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ERROR: type should be string, got "https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly93d3cubWVkc2NhcGUuY29tL2Fuc3dlcnMvMTQ0NzYwMi0xOTU4NTEvd2hhdC1hcmUtdGhlLXNpZ25zLWFuZC1zeW1wdG9tcy1vZi1idXJraXR0LWx5bXBob21hYnVya2l0dC1saWtlLWx5bXBob21hLWJsYmxs\nDrugs & Diseases > Hematology > Burkitt Lymphoma and Burkitt-like Lymphoma Q&A\nWhat are the signs and symptoms of Burkitt lymphoma/Burkitt-like lymphoma (BL/BLL)?\nAuthor: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD more...\nBurkitt and Burkitt-like lymphomas have a rapid and aggressive clinical course with frequent bone marrow and central nervous system (CNS) involvement. These are considered to be medical emergencies and require immediate diagnostic and therapeutic intervention.\nEndemic (African) Burkitt lymphoma (eBL) most commonly involves the jaw and facial bone (orbit) (> 50% of cases). Sporadic Burkitt lymphoma (sBL) most often presents as abdominal tumors with bone marrow involvement. Immunodeficiency-related Burkitt lymphoma cases usually as with nodal involvement with frequent bone marrow involvement.\nCommon findings and symptoms in patients with Burkitt lymphoma are summarized below.\nAbdominal masses, which can cause abdominal pain and distention andascites\nLoss of appetite, change in bowel habits, or both\nSigns and symptoms of acute abdomen\nIntestinal perforation\nRenal failure as a result of retroperitoneal disease and renal involvement or secondary to tumor lysis syndrome\nMandibular or maxillary mass\nMost common presentation in eBL (maxillary > mandibular)\nMay involve the orbit; jaw involvement occurs much less frequently (15-20%)\nCNS involvement\nMeningeal infiltration, with or without cranial nerve (CN) involvement (frequently, CN III and CN VII); most common mode of presentation with CNS disease\nHeadaches, visual impairment, and paraplegia from spinal involvement; these may be the initial presenting features in some cases\n\"B\" systemic symptoms\nUncommon but may be associated with other presenting symptoms (eg, fever, weight loss, night sweats, fatigue)\nBone marrow involvemen\nPainless lymphadenopathy (adults > children)\nSee Presentation for more detail.\nMolyneux EM, Rochford R, Griffin B, Newton R, Jackson G, Menon G, et al. Burkitt's lymphoma. Lancet. 2012 Mar 31. 379 (9822):1234-44. [Medline].\nSwerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016 May 19. 127 (20):2375-90. [Medline]. [Full Text].\nGong JZ, Stenzel TT, Bennett ER, et al. Burkitt lymphoma arising in organ transplant recipients: a clinicopathologic study of five cases. Am J Surg Pathol. 2003 Jun. 27(6):818-27. [Medline].\nHusain M, Grunebaum E, Naqvi A, et al. Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase. J Pediatr. 2007 Jul. 151(1):93-5. [Medline].\nFerry JA. Burkitt's lymphoma: clinicopathologic features and differential diagnosis. Oncologist. 2006 Apr. 11(4):375-83. [Medline].\nBlum KA, Lozanski G, Byrd JC. Adult Burkitt leukemia and lymphoma. Blood. 2004 Nov 15. 104(10):3009-20. [Medline].\nBurkitt DP. 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[Full Text].\nMolina-Privado I, Rodriguez-Martinez M, Rebollo P, et al. E2F1 expression is deregulated and plays an oncogenic role in sporadic Burkitt's lymphoma. Cancer Res. 2009 May 1. 69(9):4052-8. [Medline]. [Full Text].\nLove C, Sun Z, Jima D, et al. The genetic landscape of mutations in Burkitt lymphoma. Nat Genet. 2012 Dec. 44 (12):1321-5. [Medline]. [Full Text].\nRichter J, et al; ICGC MMML-Seq Project. Recurrent mutation of the ID3 gene in Burkitt lymphoma identified by integrated genome, exome and transcriptome sequencing. Nat Genet. 2012 Dec. 44 (12):1316-20. [Medline].\nDavi F, Delecluse HJ, Guiet P, et al. Burkitt-like lymphomas in AIDS patients: characterization within a series of 103 human immunodeficiency virus-associated non-Hodgkin's lymphomas. Burkitt's Lymphoma Study Group. J Clin Oncol. 1998 Dec. 16(12):3788-95. [Medline].\nCardy HA, Sharp L, Little J. Burkitt's lymphoma: a review of the epidemiology. 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Chemoimmunotherapy with hyper-CVAD plus rituximab for the treatment of adult Burkitt and Burkitt-type lymphoma or acute lymphoblastic leukemia. Cancer. 2006 Apr 1. 106(7):1569-80. [Medline].\nMagrath I, Adde M, Shad A, et al. Adults and children with small non-cleaved-cell lymphoma have a similar excellent outcome when treated with the same chemotherapy regimen. J Clin Oncol. 1996 Mar. 14(3):925-34. [Medline].\nKantarjian HM, Walters RS, Keating MJ, et al. Results of the vincristine, doxorubicin, and dexamethasone regimen in adults with standard- and high-risk acute lymphocytic leukemia. J Clin Oncol. 1990 Jun. 8(6):994-1004. [Medline].\nOstronoff M, Soussain C, Zambon E, et al. Burkitt's lymphoma in adults: a retrospective study of 46 cases. Nouv Rev Fr Hematol. 1992. 34(5):389-97. [Medline].\nHoelzer D, Ludwig WD, Thiel E, et al. Improved outcome in adult B-cell acute lymphoblastic leukemia. Blood. 1996 Jan 15. 87(2):495-508. [Medline].\nMcMaster ML, Greer JP, Greco FA, Johnson DH, Wolff SN, Hainsworth JD. Effective treatment of small-noncleaved-cell lymphoma with high-intensity, brief-duration chemotherapy. J Clin Oncol. 1991 Jun. 9(6):941-6. [Medline].\nSoussain C, Patte C, Ostronoff M, et al. Small noncleaved cell lymphoma and leukemia in adults. A retrospective study of 65 adults treated with the LMB pediatric protocols. Blood. 1995 Feb 1. 85(3):664-74. [Medline].\nTholouli E, Watt S, Lucas GS, et al. Stage IV adult sporadic Burkitt lymphoma/leukemia with complex bone marrow cytogenetics is associated with a very poor outcome. Blood. 2009 Jul 9. 114(2):485-6; author reply 486-7. [Medline].\nThomas DA, Cortes J, O'Brien S, et al. Hyper-CVAD program in Burkitt's-type adult acute lymphoblastic leukemia. J Clin Oncol. 1999 Aug. 17(8):2461-70. [Medline].\nCastillo JJ, Winer ES, Olszewski AJ. Population-based prognostic factors for survival in patients with Burkitt lymphoma: An analysis from the Surveillance, Epidemiology, and End Results database. Cancer. 2013 Jul 30. [Medline].\nArmitage JO, Weisenburger DD. New approach to classifying non-Hodgkin's lymphomas: clinical features of the major histologic subtypes. Non-Hodgkin's Lymphoma Classification Project. J Clin Oncol. 1998 Aug. 16(8):2780-95. [Medline].\nDerinkuyu BE, Boyunağa Ö, Öztunalı Ç, Tekkeşin F, Damar Ç, Alımlı AG, et al. Imaging features of Burkitt lymphoma in pediatric patients. Diagn Interv Radiol. 2016 Jan-Feb. 22 (1):95-100. [Medline]. [Full Text].\nLee AC, Li CH. Burkitt Lymphoma Presenting as Acute Pancreatitis: Report of 3 Cases and Review of the Literature. J Pediatr Hematol Oncol. 2019 Oct 29. 21(5):515-20. [Medline].\nLegault S, Couture C, Bourgault C, Bergeron S, Poirier P, Sénéchal M. Primary cardiac Burkitt-like lymphoma of the right atrium. Can J Cardiol. 2009 Mar. 25(3):163-5. [Medline]. [Full Text].\nGutierrez-Garcia L, Medina Ramos N, Garcia Rodriguez R, Barber MA, Arias MD, Garcia JA. Bilateral ovarian Burkitt's lymphoma. Eur J Gynaecol Oncol. 2009. 30(2):231-3. [Medline].\nOkudaira T, Nagasaki A, Miyagi T, Taira T, Ohshima K, Takasu N. Intensive chemotherapy for a patient with primary cutaneous diffuse large B-cell lymphoma with Burkitt-like morphology. Intern Med. 2009. 48(6):475-8. [Medline].\nBraziel RM, Arber DA, Slovak ML, et al. The Burkitt-like lymphomas: a Southwest Oncology Group study delineating phenotypic, genotypic, and clinical features. Blood. 2001 Jun 15. 97(12):3713-20. [Medline].\nKluin PM, Harris NL, Stein H, Leoncini L. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma. Swerdlow SH, Campo E, Harris NL, et al, eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008. 265-7.\nDogan A, Bagdi E, Munson P, Isaacson PG. CD10 and BCL-6 expression in paraffin sections of normal lymphoid tissue and B-cell lymphomas. Am J Surg Pathol. 2000 Jun. 24(6):846-52. [Medline].\nMcClure RF, Remstein ED, Macon WR, Dewald GW, Habermann TM, Hoering A. Adult B-cell lymphomas with burkitt-like morphology are phenotypically and genotypically heterogeneous with aggressive clinical behavior. Am J Surg Pathol. 2005 Dec. 29(12):1652-60. [Medline].\nDave SS, Fu K, Wright GW, Lam LT, et al, for the Lymphoma/Leukemia Molecular Profiling Project. Molecular diagnosis of Burkitt's lymphoma. N Engl J Med. 2006 Jun 8. 354(23):2431-42. [Medline].\nHummel M, Bentink S, Berger H, et al, for the Molecular Mechanisms in Malignant Lymphomas Network Project of the Deutsche Krebshilfe. A biologic definition of Burkitt's lymphoma from transcriptional and genomic profiling. N Engl J Med. 2006 Jun 8. 354(23):2419-30. [Medline].\nBurmeister T, Schwartz S, Horst HA, Rieder H, Gokbuget N, Hoelzer D, et al. Molecular heterogeneity of sporadic adult Burkitt-type leukemia/lymphoma as revealed by PCR and cytogenetics: correlation with morphology, immunology and clinical features. Leukemia. 2005 Aug. 19(8):1391-8. [Medline].\nKüppers R, Klein U, Hansmann ML, Rajewsky K. Cellular origin of human B-cell lymphomas. N Engl J Med. 1999 Nov 11. 341(20):1520-9. [Medline].\nGarcia CF, Weiss LM, Warnke RA. Small noncleaved cell lymphoma: an immunophenotypic study of 18 cases and comparison with large cell lymphoma. Hum Pathol. 1986 May. 17(5):454-61. [Medline].\nMagrath IT, Janus C, Edwards BK, et al. An effective therapy for both undifferentiated (including Burkitt's) lymphomas and lymphoblastic lymphomas in children and young adults. Blood. 1984 May. 63(5):1102-11. [Medline].\nSmeland S, Blystad AK, Kvaloy SO, et al. Treatment of Burkitt's/Burkitt-like lymphoma in adolescents and adults: a 20-year experience from the Norwegian Radium Hospital with the use of three successive regimens. Ann Oncol. 2004 Jul. 15(7):1072-8. [Medline].\nBishop PC, Rao VK, Wilson WH. Burkitt's lymphoma: molecular pathogenesis and treatment. Cancer Invest. 2000. 18(6):574-83. [Medline].\nTodeschini G, Bonifacio M, Tecchio C, et al. Intensive short-term chemotherapy regimen induces high remission rate (over 90%) and event-free survival both in children and adult patients with advanced sporadic Burkitt lymphoma/leukemia. Am J Hematol. 2012 Jan. 87(1):22-5. [Medline].\nHill QA, Owen RG. CNS prophylaxis in lymphoma: who to target and what therapy to use. Blood Rev. 2006 Nov. 20(6):319-32. [Medline].\nMead GM, Barrans SL, Qian W, et al, for the UK National Cancer Research Institute Lymphoma Clinical Studies Group and the Australasian Leukaemia and Lymphoma Group. A prospective clinicopathologic study of dose-modified CODOX-M/IVAC in patients with sporadic Burkitt lymphoma defined using cytogenetic and immunophenotypic criteria (MRC/NCRI LY10 trial). Blood. 2008 Sep 15. 112(6):2248-60. [Medline]. [Full Text].\nLee EJ, Petroni GR, Schiffer CA, et al. 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. J Clin Oncol. 2001 Oct 15. 19(20):4014-22. [Medline].\nRizzieri DA, Johnson JL, Niedzwiecki D, et al. Intensive chemotherapy with and without cranial radiation for Burkitt leukemia and lymphoma: final results of Cancer and Leukemia Group B Study 9251. Cancer. 2004 Apr 1. 100(7):1438-48. [Medline].\nOriol A, Ribera JM, Bergua J, et al. High-dose chemotherapy and immunotherapy in adult Burkitt lymphoma: comparison of results in human immunodeficiency virus-infected and noninfected patients. Cancer. 2008 Jul 1. 113(1):117-25. [Medline].\nDunleavy K, Pittaluga S, Janik J, et al. Novel treatment of Burkitt lymphoma with dose-adjusted EPOCH-rituximab: Preliminary results showing excellent outcome (abstract). Blood. 2006. 108:774A.\nSweetenham JW, Pearce R, Taghipour G, Blaise D, Gisselbrecht C, Goldstone AH. Adult Burkitt's and Burkitt-like non-Hodgkin's lymphoma--outcome for patients treated with high-dose therapy and autologous stem-cell transplantation in first remission or at relapse... J Clin Oncol. 1996 Sep. 14(9):2465-72. [Medline].\nSong KW, Barnett MJ, Gascoyne RD, et al. Haematopoietic stem cell transplantation as primary therapy of sporadic adult Burkitt lymphoma. Br J Haematol. 2006 Jun. 133(6):634-7. [Medline].\nPhilip T, Biron P. High-dose chemotherapy and autologous bone marrow transplantation in diffuse intermediate- and high-grade non-Hodgkin lymphoma. Crit Rev Oncol Hematol. 2002 Feb. 41(2):213-23. [Medline].\nNademanee A, Molina A, O'Donnell MR, et al. Results of high-dose therapy and autologous bone marrow/stem cell transplantation during remission in poor-risk intermediate- and high-grade lymphoma: international index high and high-intermediate risk group. Blood. 1997 Nov 15. 90(10):3844-52. [Medline].\nPhilip T, Armitage JO, Spitzer G, et al. High-dose therapy and autologous bone marrow transplantation after failure of conventional chemotherapy in adults with intermediate-grade or high-grade non-Hodgkin's lymphoma. N Engl J Med. Jun 11 1987. 316(24):1493-8. [Medline].\nGrigg AP, Seymour JF. Graft versus Burkitt's lymphoma effect after allogeneic marrow transplantation. Leuk Lymphoma. 2002 Apr. 43(4):889-92. [Medline].\nPeniket AJ, Ruiz de Elvira MC, Taghipour G, et al, for the European Bone Marrow Transplantation (EBMT) Lymphoma Registry. An EBMT registry matched study of allogeneic stem cell transplants for lymphoma: allogeneic transplantation is associated with a lower relapse rate but a higher procedure-related mortality rate than autologous transplantation. Bone Marrow Transplant. 2003 Apr. 31(8):667-78. [Medline].\nWeinthal JA, Goldman SC, Lenarsky C. Successful treatment of relapsed Burkitt's lymphoma using unrelated cord blood transplantation as consolidation therapy. Bone Marrow Transplant. 2000 Jun. 25(12):1311-3. [Medline].\nNational Cancer Institute sponsored study of classifications of non-Hodgkin's lymphomas: summary and description of a working formulation for clinical usage. The Non-Hodgkin's Lymphoma Pathologic Classification Project. Cancer. 1982 May 15. 49 (10):2112-35. [Medline].\nHarris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994 Sep 1. 84 (5):1361-92. [Medline]. [Full Text].\n[Guideline] NCCN Clinical Practice Guidelines in Oncology. B-Cell Lymphomas. National Comprehensive Cancer Network. Available at https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf. Version 6.2019 — November 26, 2019; Accessed: December 2, 2019.\nCastillo JJ, Winer ES, Olszewski AJ. Population-based prognostic factors for survival in patients with Burkitt lymphoma: an analysis from the Surveillance, Epidemiology, and End Results database. Cancer. 2013 Oct 15. 119 (20):3672-9. [Medline].\nBoerma EG, van Imhoff GW, Appel IM, Veeger NJ, Kluin PM, Kluin-Nelemans JC. Gender and age-related differences in Burkitt lymphoma--epidemiological and clinical data from The Netherlands. Eur J Cancer. 2004 Dec. 40(18):2781-7. [Medline].\nDunleavy K, Pittaluga S, Shovlin M, Steinberg SM, Cole D, Grant C, et al. Low-intensity therapy in adults with Burkitt's lymphoma. N Engl J Med. 2013 Nov 14. 369(20):1915-25. [Medline].\nOsterweil N. Burkitt's Yields to Longer-Duration, Lower-Dose Chemo. Medscape Medical News. Available at http://www.medscape.com/viewarticle/814558. Accessed: November 26, 2013.\nComputed tomography scan in a patient with a large, left-sided axillary mass from which a biopsy was obtained. Biopsy findings were consistent with small noncleaved cell non-Hodgkin lymphoma.\nPostchemotherapy computed tomography scan in a patient diagnosed with small noncleaved cell lymphoma (SNCCL) (same patient as in previous image). This image shows regression of a left axillary mass.\nCoronal magnetic resonance imaging (MRI) section in a patient with large neck mass (same patient as in previous image). Biopsy findings showed Burkitt-like non-Hodgkin lymphoma (NHL). MRI was performed to assess for cord involvement.\nSagittal magnetic resonance imaging (MRI) section of the neck area showing a large mass invading the cervical spine with epidural encroachment (same patient as in the previous image). MRI was performed to rule out cord compression. The first image shows the gallium scan of this patient that correlates with the site of the tumor.\nRight-sided pleural effusion in a patient with small noncleaved cell lymphoma (SNCCL) non-Hodgkin lymphoma.\nHematoxylin and eosin (H&E) stain. Sheets of monotonous-appearing lymphoid cells with one or more prominent nucleoli and an area of pale staining resulting from the presence of benign macrophages reveal a starry sky pattern.\nThe 2-dimensional flow cytometry demonstrates the highlighted cells to be CD5 negative and CD23 negative as well as lambda negative. Small noncleaved cell lymphoma (SNCCL) cells are typically CD19+, CD20+, CD22+, and CD10+.\nAli H Kanbar, MD Medical Oncologist/Hematologist, Dayton Cancer Center\nAli H Kanbar, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, American Society of Clinical Oncology\nCoauthor(s)\nRonald A Sacher, MBBCh, FRCPC, DTM&H Professor of Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center\nRonald A Sacher, MBBCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada\nEmmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University\nEmmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences\nPatturajah Anbumani, MD, MBBS, MS, MCh Associate Medical Director, Best Medical Care; Former Associate Medical Director, Jeanes Hospital, Temple University Health System; Former Adjunct Clinical Assistant Professor, New York College of Osteopathic Medicine; Former Clinical Assistant Professor, Department of Medicine, State University of New York-Downstate\nPatturajah Anbumani, MD, MBBS, MS, MCh is a member of the following medical societies: American College of Physicians, American Medical Association, and American Medical Women’s Association\nSamer A Bleibel, MD Staff Physician, Department of Internal Medicine, Wayne State University School of Medicine, St John's Hospital and Medical Centers\nSamer A Bleibel, MD is a member of the following medical societies: American College of Physicians\nAsher A Chanan-Khan, MD Assistant Professor, Department of Medicine, Division of Lymphoma and Bone Marrow Transplantation, Roswell Park Cancer Institute, State University of New York at Buffalo\nAsher A Chanan-Khan, MD is a member of the following medical societies: American College of Physicians, American Medical Association, and American Society of Hematology\nHanxian Huang, MD, PhD Staff Physician, Department of Internal Medicine, Leesburg Regional Medical Center, The Villages Regional Hospital\nHanxian Huang, MD, PhD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine\nAnand B Karnad, MBBS Program Director, Fellowship Programs in Hematology-Oncology, Professor of Medicine, Division of Medical Oncology, Department of Medicine, University of Texas Health Sciences Center, San Antonio\nAnand B Karnad, MBBS is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Osler Society, American Society of Hematology, Assocation of Subspecialty Professors, and Massachusetts Medical Society\nOlga Kozyreva, MD Attending Physician, Division of Hematology-Oncology, St Elizabeth's Medical Center; Assistant Professor, Tufts University School of Medicine\nSarah K May, MD Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC\nfrom Memorial Sloan-Kettering - Philip Schulman, MD Chief, Medical Oncology, Department of Medicine, Memorial Sloan-Kettering Cancer Center; Clinical Professor, Department of Medicine, New York University School of Medicine\nPhilip Schulman, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Society of Hematology, and Medical Society of the State of New York\nKaren Seiter, MD Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College\nKaren Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology\nDisclosure: Novartis Honoraria Speaking and teaching; Schering Honoraria Speaking and teaching; Cephalon Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching\nFrancisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference\nDisclosure: Medscape Salary Employment\nYubao Wang, MD, PhD Fellow, Division of Hematology/Medical Oncology, University of Texas Health Science Center, San Antonio\nYubao Wang, MD, PhD is a member of the following medical societies: American College of Physicians, American Society of Clinical Oncology, and American Society of Hematology"