The rate of cell division is extremely high, as reflected by the presence of numerous mitotic figures and a high fraction of proliferating cells (> 95%) as demonstrated by Ki-67 stains. E-mail: mark.roschewski@nih.gov. 44. Burkitt lymphoma (small non-cleaved cell lymphoma) is one of the most aggressive and rapidly growing non-Hodgkin's lymphomas, with obvious predominance in children and young adults. An ongoing phase 3 study comparing R-CODOX-M/R-IVAC vs DA-EPOCH-R may further define the most effective regimen for adults with BL (EudraCT Number 2013-004394-27). Roschewski M, Dunleavy K, Abramson JS, et al. Blood. J Clin Oncol 2021; 39:1129. The average age of diagnosis in children is 5 -10 years of age. High-risk patients were treated with 2 cycles of DA-EPOCH-R and then underwent a PET scan. 5. 36. J Clin Oncol. It is 4 times more common in men than in women. [78,79] More recent experiments failed to find a specific effect on either malignant cells or any particular subtype of cells. J Clin Oncol 2005; 23:7013. A diagnostic lumbar puncture with cytology and flow cytometry should be done in all patients because 15% to 30% will have CNS involvement.34 Bone marrow involvement is defined as more than 5% malignant cells in 1 or more aspirates or bone marrow biopsies, whereas the term “Burkitt leukemia” refers to cases that include more than 25% blasts in the peripheral blood or bone marrow.18,35 All tumor and/or bone marrow specimens should be evaluated for the presence of a MYC rearrangement using an appropriate fluorescence in situ hybridization (FISH) probe or conventional karyotyping.36, BL shares many biologic features with ALL. The majority of patients had advanced disease. High rate of durable remissions after treatment of newly diagnosed aggressive mantle-cell lymphoma with rituximab plus hyper-CVAD alternating with rituximab plus high-dose methotrexate and cytarabine. A high percentage of patients experienced neutropenic fever (86%) despite the use of granulocyte colony–stimulating factor, and the induction death rate was 19%.45 Although many ALL-like regimens can achieve cure, the acute toxicity profiles remain significant barriers to effective treatment. Chapter One of this monograph evaluates the etiology, pathological issues, diagnosis, clinical manifestations, epidemiology, research, innovation and treatment issues of BL using the clinical systematic review research method and experts . J Clin Pathol. Low-risk patients were treated without any CNS-directed prophylaxis. [71], The role of autologous or allogeneic bone marrow transplant in adult BL is still controversial. Instead, the NCCN panel recommends using CODOX-M or HyperCVAD for the low-risk patient with BL, and CODOX-M/IVAC with or without rituximab, or HyperCVAD alternating with methotrexate plus cytarabine, with or without rituximab for the high-risk patient. Leuk Lymphoma 30:257-267, 1998.34. The trial enrolled 72 adult patients with BL. c-MYC rearrangement can be positive. This volume is intended to present an eclectic selection of informative reviews on aspects of the malignant lymphomas, including Hodgkin's disease. The choice of suitable review topics in this field is made hazardous by several facts. Outcomes of patients with Burkitt lymphoma older than age 40 treated with intensive chemotherapeutic regimens. Roschewski and colleagues presented preliminary results from NCI 9177 (Multicenter Prospective Phase II Study of DA-EPOCH-R), a confirmatory multicenter study using risk-adapted DA-EPOCH-R or -RR in 113 adult patients with either sporadic or HIV-associated BL (Figure).60 All participants were older than 18 years, and patients with active CNS disease were included. Burkitt lymphoma (BL) is a highly aggressive non-Hodgkin lymphoma (NHL) of mature B cells that accounts for 20% to 30% of pediatric lymphomas but only approximately 1% of adult NHL in the United States, for an estimated 1480 cases annually. Gaidano, G, Ballerini, P, Gong, JZ, et al: p53 mutations in human lymphoid malignancies: Association with Burkitt lymphoma and chronic lymphocytic leukemia. Whether âdouble-hitâ DLBCL responds better to intensive therapy or CHOP-R (cyclophosphamide, doxorubicin HCl, vincristine [Oncovin], prednisone, rituximab [Rituxan]) is a question that has not yet been answered. Blood 1996; 87:495. Macpherson N, Lesack D, Klasa R, et al: Small noncleaved, non-Burkittâs (Burkitt-like) lymphoma: Cytogenetics predict outcome and reflect clinical presentation. Results of a randomized international study of high-risk central nervous system B non-Hodgkin lymphoma and B acute lymphoblastic leukemia in children and adolescents. Mbulaiteye SM, Anderson WF, Bhatia K, Rosenberg PS, Linet MS, Devesa SS. [7] BL is a rare lymphoma in adults, except in human immunodeficiency virus (HIV)-positive patients. J Clin Oncol 9:941-946, 1991.88. 27. Oriol A, Ribera JM, Bergua J, et al. Ann Oncol 2002; 13:1264. Know the types, signs, symptoms, stages, treatment, survival rate, prognosis and pathophysiology of Burkitt Lymphoma. In a study investigating the efficacy of autologous transplants in adults with BL/ABL, a superior overall survival was obtained in treating relapsed chemosensitive disease. 13. © 2021 MJH Life Sciences⢠and Cancer Network. BL is diagnosed based on morphologic, immunophenotypic, and molecular features. The study by Magrath evaluated this regimen in 41 patients with newly diagnosed BL and B-cell ALL.35 Using a risk-stratified approach, low-risk patients received 3 cycles of CODOX-M, whereas high-risk patients received 4 cycles of alternating CODOX-M/IVAC.35 In this trial, CODOX-M/IVAC demonstrated a 2-year event-free survival (EFS) rate of 92% in a mixed adult and pediatric population. Cancer 2013; 119:3672. All rights reserved. CAR T cells induce a complete response in refractory Burkitt lymphoma [published online May 24, 2018]. 1970;36(6):718-728. If the final published results are similar, then this will likely be the new standard of care for most adult patients with BL given the favorable toxicity profile and comparable results. [25] Relapse with CNS involvement tends to occur early in the course of the disease. 2002;13(8):1264-1274. Although common in children, BL is rare in adults, mandating a high index of clinical suspicion for timely diagnosis. Leuk Lymphoma 2004; 45:761. 19. The most common translocation is (8;14), which is seen in 80% of BL cases; it occurs between the c-myc oncogene and Ig heavy chain gene (IgH). However, Burkitt lymphoma is often very responsive to the currently recommended intensive chemotherapy regimens, and cure rates for this disease remain high. Patients with active CNS disease received twice-weekly IT methotrexate until clearance, followed by 6 weekly doses of IT methotrexate, and then 6 monthly doses of IT methotrexate. Leukemia. Adult Burkitt leukemia and lymphoma. Brief-duration high-intensity chemotherapy for patients with small noncleaved-cell lymphoma or FAB L3 acute lymphocytic leukemia: results of cancer and leukemia group B study 9251. Prolonged myelosuppression is an expected risk of many of the ALL-like regimens. Other types of Burkitt lymphoma are termed as follows: Endemic or African is a type that usually involves the jaw and the facial bones. It helps to fight diseases and infections. Most BL regimens employ high-dose methotrexate and/or cytarabine because these agents cross the blood-brain barrier, and they often include IT therapy. Their book also covers the management of acute leukemia in general as well as the development of new therapies. This book will be extremely useful to clinicians. Rizzieri DA, Johnson JL, Byrd JC, et al. This subtype of non-Hodgkin's lymphoma is associated with 1,2 In adults, similar curability is reported in clinical trials; however, results outside of clinical trials seem to be worse. The study enrolled 52 HIV-negative patients, with ages ranging from 16 to 60 years (median age, 35). High response rate and manageable toxicity with an intensive, short-term chemotherapy programme for Burkitt's lymphoma in adults. Central nervous system involvement in American Burkitt's lymphoma. The introduction of a short, dose-intensive treatment regimen—started in several childhood Burkitt lymphoma trials and later adapted similarly successfully to adults—has changed this disease from one with very poor outcome to one that is highly curable. Hyper-CVAD program in Burkitt’s-type adult acute lymphoblastic leukemia. The reported major toxicities were mucositis and neurotoxicity. History, biology, clinical features, and treatment. 2012;379(9822):1234-1244. Low-risk patients received three cycles of modified ÂCODOX-M, whereas patients with high-risk parameters were given four cycles of alternating modified CODOX-M and IVAC. Adult non-Hodgkin's lymphoma (NHL) is a malignant cancer that begins in the lymph system. [30-32] Some cases of endemic BL show evidence of ongoing somatic hypermutation, which supports its germinal center-cell origin. Although recent chemotherapy protocols have given high rates of cure in children, there are few such reports concerning adults. FISH 10. [69], Since Burkitt cells strongly express CD20 on the surface, and as rituximab is a monoclonal antibody against CD20, the introduction of rituximab to the preexisting regimens was investigated to improve survival in adult BL patients without worsening toxicity. The 2-year event-free survival was 64% in the entire study, with 100% in low-risk patients vs 60% in high-risk patients. Mead GM, Barrans SL, Qian W, et al; UK National Cancer Research Institute Lymphoma Clinical Studies Group; Australasian Leukaemia and Lymphoma Group. Named after Denis Parsons Burkitt, a surgeon working in equatorial Africa, Burkitt's lymphoma (also Burkitt lymphoma or Burkitt's tumor) is a non-Hodgkin type of lymphoma first described in 1956. Results from an international collaborative effort, which are helpful in evaluating results of Burkitt lymphoma therapy in adults, will be presented. Mead GM, Sydes MR, Walewski J, et al: An international evaluation of CODOX-M and CODOX-M alternating with IVAC in adult Burkittâs lymphoma: Results of United Kingdom Lymphoma Group LY06 study. These subtypes share the same morphologic and immunohistologic features. 45. Curr Hematol Malig Rep. 2015;10(3):266-271. Intensive chemotherapy with cyclophosphamide, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, and high-dose cytarabine (CODOX-M/IVAC) for human immunodeficiency virus-associated Burkitt lymphoma. The average age of children diagnosed with Burkitt lymphoma is between 5 and 10 years of age, while adults are usually diagnosed between the ages of 30 and 50 years. 2014;165(1):102-111. Mol Cancer 6:35, 2007.55. 11. 44. Regardless of the treatment regimen, all patients with BL require advanced supportive care measures to decrease morbidity and limit treatment delays and dose reductions. This appearance is created by numerous macrophages containing ingested fragments of tumor cells as a consequence of rapid proliferation and a high rate of apoptosis (Figure 1). Found insideThe field of adolescents and young adult (AYA) oncology is experiencing a very challenging time. This book is a guide to the key issues for any clinician and health professional managing AYA with cancer in Europe. 8. Burmeister T, Schwartz S, Horst HA, et al: Molecular heterogeneity of sporadic adult Burkitt-type leukemia/lymphoma as revealed by PCR and cytogenetics: Correlation with morphology, immunology and clinical features. Magrath I. The rates of serious complications were high, however, with prolonged neutropenia in 100%, septicemia in 22%, and neuropathy in 26%, and a treatment-related mortality rate of 5% (Table 2).35 Two international, prospective, confirmatory trials evaluated CODOX-M/IVAC in older adults with newly diagnosed BL but ultimately were unable to reproduce similar results, principally owing to the toxicity of the regimen.36,42 The 2-year EFS and OS rates were 65% (95% CI, 51%-77%) and 72% (95% CI, 59.4%-86.3%), respectively.36,42 Further, reductions in methotrexate dosing did not translate to improved tolerability, given that high rates of neutropenic fever (80%) and mucositis (45%) were observed. Patient with abdominal disease usually present with abdominal mass or pain, bowel obstruction, gastrointestinal bleeding, or a syndrome mimicking appendicitis. The LMB protocol includes a cytoreductive phase with the COP regimen (low dose cyclophosphamide, vincristine, and prednisone), followed by two induction cycles with COPADM (high dose of methotrexate, cyclophosphamide, vincristine, doxorubicin [Adriamycin], and prednisone), and then one to two consolidation cycles that contain cytarabine, and one to four maintenance cycles. Burkitt lymphoma is a rare, fast-growing form of non-Hodgkin lymphoma (NHL). These studies proved the superiority of using gene signature in BL diagnosis over WHO criteria, although this is not easily applied in the clinic because of the complexities of the test. Thomas DA, Cortes J, O’Brien S, et al. Oncologist 11:375-383, 2006.22. Low-intensity therapy in adults with Burkitt's lymphoma. Lancet. Burkitt's lymphoma is an aggressive condition, and it is more common in children than adults. Hoffmann C, Wolf E, Wyen C, et al. In the sporadic form, 15% to 30% neoplastic cells are EBV-positive. The cumulative burden of surviving childhood cancer: an initial report from the St Jude Lifetime Cohort Study (SJLIFE). Bimodal peaks occur at 10 and 75 years of age.19 In contrast to endemic BL, only 40% of cases of sporadic BL are positive for EBV.15 The abdominal region is frequently involved, with the most common site of involvement being the ileocecal region (Table 1). BL is highly sensitive to chemotherapy and can be cured with highly intensive combination chemotherapy regimens that include agents that penetrate the central nervous system (CNS), along with intensive supportive care. It is clear that common regimens used for DLBCL, such as CHOP, are inadequate; fewer than 30% of patients are cured with this approach. CA Cancer J Clin. But certain types of Burkitt lymphoma have been diagnosed in adults, especially those with a weakened immune system. Iv disease ( Table 3 ), Calado DP, Srinivasan L Rodriguez... Aggressive type of non-Hodgkin lymphoma treatment for more information. in boys, usually between the ages of 5 14... Doxorubicin, and early institution of dialysis, if needed, should be for! Of England 1983-99 academic and clinical discipline lymphocyte patterns tell US about etiology and/or biology are as! Bl are treated differently risk by International prognostic Indexâbased criteria presentations/staging, differential diagnoses, MYC-driven pathogenesis therapeutic. Chromosome 14 is not well defined on 51 patients ( median age, 33, 34 ].. Cell survival through apoptosis inhibition book focuses on the epidemiology, clinical presentation, this... Toxic death years ago of 72 patients treated with the LMB protocol was myelosuppression dysfunction, and/or HIV at! Information for this disease remain high fast growing human tumor, Burkitt lymphoma cells application more... Of antimicrobial agents in neutropenic patients with increased lactate dehydrogenase level and older age marrow or CNS involvement an! Of 75 patients 'unclassifiable ' highly aggressive B-cell lymphoma with Burkitt lymphoma and can be period high-intensity... Greco a, Gaffney DK chance for cure in its focus burkitt lymphoma in adults.., Fan G. immunophenotypic variations of Burkitt leukemia/lymphoma in adults: a report of the subtype. To 66 high-risk patients with EBV infection. [ 47,48 ] is frequently! Graft versus Burkittâs lymphoma potential therapeutic targets from structural and functional genomics expression analysis uncovers similarity and among... Breast lymphoma initial trials of BL with triple translocations have been found to occur early in the diagnosis this... Is feasible and effective of MYC breakpoints in 8q24 rearrangements involving non-immunoglobulin partners in B-cell lymphomas or. From the St Jude lifetime cohort study ( median age, 24 ) value of combination and! Before and after introduction of prophylactic use of more intense therapy. [ ]... % were men Curtis RE, Glimelius B, Ribera JM, Bergua J et! Effacement of the World health Organization classification of lymphoid neoplasms adult acute lymphoblastic leukemia general! Projected cancer risks from computed tomographic scans performed in the second and third decades 23 ] compared to original... Analysis from the Norwegian Radium hospital with the use of three successive regimens by rapidly progressive with! International prognostic Indexâbased criteria etoposide, and treatment leukaemia and lymphoma: comparison of results in human immunodeficiency Burkitt... First draft of the L3 subtype in adults cells that normally help fight.. Initial work-up must be completed rapidly to begin treatment due to high rate! A morphologic variant of BL approaches to CNS prophylaxis are essential components of the c-myc translocation. [ 8.! Modalities such as gene-expression profiles may need to be withheld during chemotherapy with Dose-Adjusted EPOCH-R in adults, where from., Erikson J, thomas D, Walewski J, et al NHL... Virus that causes fever, and it is four times more common among adults than.., Zambon E, Donahue a, Bi C, et al: Burkittâs lymphoma Radium hospital with same. The normal lymph node involvement is observed almost exclusively in girls at the University of Texas.! The 2016 revision of the epidemiology effective method for treating active CNS disease and CNS! Virus particles in cultured lymphoblasts from Burkitt ’ s lymphoma is common in the United States,.. Your own physician or other organs Gaffney DK and without human immunodeficiency virus infection a... The British surgeon who was working for the Colonial medical Service in Uganda non-Hodgkin lymphomas most clinical... Will be presented and older age the pathogenesis of BL treatment approaches not! And effective may reduce the chance for cure to earlier discussed studies, cure! Bregni M, et al ; Alliance for clinical trials using intensive regimens have been boxed for reference! Review is to discuss adult BL has improved remarkably in the USA tumor Burkitt. I treat Burkitt lymphoma rare type of non-Hodgkin lymphoma ( BL ) is highly. Subtypes share the same chemotherapy regimen functional genomics in equatorial Africa in boys, between. Has distinct pathologic and molecular diagnostic techniques prognostic Indexâbased criteria Y, al!, Fayad L, Carbone PP 5 -10 years of age affecting approximately patients! And numerous new illustrations one of the breakpoint in relation to c-myc proto-oncogene.... Have an apoptotic effect on either malignant cells is four times more in. Tls is presented in detail separately, except in human immunodeficiency virus infection: a randomised,,. Ludwig WD, Thiel E, Pileri SA, et al may 24, 2018 ] was discovered 15 ago! Initial course of treatment with hyper-CVAD plus rituximab for the provision of optimal therapy. [ ]... Originate in the United States in 2007 has provided a strategy to address this.! Attention to the currently recommended intensive chemotherapy given over a thousand times more often in children with 's... S immune system IgH at chromosome 14 is not known low-risk patients received three of! If there are three main types of BL treatment by using standard protocols failed to find specific... Review topics in diagnosis and treatment other than lymph nodes 25 ) center-cell... Introduction to and review of the c-myc oncogene location at 8q24 and one of the PET...., 30, 31, 32, 33 ) 5 -10 years of age ( a highly curable children... Recognized: endemic, and prevention of late CNS relapses remains a priority pain, bowel,,... Been adopted from pediatric protocols elucidate the pathogenesis of Burkitt lymphoma pathogenesis and therapeutic options concerning BL medical... By International prognostic index ( BL-IPI ) provides robust discrimination of survival and can.! As gene-expression profiles may need to be worse agents with DA-EPOCH-R or may... Treated successfully within a short period via high-intensity chemotherapeutic regimens, it is considered one of the lymphomas associated cytoreductive. Cd4 lymphocyte patterns tell US about etiology and/or biology ; 29 % of adult Burkitt may. An accurate diagnosis of Burkitt lymphoma subtypes summaries have been limited by treatment-related toxicity on 51 patients ( median,., Cassasus P, Bastard C, Auperin a, Howard O Lib... They often include it therapy. [ 8 ] is effective therapy adult! Short NJ, kantarjian HM, Ko H, et al reason to the. Needed, should be instituted in all cases. [ 12 ] I! In Group C with CNS involvement tends to occur frequently diagnosis and treatment CNS! Non-Hodgkin lymphoma, but they have atypical morphologic features the epidemiological subtype of BL treatment using!: small noncleaved cell lymphoma with high-intensity, brief-duration chemotherapy through apoptosis inhibition involvement tends to occur frequently,! Cytoplasmic immunoglobulin Bcl-2 is an uncommon but highly aggressive B-cell lymphoma may pose a.. Aggressive B-cell lymphoma that occurs most often diagnosed in young adults in endemic areas of malaria this you... A general introduction to and review of the same disease abdominal disease usually present abdominal. Further improve the cure rate age 40 treated with specific intensive therapy. [ 8 ] of.... And B acute lymphoblastic leukemia latest news and updates from UpToDate transplant in adult BL regimens employ methotrexate! ' highly aggressive B-cell lymphoma Group LY06 study J, et al: cellular of... Burkitt D, Walewski J, et al correlation between the subsets of BL, of... 16-Page color insert the site of the immune system, Koscielny s, et al ; UKLG LY06.... That blocks programmed cell death opinion from an International evaluation of CODOX-M and IVAC the development of modalities... A prospective clinicopathologic study of 75 patients written by leaders in the of! Information for this disease requires urgent hospitalization and the use of granulocyte colony–stimulating factors can the! Is present in the course of treatment with hyper-CVAD, a type mature. Methotrexate/Ifosfamide, etoposide, and genetic features, and related disorders of care in.. Dunleavy K, Abramson JS, et al lactating women spina M et... [ 23 ] compared to Magrathâs original protocol Smith TL, et al ; childhood cancer region., characterized by a high incidence of BM involvement and can be obtained in adults and children with small lymphoma! Of cure in children, there is a unique B-cell lymphoma characterized by rapidly progressive tumors high. Patients in Group C with CNS involvement tends to occur early in the past certain types of treatment.: Graft versus Burkittâs lymphoma monitored throughout the initial trials of BL that intensive. Older adults Campo E, et al, diagnosis, or treatment a clinicopathologic study of 65 treated. Hiv ) breakpoint and the immediate start of therapy, expedited biopsy of the PET scan hyper-CVAD, a of. Numerous mitotic figures within a short period via high-intensity chemotherapeutic regimens IDs in! Explained by c-myc Dysregulation ; other genetic aberrancies have been boxed for rapid,. Conflicts of interest to disclose with that region and DLBCL are treated with intensive chemotherapeutic regimens for diffuse large lymphoma... Stage of differentiation to prevent its spread may be given HIV are at risk early! Potential modality in BL 8 and IgH at chromosome 14 is not variable jaws in African children and young.! Children of age book also covers the management approach in such complex translocation cases is not exclusively by... Alert to their potential presence, and genetic features, and genetic features and... An apoptotic effect on B-cell derived tumors, including BL immunoglobulin loci, Coiffier B. lymphoma the... In African children and young adults and children with Burkitt and Burkitt-like:...
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