Abstract

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.

The Lancet

Source

International Network for Cancer Treatment and Research, Brussels, Belgium Uniformed University of the Health Sciences National Cancer Institute, Bethesda, MD, USA.

Abstract

Keywords:

The two major epidemiological clues to the pathogenesis of Burkitt lymphoma (BL) are the geographical association with malaria – BL incidence relates to the malaria transmission rate – and early infection by Epstein-Barr virus (EBV). Both agents cause B cell hyperplasia, which is almost certainly an essential component of lymphomagenesis in BL. The critical event in lymphomagenesis is the creation of a MYC translocation, bringing the MYC gene into juxtaposition with immunoglobulin genes and causing its ectopic expression, thereby driving the proliferation of BL cells. It is highly likely that such translocations are mediated by the activation-induced cytidine deaminase (AID) gene, which is responsible for hypervariable region mutations as well as class switching. Stimulation of the Toll-like receptor 9 by malaria-associated agonists induces AID, providing a mechanism whereby malaria could directly influence BL pathogenesis. EBV-containing cells must reach the memory cell compartment in order to survive throughout the life of the individual, which probably requires traversal of the germinal centre. Normally, cells that do not produce high affinity antibodies do not survive this passage, and are induced to undergo apoptosis. EBV, however, prevents this, and in doing so may also enhance the likelihood of survival of rare translocation-containing cells.

Wiley Online Library

Source

Department of Pathology, University of Utah, Salt Lake City, UT.

Abstract

Burkitt lymphoma/leukaemia is the most common (40%) form of non-Hodgkin lymphoma that occurs in children and adolescents. The prognosis of advanced (disseminated) Burkitt lymphoma/leukaemia in children and adolescents three decades ago had a 5-year event-free survival (EFS) of <40%, and required combination chemotherapy and radiation therapy over a 1-2 year period. Currently, the prognosis for the same advanced stage has a 5-year EFS of 85-90% with <6 months of chemotherapy only. Radiation therapy has been eliminated for children and adolescents with Burkitt lymphoma/leukaemia except in emergencies, such as superior vena cava syndrome and acute neurological impairment or in patients with relapse/progression. Current risk factors in the prognosis of childhood and adolescent Burkitt lymphoma/leukaemia include: lactate dehydrogenase level ≥ 2× the upper normal limit at diagnosis, bone marrow and central nervous system involvement, poor response to cyclophosphamide, vincristine and prednisone reduction therapy and poor risk cytogenetics. New and novel therapeutic approaches include monoclonal antibody (anti-CD20) therapy, targeted cellular immune therapy and small molecule inhibitors. Future strategies should include improved staging and risk classification, reduction of cytotoxic chemotherapy, the investigation of targeted therapy, an increased understanding of the underlying biology of Burkitt lymphoma/leukaemia, strategies for prevention and approaches to reduce acute and chronic toxicities.

Wiley Online  Library

Source

Service d’odontologie chirurgicale, centre de consultation et de traitement dentaire, faculté de médecine dentaire, BP 6212, centre hospitalier d’Ibn Sina, Rabat, Maroc.

Abstract

We report a case of Burkitt lymphoma of the jaws in an immunocompetent adolescent, revealed by intraoral swelling. An orthopantomogram showed multiple osteolytic lesions. Biopsy revealed Burkitt lymphoma. The disease was treated with chemotherapy. Complete remission was attained 15 months after the end of treatment. Burkitt lymphomas accounts for 30-40% of all non-Hodgkin lymphomas in children, with diagnosis confirmed by histology. Immunophenotyping completes the diagnosis by identifying the presence of B markers. Chemotherapy is currently the main treatment of BL, because of the high chemosensitivity of the tumor and its low radiosensitivity. Overall survival in localized stages is close to 100%.

Science Direct

Source

Department of Oncology, St. Jude Children’s Research Hospital; Department of Pediatrics, University of Tennessee College of Medicine, Memphis, TN, USA.

Abstract

The refinements in both the staging and response evaluation of children with Burkitt lymphoma (BL) have contributed to the improvements in treatment outcome observed over the past 40 years. Ziegler and Magrath designed a staging system in the 1970s for children with BL in equatorial Africa. Currently, the most widely used staging system around the world is that described by Murphy in 1980, which was developed for children with non-Hodgkin lymphoma (NHL) of any histology. There are opportunities for refinement in this system, particularly with respect to certain extra-nodal sites, such as skin and bone. The findings obtained at diagnosis with novel technologies (functional imaging [e.g., positron emission tomography [PET]] and minimal residual disease [MRD] technology), which are more sensitive with respect to disease detection than historic modalities, also need to be considered. Technological advances have also had impact on the assessment of response evaluation. Standard x-rays were routinely used in the 1960s; nuclear imaging became widely used in the 1970s; computerized axial tomography was incorporated in the 1980s; PET imaging was incorporated and, in many cases, has replaced gallium/bone scans since 2000; and MRD technology has been explored in some of the most recent clinical trials. There is clearly a need for more clinical data on the use of PET and MRD technology in the determination of response evaluation of children with BL as well as other histological subtypes of NHL. An international working group is currently addressing the refinement of both disease staging and response evaluation in children with NHL.

Wiley Online Library

Abstract

Posttransplant lymphoproliferative disorder (PTLD) is a potentially fatal complication of solid organ transplantation. The majority of PTLD is of B-cell origin, and 90% are associated with the Epstein-Barr virus (EBV). Lymphomatoid granulomatosis (LG) is a rare, EBV-associated systemic angiodestructive lymphoproliferative disorder, which has rarely been described in patients with renal transplantation. We report the case of a patient with renal transplantation for SLE, who presented, 9 months after renal transplantation, an EBV-associated LG limited to the intracranial structures that recovered completely after adjustment of her immunosuppressive treatment. Nine years later, she developed a second PTLD disorder with central nervous system initial manifestation. Workup revealed an EBV-positive PTLD Burkitt lymphoma, widely disseminated in most organs. In summary, the reported patient presented two lymphoproliferative disorders (LG andBurkitt’s lymphoma), both with initial neurological manifestation, at 9 years interval. With careful reduction of the immunosuppression after the first manifestation and with the use of chemotherapy combined with radiotherapy after the second manifestation, our patient showed complete disappearance of neurologic symptoms and she is clinically well with good kidney function. No recurrence has been observed by radiological imaging until now.

PubMed

http://www.ncbi.nlm.nih.gov/pubmed/22312369

PNAEmu can significantly reduce Burkitt’s lymphoma tumor burden in a SCID mice model: cells dissemination similar to the human disease.

Cancer Gene Ther.  2009 Apr 10

Matis S, Mariani MR, Cutrona G, Cilli M, Piccardi F, Daga A, Damonte G, Millo E, Moroni M, Roncella S, Fedeli F, Boffa LC, Ferrarini M.

S.C. Oncologia Medica C, Istituto Nazionale per la Ricerca sul Cancro, IST, Genova, Italy.

In human Burkitt’s Lymphoma (BL) BRG cells, a t(8;14) translocation, placing c-myc near the Emu enhancer of the H chain locus, causes tumor expansion. Earlier, we showed that a peptide nucleic acid complementary to the Emu sequence (PNAEmu), specifically inhibited the expression of translocated c-myc and impaired the growth of BRG cells-induced subcutaneous tumors in mice suffering from severe combined immunodeficiency (SCID). In this study, the therapeutic potential of PNAEmu was evaluated in a systemic mouse model. BRG-BL cells transfected with the luciferase gene were inoculated intravenously into SCID mice resulting in a preferential expansion, similar to the one of human adult patients, in the abdominal cavity, central nervous system and bone marrow. The mice were chronically injected intraperitoneally either with PNAEmu or with control PNA. The treatment was stopped when the control animals developed severe neurological symptoms. As detected both by inspection at necropsy and imaging, overall tumor growth in PNAEmu-treated mice decreased by >80%. Histological and immunohistochemical studies showed, only in PNAEmu-treated mice, a substantially reduced BL cell growth at the major sites of invasion and vast areas of necrosis in the lymphomatous tissues, with concomitant c-myc expression downregulation. Altogether, the data support the therapeutic potential of PNAEmu in human adult BL.Cancer Gene Therapy advance online publication, 10 April 2009; doi:10.1038/cgt.2009.26.

Nature – Cancer Gene therapy

Burkitt’s lymphoma of the caecum in a pa-tient with AIDS: clinical case and review of the literature.

Minerva Chir. 2009 Apr

Siani LM, Siani A, Ricci V, D’Elia M, Masoni T, Uggeri G.

Unità Operativa Complessa di Chirurgia Generale, Ospedale S. Giacomo e Cristoforo, Massa, Massa Carrara, Italia lucamaria.siani@fastwebnet.it.

Overall, lymphomas of the gastrointestinal tract are rare, although they are the most frequent extranodal location. The incidence of primary colic lymphoma, above all in the non-Hodgkin variant, is clearly higher in the HIV positive population, especially in subjects with AIDS. The authors present the case of a 51-year-old patient with AIDS undergoing antiviral therapy; he was suffering from abdominal pain and presented a palpable mass in the right iliac fossa; diagnosis was caecal non-Hodgkin lymphoma (NHL); radical right hemicolectomy was carried out with definitive histological diagnosis of Burkitt-type small cell NHL. The NHL of the colon represents no more than 1.2% of all malignant cancers of this part of the intestinal tract. Nevertheless such cases are comparatively frequent in patients with HIV virus, especially in the active phase and clinically proven to be due to immunodeficient syndrome. Of cardinal importance is the differential diagnosis between primary and secondary forms because of the different treatment and prognosis. Frequently such forms are observed in patients with AIDS, at advanced stages and with differentiated and hence more aggressive histotypes, also because they are present in organisms weakened by the underlying disease and by immunodeficiency. Primary NHLs of the colon are relatively frequent and aggressive in patients with AIDS; early diagnosis and treatment are therefore of fundamental importance to improve the oncological outcome for these patients.

Minerva Medica

Intensive chemotherapy failure in Burkitt’s lymphoma with cavernous sinus involvement.

Med Oncol. 2009 Apr 14

Chen ZY, Wu B, Yin JL.

Department of Medical Oncology, Cancer Hospital, Fudan University, 270 Dong An Road, 200032, Shanghai, China.

Burkitt’s lymphoma is an aggressive B-cell lymphoma with rapid proliferative index, which makes it disseminate easily to distal sites, especially to bone marrow and the central nerve system. We report here a 22-year-old woman with Burkitt’s lymphoma involving multiple organs, including kidneys, breasts, left ovary, and bone marrow at the time of diagnosis. The patient responded well to intensive chemotherapy before the onset of retro-orbital pain, vomiting, and photophobia. The contrast-enhanced T1-weighted image in a second magnetic resonance image (MRI) showed a 2 x 1.9 cm mass in her left cavernous sinus that was not found in her initial MRI. Central nervous system (CNS)-directed high-dose chemotherapy and whole-brain radiation could not change the final failed treatment outcome caused by the cavernous sinus involvement disseminating to her entire CNS. Further study should provide well-designed therapeutic strategies to Burkitt’s patients with cavernous sinus involvement.

PubMed