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07-SEPTEMBER-2008 03:17:44 - Burkitt's lymphoma Burkitt's lymphoma Classification and external resources Burkitt lymphoma, touch prep, Wright stain ICD-10 C83.7 ICD-9 200.2 ICD-O: 9687/3 OMIM 113970 DiseasesDB 1784 eMedicine med/256 MeSH D002051 Burkitt lymphoma or Burkitt's tumor, or Malignant lymphoma, Burkitt's type is a cancer of the lymphatic system in particular, B lymphocytes. It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1956 while working in equatorial Africa.12 Almost by definition, Burkitt lymphoma are associated with c-myc gene translocation. The most common variant is t8;14q24;q32 while rarer variants include t2;8p12;q24 and t8;22q24;q11. A three-way translocation, t8;14;18, has also been identified.3 It is curable. Contents 1 Classification 2 Microscopy 3 Malignant B cell characteristics 4 Treatment 5 Epidemiology 6 References 7 External links Classification Currently Burkitt's lymphoma can be divided into three main clinical variants: the endemic, the sporadic and the immunodeficiency-associated variants. Seven-year-old Nigerian boy with a several month history of jaw swelling which had been treated with antibiotics. The tumor was ulcerated and draining. Seven-year-old Nigerian boy with a several month history of jaw swelling which had been treated with antibiotics. The tumor was ulcerated and draining. Picture of a mouth of a patient with Burkitt's lymphoma showing disruption of teeth and partial obstruction of airway. Picture of a mouth of a patient with Burkitt's lymphoma showing disruption of teeth and partial obstruction of airway. The endemic variant occurs in equatorial Africa. It is the most common malignancy of children in this area. Children affected with the disease often also had chronic malaria which is believed to have reduced resistance to the Epstein-Barr virus and allowed it to take hold. The disease characteristically involves the jaw or other facial bone, distal ileum, cecum, ovaries, kidney or the breast. The sporadic type of Burkitt lymphoma also known as non-African is another form of non-Hodgkin lymphoma found outside of Africa. The tumor cells have a similar appearance to the cancer cells of classical African or endemic Burkitt lymphoma. Again it is believed that impaired immunity provides an opening for development of the Epstein-Barr virus. Non-Hodgkins, which includes Burkitt's, accounts for 30-50% of childhood lymphoma. Jaw is less commonly involved, comparing with the endemic variant. Ileo-cecal region is the common site of involvement. Immunodeficiency-associated Burkitt lymphoma is usually associated with HIV infection or occurs in the setting of post-transplant patients who are taking immunosuppressive drugs. Actually, Burkitt lymphoma can be the initial manifestation of AIDS. By morphology i.e. microscopic appearance or immunophenotype, it is almost impossible to differentiate these three clinical variants. Immunodeficiency-associated Burkitt lymphoma may demonstrate more plasmacytic appearance or more pleomorphism, but these features are not specific. Microscopy Burkitt's lymphoma, standard HE stain. Burkitt's lymphoma, standard HE stain. Consists of sheets of monotonous i.e. similar in size and morphology population of medium size lymphoid cells with high proliferative activity and apoptotic activity. The starry sky appearance seen under low power is due to scattered tingible-bodies laden macrophages macrophages containing dead body of apoptotic tumor cells. The old descriptive term of small non-cleaved cell is misleading. The tumor cells are mostly medium in size i.e. tumor nuclei size similar to that of histiocytes or endothelial cells. Small non-cleaved cells are compared to large non-cleaved cells of normal germinal center lymphocytes. Tumor cells possess small amount of basophilic cytoplasm. The cellular outline usually appears squared off. Malignant B cell characteristics Normal B cells possess rearranged immunoglobulin heavy IgH and light chain genes, unlike most T-cells and other cells of the body in which the genes are germline. Each isolated B-cell possesses a unique IgH gene rearrangement, reminiscent of the fingerprint of a person. Since Burkitt lymphoma and other B-cell lymphomas are a clonal proliferative process, all tumor cells from one patient are supposed to possess identical IgH genes. When the DNA of tumor cells is analyzed using electrophoresis, a clonal band can be demonstrated since identical IgH genes will move to the same position. On the contrary, when a normal or reactive lymph node is analyzed using the same technique, a smear rather than a distinct band will be seen. This technique is useful since sometimes benign reactive processes e.g. infectious mononucleosis and malignant lymphoma can be difficult to distinguish. Treatment Effect of the chemotherapy, as with all cancers, depends on the time of diagnosis. With faster growing cancers, such as this one, the cancer actually responds faster than with slower growing cancers. This rapid response to chemotherapy can be hazardous to patient as a phenomenon called tumor lysis syndrome could occur. Close monitoring of patient and adequate hydration is essential during the process. Chemotherapy cyclophosphamide doxorubicin vincristine methotrexate cytarabine ifosfamide etoposide rituximab4 Other treatments are immunotherapy, bone marrow transplants, surgery to remove the tumor, and radiotherapy. Epidemiology Of all cancers involving the same class of blood cell, 2% of cases are Burkitt's lymphoma.5 References ^ synd/2511 at Who Named It ^ Burkitt D 1958. A sarcoma involving the jaws in African children. The British journal of surgery 46 197: 218-23. doi:10.1002/bjs.18004619704. PMID 13628987. ^ Liu D, Shimonov J, Primanneni S, Lai Y, Ahmed T, Seiter K 2007. t8;14;18: a 3-way chromosome translocation in two patients with Burkitt's lymphoma/leukemia. Mol. Cancer 6: 35. doi:10.1186/1476-4598-6-35. PMID 17547754. ^ Yustein JT, Dang CV 2007. Biology and treatment of Burkitt's lymphoma. Curr. Opin. Hematol. 14 4: 375-81. doi:10.1097/MOH.0b013e3281bccdee. PMID 17534164. ^ Turgeon, Mary Louise 2005. Clinical hematology: theory and procedures. Hagerstown, MD: Lippincott Williams Wilkins, 283. ISBN 0-7817-5007-5. Frequency of lymphoid neoplasms. Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001. External links 1 General information v d e Pathology: chromosome abnormalities Q90-Q99, 758 Autosomal Trisomies Down syndrome 21, Edwards syndrome 18, Patau syndrome 13 Trisomy 9, Warkany syndrome 2 8, Trisomy 22/Cat eye syndrome 22, Trisomy 16 Monosomies/deletions Wolf-Hirschhorn syndrome 4, Cri du chat 5, Williams syndrome 7, Jacobsen syndrome 11, Miller-Dieker syndrome/Smith-Magenis syndrome 17, 22q11.2 deletion syndrome 22 genomic imprinting Angelman syndrome/Prader-Willi syndrome 15 X/Y linked Monosomy Turner syndrome XO Trisomy/ other karyotypes Klinefelter's syndrome 47,XXY, 48,XXYY, 49,XXXXY Triple X syndrome 47,XXX, 48,XXXX, 49,XXXXX 47,XYY Translocations Philadelphia chromosome, Burkitt's lymphoma Gonadal dysgenesis Mixed gonadal dysgenesis, XX gonadal dysgenesis Other Fragile X syndrome, Uniparental disomy v d e Hematological malignancy/leukemia histology ICD-O 9590-9989, C81-C96, 200-208 Lymphoid/ Lymphoproliferative Lymphomas/ Lymphoid leukemias 9590-9739, 9800-9839 B cell lymphoma, leukemia most CD19, CD20 TdT+ ALL Precursor B acute lymphoblastic leukemia/lymphoma CD5+ SLL/CLL - mantle zone Mantle cell CD22+ Prolymphocytic - Hairy cell leukemia CD79a+ marginal zone Splenic marginal zone, MALT, Nodal marginal zone germinal center Follicular, Burkitt's, Diffuse large Other B Primary effusion - Lymphomatoid granulomatosis Plasma cells Plasmacytoma - Multiple myeloma - Waldenström macroglobulinemia T cell/NK cell lymphoma, leukemia most CD3, CD4, CD8 TdT+ ALL Precursor T acute lymphoblastic leukemia/lymphoma TdT- Cutaneous - Mycosis fungoides/Sézary's disease, Angioimmunoblastic - Anaplastic large cell - Hepatosplenic - Lymphomatoid papulosis Adult - Large granular lymphocyte - Prolymphocytic - Acute lymphoblastic Aggressive NK-cell leukemia Other Hodgkin's lymphoma usually B cell - Post-transplant lymphoproliferative disorder - Acute biphenotypic leukaemia Myeloid Myeloid leukemias 9840-9939 AML: Acute myeloblastic leukemia M1, M2 - APL/M3 - monocyte M4, AMoL/M5 - Erythroleukemia/M6 - AMKL/M7 - megakaryoblast - granulocyte Acute basophilic, Acute eosinophilic, Mast cell leukemia CML: CMoL, Philadelphia chromosome - Myeloid sarcoma Myelodysplastic- myeloproliferative 9945-9946 Juvenile myelomonocytic leukemia - Chronic myelomonocytic leukemia Myeloproliferative 9950-9964 Polycythemia vera - Essential thrombocytosis - Myelofibrosis - granulocyte CNL, Chronic eosinophilic leukemia/Hypereosinophilic syndrome Myelodysplastic 9980-9989 Sideroblastic anemia - Paroxysmal nocturnal hemoglobinuria Mast cell tumor 9740-9749 Mast-cell sarcoma - Malignant mastocytosis Malignant histiocytosis 9750-9759 Langerhans cell histiocytosis See also hematology and immune disorders Retrieved from http://en..org/wiki/Burkitt%27s_lymphoma Categories: Hematology | Types of cancer | Oncology Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Bahasa Indonesia Italiano Polski Português РуÑ?Ñ?кий This page was last modified on 7 August 2008, at 15:0
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