Burkitts lymphoma society support and advocacy here to share. The african type of bl is closely associated with the epsteinbarr virus, the main cause of infectious mononucleosis. Burkitt lymphoma part 1 of 3 the selection, dosing, and administration of anticancer agents and the management of associated toxicities are complex. Sep 25, 2017 burkitt lymphoma bl is a very fastgrowing type of cancer.
Types of nonhodgkin lymphoma in children nonhodgkin lymphomas nhls are most often classified by how the cancer cells look under the microscope. Drug dose modifications and schedule and initiation of supportive care interventions are often necessary because of expected toxicities and. Discuss the treatment strategies used for treating burkitts lymphoma. Burkitt s lymphoma is the most common childhood cancer in areas where malaria is holoendemic. Nonhodgkin lymphoma causes, symptoms, diagnosis, treatment. Nonhodgkin lymphoma is a type of cancer of the lymphatic system, which helps your body fight infections. Treatment approaches are not modified by the presence of ebv. In the types commonly seen in the united states, the cancer often starts in the belly area abdomen. The role of ebv in the pathogenesis of burkitts lymphoma. Apr, 2012 gisele rodrigues gouveia, sheila aparecida coelho siqueira, and juliana pereira diffuse large bcell lymphoma is the most common subtype of nonhodgkin lymphoma in the west. It often responds well to treatment and, in many cases, can be cured with intensive. A combination of cyclophosphamide, doxyrubicin, vincristine and predinisone is one example of a drug therapy.
Burkitt lymphoma bl is a very fastgrowing type of cancer. Tumors can grow extremely quickly, sometimes doubling their size within 18 hours. This type of lymphoma is common wherever burkitt lymphoma is endemic in africa. Recognized as a fast growing human tumor, burkitt lymphoma is associated with impaired. The 2016 world health organization who classification of lymphoid neoplasms recognizes burkittlike lymphoma with 11q aberration as a new provisional entity that lacks myc. Burkitts lymphoma bl is a rare but very aggressive nonhodgkins lymphoma nhl accounting for 0.
Burkitt lymphoma pathogenesis and therapeutic targets from. Tumor lysis and acute renal failure in burkitts lymphoma. Nov 22, 2017 nonhodgkin lymphoma is a type of lymphoma characterized by the absence of reedsternberg cells. The disease is associated with epsteinbarr virus and was one of the first tumours. Burkitt lymphoma bl, a tumor occurring in endemic, sporadic and aidsassociated forms, is the classic example of a human malignancy whose pathogenesis involves a specific cellular genetic change, namely, a chromosomal translocation deregulating expression of the cmyc oncogene, complemented in many cases by the action of an oncogenic virus, the epsteinbarr virus ebv. Spacetime case clusters of endemic burkitts lymphoma. Burkitt lymphoma is a rare but highly aggressive fastgrowing bcell nonhodgkin lymphoma nhl. Diagnosis and initial workup must be completed rapidly to start treatment as. Hummel m, bentink s, berger h, et al, for the molecular mechanisms in malignant lymphomas network project of the deutsche krebshilfe. Burkitts lymphoma is a highly aggressive bcell nonhodgkin lymphoma and is the fastest growing human tumour. The overall cure rate for burketts lymphoma in developed countries is about 90%, but worse in lowincome countries.
Burkitts lymphoma an overview sciencedirect topics. Lymphomas are developed due to an abnormal reproduction and enlargement of the white blood cells called lymphocytes and they can be classified as hodgkins or nonhodgkins lymphoma. The experience with burkitts lymphoma is very intimidating and frightening. It is generally associated with epsteinbarr virus ebv sporadic burkitt lymphoma of breast. Burkitts lymphoma bl is the most common childhood tumor found in the tropics and subtropics. In africa, burkitt lymphoma is common in young children who also have malaria and epsteinbarr, the virus that causes infectious mononucleosis. Jun 20, 2012 understand nonhodgkin s lymphoma with this clear explanation from dr. It develops from b lymphocytes white blood cells that fight infection and usually grows very quickly. Burkitt lymphoma is a cancer of the lymphatic system, particularly b lymphocytes found in the germinal center. Burkitts lymphoma is a rare and aggressive form of nonhodgkins lymphoma. Burkitts lymphoma is a highly aggressive bcell nonhodgkin lymphoma and is the fastest.
Burkitt lymphoma belongs to the extended family of nonhodgkins lymphoma nhl which is solid tumour of lymphoid organs. Other types of burkitt lymphoma are termed as follows. Burkitt lymphoma bl is a highly malignant bcell tumor associated with epsteinbarr virus ebv infection. Burkitt s lymphoma is commonly associated with the infection of b cell lymphocytes with the epsteinbarr virus ebv and in these cases in considered to be one form of the epsteinbarr virusassociated lymphoproliferative diseases. How i treat burkitt lymphoma in adults blood american. Key features include the size and shape of the cells and how they are arranged their pattern of growth. There are three distinct clinical variants of burkitts lymphoma, each manifesting differences in epidemiology, clinical presentation, morphology, biology and genetic features. These genes are expressed only in b lymphocytes because only b cells have the necessary transcription factors for the.
Endemic burkitt s lymphoma is geographically associated with plasmodium falciparum malaria and usually arises as a tumor of the jaw. Bhs guidelines for the treatment of burkitts lymphoma. Burkitt lymphoma bl is a malignant neoplasm of lymphoid tissue, affecting the blymphocytes. Diffuse large bcell lymphoma is the most common subtype of nonhodgkin lymphoma in the west. Nonhodgkin lymphoma is a type of lymphoma characterized by the absence of reedsternberg cells. Bl was first discovered in children in certain parts of africa. The epidemic variant of burkitt s lymphoma ebl is in almost all cases associated with ebv infection. This type is similar to the endemic form of burkitt lymphoma, but occurs in other parts of the world outside africa. A role for rna viruses in the pathogenesis of burkitts. Burkitts lymphoma is a solid tumor of b lymphocytes, the lymphocytes that the immune system uses to make antibodies. A statistically significant association between infection with the arbovirus, chikv and the onset of ebl was observed in mala i, at the time of a chikv epidemic, when spacetime case clusters were also being observed. It is a tumor that is capable of doubling in just 24 hours.
Treatment options since burkitt lymphoma is very aggressive, the diagnosis of this disease requires urgent hospitalization and the immediate start of therapy. Oct 15, 2014 the exact worldwide incidence of burkitts lymphoma is not known. The world health organisation classification reports three subcategories of burkitts lymphoma. Burkitt lymphoma treatment options lymphoma research. These swollen lymph nodes are often painless, but can grow very rapidly. Burkitts lymphoma is relatively rare in western countries, but is quite common in central africa. Bl may first be noticed as a swelling of the lymph nodes glands in the head and neck. Although bl can be effectively treated in adults and children, leading to high survival rates, its ability to mask itself from the. Burkitts lymphoma is a type of blood cancer, considered rare and particularly aggressive. Molecular epidemiology of burkitts lymphoma from south america. Endemic or african is a type that usually involves the jaw and the facial bones. List the criteria for establishing a diagnosis of burkitts lymphoma and discuss the entities. How these factors complement one another in the oncogenic process, and what other. However, burkitt lymphoma is often very responsive to the currently recommended intensive chemotherapy regimens, and cure rates for this disease remain high.
In particular, we reported data regarding burkitts. Burkitts lymphoma is the most common childhood cancer in areas where malaria is holoendemic. It is characterized by deregulation of the gene encoding myc as a result of a chromosomal translocation most commonly involving the myc gene locus on chromosome 8 and the immunoglobulin heavy chain igh locus on chromosome 14 t8. Dennis burkitt, a british surgeon, while he was working in uganda. Jul 27, 2018 burkitts lymphoma is a rare and aggressive form of nonhodgkins lymphoma. The clinical and epidemiological observations pertinent to the pathogenesis of this tumor are also presented. This aggressive neoplasm is classified as a nonhodgkins lymphoma nhl and has the fastest doubling time among human tumors. Burkitt lymphoma is a cancer of the lymphatic system.
Pathophysiology and molecular aspects of diffuse large bcell. Dec 20, 2019 burkitt lymphoma bl or small noncleaved cell lymphoma is one of the highly aggressive bcell nonhodgkin lymphomas nhl that is characterized by the translocation and deregulation of the cmyc gene on chromosome 8. Burkitt lymphoma also known as small noncleaved cell lymphoma and burkitt s lymphoma has a variant termed burkitt like lymphoma or bll. Although the fact that more than 90% of burkitts lymphomas are. Pathophysiology, diagnostics and treatment burkitt s lymphoma is a highly aggressive mature bcell malignancy with a characteristic chromosomal translocation of. The pathogenesis of burkitts lymphoma sciencedirect. Burkitt lymphoma is a form of nonhodgkins lymphoma in which cancer starts in immune cells called bcells. Although the fact that more than 90% of burkitt s lymphomas are. Know the types, signs, symptoms, stages, treatment, survival. Burkitts 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. Pathophysiology, diagnostics and treatment burkitts lymphoma is a highly aggressive mature bcell malignancy with a characteristic chromosomal translocation of. A case of bcell lymphoma, unclassifiable, with features intermediate between diffuse large bcell lymphoma and burkitt lymphoma in a korean child.
Burkitt lymphoma bl, first described by denis burkitt as an obscure tumor of african children, 1 has since assumed paradigmatic status as the first human tumor whose pathogenesis could be linked both to an oncogenic virus, epsteinbarr virus ebv, and to the activation of a specific cellular oncogene, cmyc. Burkitt s lymphoma is a highly aggressive bcell nonhodgkin lymphoma and is the fastest growing human tumour. About burkitts lymphoma society is a support and advocacy nonprofit organization. Burkitt lymphoma genetic and rare diseases information. Burkitts lymphoma is humanities fastest growing cancerous tumor. Burkitt lymphoma is a form of nonhodgkin s lymphoma in which cancer starts in immune cells called bcells.
Burkitt lymphoma is named after british surgeon denis burkitt, who first identified this unusual disease in 1956 among children in africa. The sporadic form is the subentity most frequently encountered in belgium. Burkitt lymphoma bl is a highgrade bcell malignancy occurring most frequently in children in areas with holoendemic and hyperendemic malaria, and with lesser frequency in all other parts of the world 1, 2. Nonhodgkin lymphoma causes, symptoms, diagnosis, treatment, pathology. The north american form of bl is not linked to ebv.
Monitoring complete blood counts and cognitive functions is important to detect late toxicity of the applied therapies. Know the types, signs, symptoms, stages, treatment, survival rate, prognosis and pathophysiology of burkitt lymphoma. In brazil, it is the fifth cause of cancer, with more than 55,000 cases and 26,000 deaths per year. The symptoms of endemic burkitt s lymphoma include swelling and distortion of facial bones and a rapid growth of lymph nodes. Bl is a highly aggressive bcell nonhodgkin lymphoma nhl with a doubling time of 25 hours. Highgrade nhls comprise of 3 histological subtypes.
Burkits lymphoma, by dr opiro keneth linkedin slideshare. Sporadic burkitt lymphoma involves abdominal tumors. This chapter discusses the evidence that deregulation of cmyc is a critical component of the pathogenesis of burkitts lymphoma, and consideration is given to the potential pathogenetic role of epsteinbarr virus ebv. Burkitt s lymphoma is a highgrade lymphoma with characteristic small, noncleaved b cells and is endemic in equatorial africa. It is sometimes linked with the epsteinbarr virus, but many people who develop sporadic burkitt lymphoma are not infected with this virus. Treatment advances for burkitt lymphoma longdom publishing sl. Burkitt lymphoma treatment options lymphoma research foundation.
Radiotherapy is reserved for overt central nervous system disease that is resistant. Malignant small noncleaved lymphoma, a historical term, was used to describe this disease in patients presenting with a soli. It is named after dennis burkitt who first described this kind of tumour in children. Endemic burkitts lymphoma is geographically associated with plasmodium falciparum malaria and usually arises as a tumor of the jaw. Translocation a type of rearrangement of a gene called myc is a hallmark of burkitt lymphoma, making this an important finding for diagnosis. Burkitt lymphoma may spread to the central nervous system cns. Bcell lymphomas arise at distinct stages of cellular development and maturation, potentially influencing antigen ag presentation and tcell recognition. Sporadic burkitt lymphoma is also called nonafrican, or nonendemic, burkitt lymphoma. This disease may affect the jaw, central nervous system, bowel, kidneys, ovaries, or other organs. Burkitts lymphoma is a highgrade lymphoma with characteristic small, noncleaved b cells and is endemic in equatorial africa. Bl patients were significantly more likely to be chikv seropositive on first admission or to have seroconverted. Aug 12, 2012 burkitts 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. The children of age between 510 years have a higher risk of burkitt lymphoma. Bhs guidelines for the treatment of burkitts lymphoma belgian.
Certain infectious agents are associated with lymphomas, but the strength of the association varies geographically, suggesting that local environmental factors make important contributions to lymphomagenesis. Types of nonhodgkin lymphoma in children burkitt lymphoma. We seek to bring peace to the struggle by sharing experiences, information and hope to others who seek it. Burkitt lymphoma bl is a highly aggressive bcell non hodgkin lymphoma characterized by marked tumor proliferation resulting from translocation. Translocation a type of rearrangement of a gene called myc is a hallmark of. Apr 09, 2019 the children of age between 510 years have a higher risk of burkitt lymphoma.
Burkitt lymphomas are aggressive bcell cancers nonhodgkin type of lymphomas that frequently involve aggressive progression in the bone marrow and in the central nervous system. Pathophysiology and molecular aspects of diffuse large b. Understand nonhodgkins lymphoma with this clear explanation from dr. Management of adults with burkitt lymphoma hematology. The signs and symptoms of burkitt lymphoma may develop quickly as the lymphoma cells grow significantly.
In adults, burkitt lymphoma is often indistinguishable from diffuse large bcell lymphoma dlbclanother aggressive bcell lymphoma. Burkitts lymphoma society support and advocacy here to. Burkitt lymphoma also known as small noncleaved cell lymphoma and burkitts lymphoma has a variant termed burkittlike lymphoma or bll. It is named after denis parsons burkitt, a surgeon who first described the disease in 1958 while working in equatorial africa. Mar 07, 2014 types of nonhodgkin lymphoma in children nonhodgkin lymphomas nhls are most often classified by how the cancer cells look under the microscope. Its a nhl of the high grade type small noncleaved cell lymphoma, exclusively of bcell origin burkitt lymphoma is named after denis parsons burkitt, 1958,who mapped its peculiar geographic distribution. Endemic african the most common form, found mainly in central africa, where it is associated with the epstein barr virus ebv. Burkitt lymphoma is a type of nonhodgkin lymphoma, and research suggests that it is caused by a genetic mutation in which a piece of chromosome 8 is translocated to chromosome 14. Quick overview of burkitt lymphoma this section is an overview of the information on this page. Burkitts lymphoma is a rare and aggressive form of nonhodgkin lymphoma that affects as few as 300 individuals in the united states each year.
It is the most common type of burkitt lymphoma in north american and european countries. Burkitts lymphoma accounts for 60% of childhood tumors in most parts of tropical africa. Burkitt lymphoma and burkittlike lymphoma guidelines. This form of cancer can spread very rapidly, with tumors even doubling in size in less than 24 hours. The genes for making antibodies are located on chromosomes 14 the heavy h chains, 2 kappa light chains, and 22 lambda light chains. A biologic definition of burkitt s lymphoma from transcriptional and genomic profiling. Burkitts lymphoma is an aggressive form of lymphoma that affects the blymphocytes. Although bl can be effectively treated in adults and children, leading to high survival rates, its ability to mask itself from the immune system makes bl an intriguing disease to study.
1052 913 1465 641 222 901 235 1454 18 974 1382 224 1395 517 576 1185 1401 905 1487 818 1434 519 1301 1131 1031 1156 1506 205 1103 666 1517 1516 1141 1518 836 572 138 1441 616 1402 778 1379 671 888 571