800142)Fundamental Research Funds for the Central Universities(No.10ykpy36)Research Award Funds for Outstanding Young Researchers in Sun Yat-sen University Cancer Center
文摘Background:The prognostic values of interim and post-therapy fluorine-18-fluorodeoxyglucose(^(18)F-FDG) positron emission tomography(PET) and PET/computed tomography(CT) scanning have been confirmed in several subtypes of lymphoma.However,its prognostic value in Burkitt's lymphoma has not been clearly defined.The aim of the present study was to assess the prognostic value of PET/CT scanning during different treatment processes of Burkitt's lymphoma.Methods:A total of 29 adult patients with newly diagnosed Burkitt's lymphoma were retrospectively involved in this study;of them,23 patients underwent baseline PET/CT,15 patients underwent mid-therapy PET/CT after 1-4 cycles of chemotherapy,and 17 patients underwent post-therapy PET/CT after all planned first-line chemotherapy cycles.Mid-therapy and post-therapy PET/CT results(positive vs.negative) were visually interpreted according to the criteria of the International Harmonization Project.The reduction in the maximum standardizes uptake values(ASUVmax)of 25%,50%,and 75%were regarded as cutoff points.Overall survival(OS) and progression-free survival(PFS) were regarded as the major endpoints.Results:The median OS and PFS were 27.6 months(range 6.5-78.3 months) and 27.2 months(range 3.0-78.3 months),respectively.The median SUVmax of the baseline PET/CT was 18.3(range 1.6-35.9),whereas the median SUVmax of the mid-therapy and post-therapy PET/CT decreased to 4.0(range 0-17.6) and 3.0(range 0-14.5),respectively.The patients' Eastern Cooperative Oncology Group(ECOG) scores(<2 vs.≥2) were significantly associated with the baseline PET/CT SUVmax.The mid-therapy and post-therapy PET/CT results(positive vs.negative) showed no significant association with OS or PFS.The optimal cutoff ASUVmax from the baseline to the post-therapy PET/CT that could predict a change in OS in patients with Burkitt's lymphoma was 50%(P = 0.019).Conclusions:^(18)F-FDG uptake was intense in Burkitt's lymphoma,and there was a significant reduction in SUVmax during the interim and post-therapy PET/CT procedures.A ASUVmax of greater than 50%was a favorable cutoff point to predict the OS of Burkitt's lymphoma patients.
文摘Burkitt's lymphoma is a highly aggressive non-Hodgkin lymphoma, often presenting in extra-nodal sites. It generally has a poor spontaneous outcome and needs aggressive treatment with systemic and intrathecal chemotherapy. Occurrence at the gastric site is rare. We report the case of a 39-year old woman who presented with a prominent ulcerated lesion of the antrum corresponding histologically to a Burkitt's lymphoma associated with He/icobacter py/ori (H pylori) infection. Interphase fluorescence in situ hybridization (FISH) demonstrated c-MYC gene rearrangement in tumour cells without BCL2 or BCL6 gene translocations. Ulcer healing and tumour regression with a complete histological response were obtained 8 wk after Hpylori eradication. In spite of this complete remission, taking into account the high risk of recurrence, the patient received systemic and intrathecal chemotherapy. Two years later, the patient remained in complete remission. This is the first report of a gastric Burkitt's lymphoma responding to Hpylori eradication. These findings raise the question of the potential role of H pylori in the pathogenesis of some gastric Burkitt's lymphomas, and show the importance of searching for and eradicating the bacteria in combination with conventional chemotherapy regimens.
文摘Burkitt淋巴瘤(Burkitt lymphoma, BL)是一种来源于生发中心B细胞的高度侵袭性淋巴瘤,常发生在结外或表现为急性白血病形式,临床可分为地方性BL、散发性BL、免疫缺陷相关性BL。免疫缺陷相关性Burkitt淋巴瘤多与HIV感染有关。在HIV感染相关淋巴瘤中,BL约占25%~40%[1]。前列腺淋巴瘤罕见,其中常见的淋巴瘤是弥漫性大 B 细胞淋巴瘤,BL罕见,已报道前列腺原发性BL有4例,均为散发性BL[2-5]。
文摘This article reported the distribution of DNase-1 sensitive regions in genomes of three Burkitt's lymphoma cell lines, P3HR-1, Raji and Ramos cell lines using a new method of in situ nick translation of chromosomes substituted completely by BrdU. The results showed that the Blym locus on chromosomes In three cell lines and the c-myc locus on chromosomes in P3HR-1 were the DNase-1 sensitive regions and found that the rearrangemental sites of chromosomes present in three Burkltt' s lymphoma cell lines were sensitive to DNase-1 digestion, Indicating that c-myc, bcl-1 genes located at the rearrangemental sites and the Blym gene in Burkltt' s lymphoma are the active genes having the capability of expression.