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The Oncologist Apr 2006Burkitt's lymphoma is a highly aggressive lymphoma identified and described in the last century by Denis Burkitt in Africa, in areas endemic for malaria. Since its... (Review)
Review
Burkitt's lymphoma is a highly aggressive lymphoma identified and described in the last century by Denis Burkitt in Africa, in areas endemic for malaria. Since its description in African children, it has been recognized outside areas with endemic malaria, frequently also in children as well as among individuals with an underlying immunodeficiency. Since its initial designation as Burkitt's lymphoma, this type of lymphoma and lymphomas closely resembling it have received a variety of names in different classifications of lymphomas and leukemias: undifferentiated lymphoma, Burkitt's and non-Burkitt's type in the modified Rappaport Classification, malignant lymphoma, small non-cleaved cell, Burkitt's type in the Working Formulation, Burkitt's lymphoma and high-grade B-cell lymphoma, Burkitt-like in the REAL Classification, and acute lymphoblastic leukemia, L3 type in the FAB Classification. With the publication of the WHO Classification of Haematopoietic and Lymphoid Tumors, the nomenclature of this lymphoma has come full circle, and it is once again known simply as Burkitt's lymphoma. In recent years, efforts have focused on improving therapy for this rapidly proliferating neoplasm while minimizing, to the extent possible, treatment-associated toxicity. These efforts have led to the development of high-intensity, short-duration combination chemotherapy that has proven extremely effective for a high proportion of Burkitt's lymphoma patients. The differential diagnosis of Burkitt's lymphoma is broad, and precise diagnosis based on histologic, immunophenotypic, and genetic features remains the critical first step in planning appropriate therapy.
Topics: Burkitt Lymphoma; Diagnosis, Differential; Humans; Neoplasm Staging
PubMed: 16614233
DOI: 10.1634/theoncologist.11-4-375 -
Lancet (London, England) Mar 2012Burkitt's lymphoma is a highly aggressive B-cell non-Hodgkin lymphoma and is the fastest growing human tumour. The disease is associated with Epstein-Barr virus and was... (Review)
Review
Burkitt's lymphoma is a highly aggressive B-cell non-Hodgkin lymphoma and is the fastest growing human tumour. The disease is associated with Epstein-Barr virus and was one of the first tumours shown to have a chromosomal translocation that activates an oncogene (c-MYC). Burkitt's lymphoma is the most common childhood cancer in areas where malaria is holoendemic. The incidence is very high in immunosuppressed patients in non-endemic areas, especially when associated with HIV infection. Outcome with intensive chemotherapy has improved and is now excellent in children, but the prognosis is poor in elderly adults. The success of intensive treatment relies on good supportive care. The therapy offered in oncology units in low-income countries is not as aggressive as in centres in high-income countries and outcomes are less successful. Adjuvant monoclonal antibody therapy with rituximab shows promise for improved outcomes and reduced toxic effects in the future.
Topics: Adolescent; Antibodies, Monoclonal, Murine-Derived; Antineoplastic Combined Chemotherapy Protocols; Burkitt Lymphoma; Child; Child, Preschool; Cross-Sectional Studies; Endemic Diseases; Epstein-Barr Virus Infections; HIV Infections; Health Services Accessibility; Humans; Incidence; Infant; Malaria; Poverty; Prognosis; Proto-Oncogene Proteins c-myc; Risk Factors; Rituximab; Transcriptional Activation; Translocation, Genetic
PubMed: 22333947
DOI: 10.1016/S0140-6736(11)61177-X -
Nature Oct 2012Burkitt's lymphoma (BL) can often be cured by intensive chemotherapy, but the toxicity of such therapy precludes its use in the elderly and in patients with endemic BL...
Burkitt's lymphoma (BL) can often be cured by intensive chemotherapy, but the toxicity of such therapy precludes its use in the elderly and in patients with endemic BL in developing countries, necessitating new strategies. The normal germinal centre B cell is the presumed cell of origin for both BL and diffuse large B-cell lymphoma (DLBCL), yet gene expression analysis suggests that these malignancies may use different oncogenic pathways. BL is subdivided into a sporadic subtype that is diagnosed in developed countries, the Epstein-Barr-virus-associated endemic subtype, and an HIV-associated subtype, but it is unclear whether these subtypes use similar or divergent oncogenic mechanisms. Here we used high-throughput RNA sequencing and RNA interference screening to discover essential regulatory pathways in BL that cooperate with MYC, the defining oncogene of this cancer. In 70% of sporadic BL cases, mutations affecting the transcription factor TCF3 (E2A) or its negative regulator ID3 fostered TCF3 dependency. TCF3 activated the pro-survival phosphatidylinositol-3-OH kinase pathway in BL, in part by augmenting tonic B-cell receptor signalling. In 38% of sporadic BL cases, oncogenic CCND3 mutations produced highly stable cyclin D3 isoforms that drive cell cycle progression. These findings suggest opportunities to improve therapy for patients with BL.
Topics: Basic Helix-Loop-Helix Transcription Factors; Burkitt Lymphoma; Cell Cycle; Cyclin D3; Cyclin-Dependent Kinase 6; Genes, myc; Genomics; High-Throughput Nucleotide Sequencing; Humans; Inhibitor of Differentiation Proteins; Molecular Targeted Therapy; Neoplasm Proteins; Phosphatidylinositol 3-Kinases; RNA Interference; Receptors, Antigen, B-Cell; Signal Transduction
PubMed: 22885699
DOI: 10.1038/nature11378 -
Oncology Reports Feb 2020The aim of the present study was to explore the possible mechanisms of phosphatase and tensin homolog (PTEN) in the pathogenesis of Burkitt's lymphoma, and provide novel...
The aim of the present study was to explore the possible mechanisms of phosphatase and tensin homolog (PTEN) in the pathogenesis of Burkitt's lymphoma, and provide novel information that can be used in the targeted treatment of this disease. PTEN lentiviral overexpression vector and short‑hairpin PTEN silencing vectors were constructed. The effect of PTEN on the growth and proliferation of CA46 and RAJI cells was analyzed using a Cell Counting Kit‑8 assay. Apoptosis was detected by Hoechst 33342 and propidium iodide double staining. Flow cytometry was used to analyze the cell cycle. A Transwell chamber was used to detect cell migration and invasion abilities. Western blot analysis was used to detect related protein changes. The mechanism of the effect of PTEN on the biological characteristics of Burkitt's lymphoma cells was subsequently analyzed. The results revealed that PTEN inhibited the proliferation of CA46 and RAJI cells by downregulating the expression of p‑AKT, It was indicated that the upregulation of proapoptotic proteins (including Bad and Bax) induced apoptosis, regulated cyclin (including P53, P21, CDK4, CDK6, cyclin D3 and cyclin H) to inhibit cell cycle progression, and mediated epithelial‑mesenchymal transition‑like cell markers (including E‑cadherin, N‑cadherin, β‑catenin, TCF‑8, vimentin, Slug and Snail) to inhibit cell migration and invasion. In conclusion, the tumor‑suppressor gene PTEN inhibited the phosphoinositide 3‑kinase/protein kinase B (PI3K/AKT) signaling pathway and inhibited the proliferation and migration of Burkitt's lymphoma cells, induced apoptosis and cell cycle arrest, thus playing a crucial role in the pathogenesis of Burkitt's lymphoma.
Topics: Apoptosis; Burkitt Lymphoma; Cell Cycle; Cell Line, Tumor; Cell Proliferation; Epithelial-Mesenchymal Transition; Gene Expression Regulation, Neoplastic; Humans; PTEN Phosphohydrolase; Phosphorylation; Proto-Oncogene Proteins c-akt; Signal Transduction
PubMed: 31922234
DOI: 10.3892/or.2020.7457 -
Acta Medica Portuguesa 2011Burkitt's lymphoma (BL) is a highly aggressive B-cell neoplasm characterized by the translocation and deregulation of the c-myc gene on chromosome 8. Three distinct...
BACKGROUND
Burkitt's lymphoma (BL) is a highly aggressive B-cell neoplasm characterized by the translocation and deregulation of the c-myc gene on chromosome 8. Three distinct clinical forms of BL are recognized: endemic, sporadic, and human immunodeficiency-associated. BL is a rapidly growing neoplasm requiring immediate diagnosis and treatment.
AIM
We described and analyzed our experience with Burkitt's lymphoma (BL) diagnosis, treatment and outcome, during ten years.
MATERIALS AND METHODS
Retrospective study; clinical records of all children admitted with BL between 1st January 1998 and 31st December 2008 were analyzed. The following data were collected: age at admission, gender, clinical presentation, and time elapsed from initial complaints until diagnosis, disease localization, treatment and evolution.
RESULTS
During the time period 21 children were admitted (19 boys), seven (33.3%) of which were diagnosed in 2008. The median age at diagnosis was seven years with a mean delay to diagnosis of 20,8 days (range 2-125 days). The most frequent site of primitive tumour was the abdomen (13), followed by tonsils (three), orbit (one), central nervous system CNS (two), tongue (one) and nasopharynx (one). The majority of patients in our study were presenting with a painfull abdominal mass. Diagnosis was established through tumour biopsy in 17 children, three by paracentesis or toracocentesis and one case was diagnosed only by genetic tests to the bone marrow. Genetic tests were positive in 11 patients. According to the Murphy classification, there were three stage II, 12 stage III and six stage IV tumours; 29% and 19% had bone marrow and central nervous system involvement, respectively. One child relapsed and was successfully treated with Rituximab® and autologous stem cell transplantation. The overall survival rate was 100%.
Topics: Adolescent; Burkitt Lymphoma; Child; Child, Preschool; Female; Humans; Male; Retrospective Studies
PubMed: 22525625
DOI: No ID Found -
British Medical Journal Jun 1978
Topics: Antibodies, Viral; Burkitt Lymphoma; Herpesvirus 4, Human; Humans
PubMed: 207381
DOI: No ID Found -
African Health Sciences Sep 2007Burkitt's lymphoma (BL) was first described in Eastern Africa, initially thought to be a sarcoma of the jaw. Shortly it became well known that this was a distinct form... (Review)
Review
Burkitt's lymphoma (BL) was first described in Eastern Africa, initially thought to be a sarcoma of the jaw. Shortly it became well known that this was a distinct form of Non Hodgkin's lymphoma. The disease has given insight in all aspects of cancer research and care. Its peculiar epidemiology has led to the discovery of Epstein Barr virus (EBV) and its importance in the cause of several viral illnesses and malignancies. The highest incidence and mortality rates of BL are seen in Eastern Africa. BL affects mainly children, and boys are more susceptible than girls. Evidence for a causal relationship between EBV and BL in the endemic form is fairly strong. Frequency of association between EBV and BL varies between different patient groups and different parts of the world. EBV may play a role in the pathogenesis of BL by deregulation of the oncogene c-MYC by chromosomal translocation. Although several studies suggest an association between malaria and BL, there has never been a conclusive population study in support of a direct role of malaria in causation of BL. The emergence of HIV and a distinct subtype of BL in HIV infected have brought a new dimension to the disease particularly in areas where both HIV and BL are endemic. BL has been reported as a common neoplasmin HIV infected patients, but not in other forms of immuno-depression, and the occurrence of BL seems to be higher amongst HIV positive adults, while the evidence of an association amongst children is still disputed. The role of other possible risk factors such as low socio-economical status, exposure to a plant species common in Africa called Euphorbiaceae, exposure to pesticies and to other infections such as schistosomiasis and arbovirus (an RNA virus transmitted by insect vectors) remain to be elucidated.
Topics: Adolescent; Adult; Africa; Aged; Aged, 80 and over; Burkitt Lymphoma; Child; Child, Preschool; Female; Humans; Incidence; Infant; Infant, Newborn; Male; Middle Aged
PubMed: 18052871
DOI: 10.5555/afhs.2007.7.3.166 -
Cancer Chemotherapy and Pharmacology Apr 2024Burkitt's lymphoma, one of the most common subtypes of pediatric malignant lymphoma, is notorious for its swift onset, aggressive proliferation, pronounced invasiveness,...
BACKGROUND
Burkitt's lymphoma, one of the most common subtypes of pediatric malignant lymphoma, is notorious for its swift onset, aggressive proliferation, pronounced invasiveness, and marked malignancy. The therapeutic landscape for Burkitt's lymphoma currently falls short of providing universally effective and tolerable solutions. Andrographolide, a primary active component of Andrographis paniculata, is renowned for its properties of heat-clearing, detoxification, inflammation reduction, and pain relief. It is predominantly used in treating bacterial and viral infections of the upper respiratory tract, as well as dysentery. Various reports highlight the antitumor effects of andrographolide. Yet, its specific impact and the underlying mechanism of action on Burkitt's lymphoma remain an uncharted area of research.
METHOD
We employed network pharmacology to pinpoint the targets of andrographolide's action on Burkitt's lymphoma and the associated pathways. We then evaluated the impact of andrographolide on Burkitt's lymphoma using both in vitro and in vivo patient-derived xenograft (PDX) models. Concurrently, we confirmed the molecular targets of andrographolide in Burkitt's lymphoma through immunofluorescence assays.
RESULT
Utilizing network pharmacology, we identified 15 relevant targets, 60 interrelationships between these targets, and numerous associated signaling pathways for andrographolide's action on Burkitt's lymphoma. In vitro efficacy tests using High-throughput Drug Sensitivity Testing and in vivo PDX model evaluations revealed that andrographolide effectively curtailed the growth of Burkitt's lymphoma. Moreover, we observed a increased in the expression of JUN (c-Jun) and CASP3 (Caspase 3) proteins in Burkitt's lymphoma cells treated with andrographolide.
CONCLUSION
Andrographolide inhibits the growth of Burkitt's lymphoma by inhibiting JUN and CASP3 proteins.
Topics: Humans; Child; Burkitt Lymphoma; Caspase 3; Diterpenes
PubMed: 38148335
DOI: 10.1007/s00280-023-04626-4 -
International Journal of Cancer Apr 2009Burkitt's lymphoma (BL) was first described as a clinical entity in children in Central Africa by Denis Burkitt in 1958. The particular epidemiological features of this... (Review)
Review
Burkitt's lymphoma (BL) was first described as a clinical entity in children in Central Africa by Denis Burkitt in 1958. The particular epidemiological features of this tumor initiated the search for a virus as the causative agent and led to the discovery of Epstein-Barr virus (EBV) by Epstein and coworkers in 1964. It became apparent in the seventies and eighties that the tumor is not restricted to Central Africa, but occurs with lesser incidence all over the world (sporadic BL) and is also particularly frequent in HIV infected individuals, and that not all BL cases are associated with EBV: about 95% of the cases in Central Africa, 40 to 50% of the cases in HIV-infected individuals and 10 to 20% of the sporadic cases harbour the viral information and express at least one viral antigen (EBNA1) and a number of non-coding viral RNAs. In contrast, all BL cases regardless of their geographical origin exhibit one of three c-myc/Ig chromosomal translocations leading to the activation of the c-myc gene as a crucial event in the development of this disease. Although epidemiological evidence clearly points to a role of the virus in the African cases, the role of EBV in the pathogenesis of BL has remained largely elusive. This review summarizes current concepts and ideas how EBV might contribute to the development of BL in the light of the progress made in the last decade and discusses the problems of the experimental systems available to test such hypotheses.
Topics: Animals; B-Lymphocytes; Burkitt Lymphoma; Epstein-Barr Virus Infections; Epstein-Barr Virus Nuclear Antigens; Genes, myc; Herpesvirus 4, Human; Humans; Immunologic Memory; Malaria; MicroRNAs; Models, Biological; Translocation, Genetic
PubMed: 19165855
DOI: 10.1002/ijc.24223 -
Zhongguo Dang Dai Er Ke Za Zhi =... May 2022To study the clinical features and chemotherapy response of Burkitt's lymphoma (BL) in children and the influence of rituximab on the prognosis of children with BL.
OBJECTIVES
To study the clinical features and chemotherapy response of Burkitt's lymphoma (BL) in children and the influence of rituximab on the prognosis of children with BL.
METHODS
A retrospective analysis was performed for the medical data of 62 children with BL, including clinical features, therapeutic efficacy, and prognostic factors. The Cox regression model was used to identify the factors associated with poor prognosis in children with BL. According to whether rituximab was used, the children with advanced (stage III/IV) BL were divided into two groups: chemotherapy plus rituximab and chemotherapy alone. The prognosis was compared between the two groups.
RESULTS
For these 62 children, the median age of onset was 5 years (range 1-14 years), and there were 58 boys (94%) and 4 girls (6%). The primary site was abdominal cavity in 41 children (66%), and head and neck in 16 children (26%). There were 1 child with stage I BL (2%), 8 with stage II BL (13%), 33 with stage III BL (53%), and 20 with stage IV BL (32%). The median follow-up time was 29 months, with progression/recurrence observed in 15 children (24%), and the 3-year overall survival (OS) rate and event-free survival (EFS) rate were 82.8%±5.2% and 77.3%±5.8%, respectively. For the children with stage III/IV BL, there was a significant difference in the 3-year the OS rate between the chemotherapy plus rituximab group (16 children) and the chemotherapy alone group (30 children) (93.3%±6.4% vs 65.6%±9.9%, =0.042), while there was no significant difference in the 3-year EFS rate between the two groups (86.2%±9.1% vs 61.8%±10.1%, >0.05). The Cox regression analysis showed that central nervous system involvement, lactate dehydrogenase >1 000 U/L, and early incomplete remission were the factors associated with poor prognosis (<0.05).
CONCLUSIONS
Chemotherapy combined with rituximab can improve the prognosis of children with stage III/IV BL. Central nervous system involvement, elevated lactate dehydrogenase level, and early incomplete remission may indicate a poor prognosis in children with BL.
Topics: Adolescent; Antineoplastic Combined Chemotherapy Protocols; Burkitt Lymphoma; Child; Child, Preschool; Female; Humans; Infant; Lactate Dehydrogenases; Male; Prognosis; Retrospective Studies; Rituximab
PubMed: 35644197
DOI: 10.7499/j.issn.1008-8830.2111064