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Burkitt's lymphoma
From Wikipedia, the free encyclopedia
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| 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.[1][2]
Almost by definition, Burkitt lymphoma are associated with c-myc gene translocation. The most common variant is t(8;14)(q24;q32) while rarer variants include t(2;8)(p12;q24) and t(8;22)(q24;q11). A three-way translocation, t(8;14;18), has also been identified.[3]
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Currently Burkitt's lymphoma can be divided into three main clinical variants: the endemic, the sporadic and the immunodeficiency-associated variants.
- 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. 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. It 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.
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.
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-cells possess a unique IgH gene rearrangement, reminiscent the fingerprint of a person. Readers are referred to other articles in Wikipedia for the mechanism of this diversity (e.g. immunoglobulin). Since Burkitt lymphoma and other B-cell lymphomas are clonal proliferative process, all tumor cells from one patient are supposed to possess identical IgH gene. When the DNA of tumor cells is analyzed using electrophoresis, a clonal band can be demonstrated since identical IgH gene 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 sometime benign reactive process (e.g. infectious mononucleosis) and malignant lymphoma can be difficult to distinct.
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 so called "tumor lysis syndrome" could occur. Close monitoring of patient and adequate hydration is essential during the process.
Other treatments are immunotherapy, bone marrow transplants, surgery to remove the tumor, and radiotherapy.
- ^ 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. PMID 13628987.
- ^ Liu D, Shimonov J, Primanneni S, Lai Y, Ahmed T, Seiter K (2007). "t(8;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.
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| Autosomal trisomies | Down syndrome (21), Edwards syndrome (18), Patau syndrome (13), Trisomy 9, Warkany syndrome 2 (8), Cat eye syndrome (22) |
| Autosomal monosomies/deletions | Wolf-Hirschhorn syndrome (4), Cri du chat (5), Angelman syndrome/Prader-Willi syndrome (15), Miller-Dieker syndrome/Smith-Magenis syndrome (17), 22q11.2 deletion syndrome (22) |
| X/Y linked | Monosomy: Turner syndrome (XO) Trisomy: Triple X syndrome (XXX), Klinefelter's syndrome (XXY), XYY |
| Translocations | Philadelphia chromosome, Burkitt's lymphoma |
| Other | Fragile X syndrome, Gonadal dysgenesis (Mixed gonadal dysgenesis) |